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1295
Chest Case of the Day David J. Eschelman,1 John F. O’Connor
Douglas
T. Gibbens,
Case 1: Tuberculous
Empyema
Necessitatis
Julia
R. Fielding,
loss.
Romo,
Daryl
R. Parker,
Nicolas
Argy,
ninth
and
mass
1 2th
1: Tuberculous
empyema necessitatis. A, Chest radiograph shows an area of fibronodular scarring in left upper lobe with retraction of left hilum. Pleural collection is present in right costophrenic angle with an adjacent chest wall mass. Periosteal reaction is noted in anterior aspect of 10th and 11th ribs on right side. B, Chest CT scan at lung win-
dow setting shows fibronodular scarring
in left upper
lobe.
C, CT scan of upper abdomen shows right-sided collection extending
pleural fluid into overly-
ing soft tissues.
I
All authors:
Department
AJR 156:1295-1300,
June
of Radiology,
Boston
University
1991 0361-803X/91/1566-1295
Medical
Center,
© American
and
in the right lateral chest wall between the ribs. The right side of the lower thorax was dull to percussion, and breath sounds were diminished at the lung base.
man had an enlarging, painless mass in the lateral chest wall. He had also noted a recent weight Physical examination revealed a soft, nontender, subFig. 1.-Case
Vitale
cutaneous
A 24-year-old right
Laura
88 E. Newton
Roentgen
St., Boston,
Ray Society
MA 021 18. Address
reprint
requests
to J. F. OConnor.
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1296
ESCHELMAN
A posteroanterior chest radiograph (Fig. 1 A) showed a right-sided, homogeneous, mass lesion obscuring the costophrenic angle and involving the adjacent extrathoracic soft tissues. Periosteal reaction was seen in the anterior aspects of the right 1 0th and 1 1 th ribs. Fibronodular scarring, especially in the left upper lobe, was noted in addition to retraction ofthe left hilum. CT of the chest (Fig. 1 B) revealed fibronodular scarring, mainly in the upper lobe of the left lung. CT of the upper abdomen (Fig. 1 C) showed a large, right-sided, wellencapsulated, pleural, low-attenuation collection with extension through the parietal pleura into the adjacent soft tissues of the inferolateral chest wall. A needle biopsy yielded acidfast bacilli. Subsequent culture was positive for Mycobacterium tuberculosis. Empyema necessitatis (a cold abscess necessitating drainage) is a collection of inflammatory tissue that usually extends directly from the pleural cavity into the thoracic wall, forming a mass in the extrapleural soft tissues [1 2]. It is a rare complication of a chronic pleural empyema, usually becoming clinically apparent many years after the acute infection. Although accounting for only 1 0% of pleural empyemas, M. tuberculosis is responsible for 73% of empyema necessitatis [3]. Rarer causes of empyema necessitatis include malignancy, other pyogenic lung abscesses, blastomycosis, and actinomycosis [1 4]. Tuberculous empyema is usually the result of a subpleural caseous focus rupturing into the pleural space [5]. Rarely, it is due to hematogenous spread, extension from thoracic lymph nodes, or extension from a subdiaphragmatic focus [3, 6]. If tuberculous empyema is inadequately treated, it may spontaneously decompress or require operative drainage. After spontaneous pleural perforation, extension usually follows fascial planes. Other sites of empyema extension include the vertebral column, paravertebral soft tissues, retroperitoneum, and more rarely the esophagus, flank, groin, and pericardium [1 -3, 7, 8]. Clinically, patients with isolated pleural empyema are frequently asymptomatic. In empyema necessitatis, patients can have pleuritic chest pain worsening with deep inspiration and rarely a nonproductive cough. Most commonly they have an enlarging, painful mass in the chest wall. Radiologically, CT findings of a thick-walled, well-encapsulated, pleural mass associated with an extrapleural mass in the chest wall are highly suggestive of tuberculous empyema necessitatis. Although direct communication between the pleural and chest wall collections is rarely demonstrated, CT is helpful in the detection and characterization of this complication of pleural tuberculosis [1 9]. Douglas T. Gibbens Nicolas Argy ,
,
,
REFERENCES 1 . Bhatt 2.
3. 4.
5.
GM, Austin HM. CT demonstration of empyema necessitatis. J Comput Assist Tomogr 1985;9: 1108-1109 Peterson MW, Austin JHM, Yip CK, McManus AP, Jaretzki A. CT findings in transdiaphragmatic empyema necessitatis due to tuberculosis. J Comput Assist Tomogr i987;1 1 :704-706 Sindel EA. Empyema necessitatis. Q Bull Sea View Hosp 1940:6:1-49 Felson B. The extrapleural space. Semin Roentgenol i977;12:327-333 Berger HW, Mejia E. Tuberculous pleurisy. Chest i973;63:88-92
ET AL.
AJA:156,
6. Sibley JC. A study of 200 cases Rev Tuberculosis i950;62:314-323 7. Tees FJ. Empyema necessitatis.
8. Marks i970;101
MI, Eickhoff
of tuberculous Arch
TC. Empyema
Surg
pleurisy
June 1991
with effusion.
i923;7:321-331 Am
necessitatis.
Rev
Respir
Am
Dis
:759-761
9. Glicklich M, Mendelson DS, Gendal ES, Teirstein AS. Tuberculous empyema necessitatis: computed tomographic findings. Cl/n Imaging i990;14:23-25
Case 2: Cutaneous Breasts
T-CelI Lymphoma
Involving
Both
This 61 -year-old woman had a 1 0-year history of progressive skin ulcerations diagnosed by skin biopsy in 1 982 as cutaneous T-cell lymphoma (CTCL), previously known as mycosis fungoides. She complained to her dermatologist at this time of dysphagia and groin swelling. Physical examination revealed diffuse lymphadenopathy and ulcerating, nodular skin lesions of the trunk and extremities. Multiple, bilateral breast masses also were palpated. Results of a barium swallow were normal. A CT study was performed to delineate the extent of the lymphadenopathy (Figs. 2A and 2B). CT scans through the chest showed extensive axillary adenopathy, left internal mammary adenopathy, and multiple soft-tissue masses in both breasts. The breast masses were further evaluated by mammography (Figs. 2C and 2D), which revealed several well-defined, round lesions in both breasts without associated microcalcifications or skin changes. Biopsy of each breast was performed, and the masses were found to be T-cell lymphoma. CTCL encompasses mycosis fungoides, S#{233}zary syndrome, reticulum cell sarcoma, lymphoma cutis, and most T-ceII leukemias [1]. First described in 1806 by Jean Louis Alibert, CTCL is a malignant lymphoma that begins in the skin. The annual incidence in the United States is increasing and now surpasses that of Hodgkin disease [2]. Prevalence is estimated at 40,000 to 50,000 cases [3]. CTCL occurs twice as often in males as in females and is the only lymphoma more common in blacks than whites. It is usually diagnosed in the fifth decade of life. The causative agent is unknown; however, there is an association with human T-cell lymphoma virus (HTLV) [1 , 3]. There is no cure for the disease, but many palliative therapies are in use. Cutaneous lesions are treated with topical steroids, nitrogen mustard, ultraviolet light, and electron beam therapy. Extracutaneous disease requires systemic chemotherapy, radiation therapy, or photophoresis, sometimes in combination. The mean survival time after diagnosis is 3 to 1 0 years. CTCL progresses through three clinical stages, termed patch or premycotic, plaque, and tumor [1 3]. The macular lesions in the patch stage resemble several benign dermatoses and are localized to the trunk and extremities. Plaques may develop from patch lesions or arise de novo in any area of the body. They are raised, scaly, and pruritic. Painless tumors or nodules generally originate in plaques and often ulcerate. Extracutaneous disease eventually occurs in 50% of all patients. Spread occurs hematogenously and via the lymphatics, although not in an organized fashion. The liver is the organ most often affected. Gastrointestinal, cardiac, pulmonary, renal, and central and peripheral nervous system ,
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AJA:156,
CHEST
June 1991
Fig. 2.-Case cell
2: Cutaneous
lymphoma
breasts. A, Transaxial
involving
CASE
OF
THE
1297
DAY
Tboth
CT scan at level
of aortic arch shows bilateral axillary lymphadenopathy, greater on left side than on right side. B, Transaxial CT scan at a level 5 cm below carina shows multiple soft-tissue masses in right breast
and left internal mammary lymphadenopathy. C and D, Bilateral mammograms (mediolateral-oblique
views) show multiple round, welldefined
breasts,
masses
cations, and lymphadenopathy.
involvement
both
macrocalcifi-
bilateral
axillary
been observed [1 ]. To our knowledge, no described involvement of the breast. The bones are usually spared. When present, lesions are generally osteolytic. The presence of extracutaneous disease mandates the use of systemic therapy and has a poorer prognosis. The diagnosis of CTCL is generally made by punch biopsy of a skin lesion in the patch or plaque stage. Imaging defines the extent of extracutaneous disease and can be used to monitor the response to therapy. CT is useful in identifying lymphadenopathy and visceral involvement. Chest radiographs may reveal pulmonary nodules, patchy infiltrates, and hilar lymphadenopathy in the late stages of the disease [1]. Lymphorna manifests itself in the breast parenchyrna in many ways. Mammography may show a single nodule, multiple masses, an increased interstitial pattern, or diffuse increase in density [4, 5]. Massive axillary lymphadenopathy is rarely seen with breast carcinoma and should raise the suspicion of lymphorna [6]. In identifying the presence of extracutaneous disease, the radiologist assists in defining appropriate therapy for CTCL for prolonging patients’ survival. Julia A. Fielding prior
reports
have
within
scattered
have
REFERENCES 1 . Knoble AM, Edelson AL. Cutaneous T cell lymphorna. Med C/in North Am 1986;70: 109-138 2. Weinstock MA, Horm JW. Mycosis fungoides in the United States. JAMA
1988;260:42-46 3. Worobec-Victor S. Cutaneous T cell lymphoma. N J Med 1989:86: 395-400 4. Meyer JE, Kopans DB, Long JC. Mammographic appearance of malignant lymphoma of the breast. Radiology 1980: 1 35 : 623-626 5. Aubanel D, Bruneton JN. Gynecologic lymphomas. In: Bruneton JN, Schneider M, eds. Radiology of lymphomas. New York: Springer-Verlag, 1986:99-102 6. Kopans DB. Breast imaging. Philadelphia: Lippincott, 1989:305-307
Case
3: Endodermal
Sinus
Tumor
of the Mediastinum
This 28-year-old man had a 3-month history of pain in the right shoulder and a 1 -month history of hemoptysis, dysphagia, and night sweats. He had an elevated serum level of the beta subunit of human chorionic gonadotropin and alphafetoprotein (AFP). Chest radiographs (Figs. 3A and 3B) showed a vaguely defined anterior mediastinal mass with
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1298
ESCHELMAN
confluent densities in the upper, mid, and lower right hemithorax. Chest CT (Figs. 3C and 3D) revealed a large, heterogeneous anterior mediastinal mass with irregular borders. The tumor extended into the right hemithorax, displacing the right middle lobe bronchus, with growth continuing to the anterior chest wall through the upper sternum and into the middle mediastinum with obliteration of the fat planes between the mass and the right cardiac border, suggesting invasion. A second necrotic-appearing mass was present next to the major fissure on the right side within the right lower lobe. Findings on scrotal sonography (not shown) were normal. Abdominal CT, MR imaging of the thoracic and lumbar spine, and bone scanning (not shown) revealed hepatic, vertebral, sternal, and calvarial metastases. A pathologic specimen obtained by open biopsy was diagnostic of an endodermal sinus tumor of the mediastinum. Endodermal sinus tumors are the rarest of the germ cell tumors. Germ cell tumors are divided into the serninomas and the nonserninomas, namely the endodermal sinus tumors, teratomas, choriocarcinomas, and embryonal carcinomas. Although this group of tumors is classically associated with the
ET AL.
AJR:156,
June
1991
gonads, extragonadal sites such as the mediastinum are primary sites of occurrence. On the whole, germ cell tumors account for approximately 20% of all mediastinal tumors and cysts. Like their gonadal counterparts, mediastinal germ cell tumors also are derived from primitive germ cell elements [1]. The presence of germ cell elements in the mediastinum has been a topic of great debate. Originally, it was proposed that these mediastinal tumors were metastatic from gonadal tissue. In the absence of definable gonadal tumor, it was theorized that there must be an undefinable regressed focus. Today, however, the most favored explanation is failure of germ cells to migrate along the urogenital ridge to the gonads [2]. Whereas serninomas affect males only, the nonserninomatous tumors also affect females, although a male predilection is maintained. Germ cell tumors affect persons in the 20-30 year age range. Mediastinal endodermal sinus tumors, embryonal carcinomas, and choriocarcinomas are not only more aggressive lesions than their seminomatous counterparts, but they are also more aggressive when compared with their
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AJA:156,
June 1991
CHEST
CASE
identical histologic testicular counterparts. The single most favorable prognostic factor for mediastinal endodermal sinus tumors is complete resectability. Endodermal sinus tumors secrete AFP, and tumor activity is strongly correlated with the serum level. Human chorionic gonadotropin is detectable in some cases of endodermal sinus tumor; however, it is generally accepted that this endocrinopathy is due to small foci of choriocarcinorna or embryonal carcinoma within the endoderrnal sinus tumor [3, 4]. As do all germ cell tumors, endodermal sinus tumors arise within the anterior mediastinum. Extension into the middle or posterior mediastinum and the lung may occur. Bony invasion by direct extension, particularly of the sternum, is not uncommon. Chest radiographs are used in making initial observations. In the last decade, CT has been found to better define the tissue characteristics and extent of tumor invasion of endodermal sinus tumors and germ cell tumors as a whole. CT has been very useful in differentiating between the nonseminomatous and seminomatous germ cell tumors. Seminomas have been described as large, sharply demarcated masses that are usually of homogeneous attenuation. Chest wall invasion is rare, as is the development of pleuropericardial effusions. In contrast, the nonseminomatous tumors have spiculated borders and are of heterogeneous attenuation. The areas of lower attenuation within the tumors are due to necrosis and hemorrhage. A strong tendency for bony invasion and fat plane obliteration exists. Calcification and thick capsules that enhance after IV administration of contrast material have been described. Pleural effusions are more common [5-8]. Although CT may be helpful in differentiating between the two subtypes of germ cell tumors, its use in differentiating germ cell tumors from other tumors of the anterior mediastinum is more questionable. Thymomas, carcinoid tumors, lymphoma, and mediastinal metastases must also be considered in the differential diagnosis of heterogeneous anterior mediastinal masses by CT. Although benign thymomas are usually round or oval masses of homogeneous attenuation, thymomas that undergo cystic degeneration gain irregular borders along with the tendency to invade fat planes. Some may also calcify. CT features do not differentiate these thymomas from the nonseminomatous germ cell tumors [5, 7, 8]. In addition, cystic forms of Hodgkin disease or mediastinal lymph nodes involved by metastatic disease may also resemble the nonseminomatous germ cell tumors [5, 6]. Carcinoid, although usually a well-circumscribed mass, may show fat plane obliteration and calcification [5]. In conclusion, some advances have been made to better characterize germ cell tumors of the anterior mediastinum. Difficulty still exists, however, in differentiating germ cell tumors from other anterior mediastinal tumors. Germ cell tumors should always be considered in the differential diagnosis of an anterior mediastinal mass in a young patient. Laura Vitale Romo Daryl R. Parker
REFERENCES 1 . Rosai J. Ackerman s surgical pathology. St. Louis: Mosby. 1989 :367 2. Luna MA, Valenzuela-Tamariz J. Germ-cell tumors of the mediastinum:
OF
THE
DAY
postmortem 3. Knapp RH, mediastinum.
1299
findings. Am J C/in Pathol 1976;65:450-454 Hurt AD, Payne 5, et al. Malignant germ cell tumors J Thorac Cardiovasc Surg 1985;89:82-89
4. Truong LD, Hams L, Mattioli C, et al. Endodermal 5.
6.
7. 8.
of the
sinus tumor of the
mediastinum. Cancer i986;58: 730-739 Lee KS, Im JG, Han CH. Han MC, Kim CW, Kim WS. Malignant primary germ cell tumors of the mediastinum: CT features. AJR i989;1 53: 947-951 Blomlie V, Lien H, Fossa SD, Jacobsen AB, Stenwig AE. Computed tomography in primary non-seminomatous germ cell tumors of the mediastinum. Acta Radiol i988;29:289-292 Levitt AG, Husband JE, Glazer HS. CT of primary germ-cell tumors of the mediastinum. AJR i984;142:73-78 Mon K, Eguchi K, Moriyama H, Miyazawa N, Kodama T. Computed tomography of anterior mediastinal tumors: differentiation between thymoma and germ cell tumor. Acta Radiol 1987:28: 395-398
Case 4: Lateral Neurofibromatosis
Thoracic
Meningocele
in a Patient
with
A 52-year-old man came to the emergency department with shortness of breath and wheezing. A posteroanterior chest radiograph (Fig. 4A) revealed a round, well-circumscribed, left paravertebral, soft-tissue mass measuring 6 cm next to the T5-T6 vertebral bodies. A short focal scoliosis convex toward the lesion, cutaneous neurofibromas in the extrathoracic soft tissues on the right side, and rib notching also were seen. A lateral chest radiograph (Fig. 4B) showed a posterior mediastinal mass in the upper thoracic region with enlarged neural foramina and scalloping of the dorsal surfaces of the T5-T8 vertebral bodies. Lateral view of myelogram (Fig. 4C) showed a rounded meningocele corresponding to the posterior mediastinal soft-tissue mass on chest radiographs, as well as widening of the subarachnoid space with extension of contrast material into scalloped vertebral bodies. Lateral paravertebral meningoceles are an uncommon anomaly of the meninges manifested by lateral extrusion of the dura and arachnoid through an enlarged intervertebral foramen into the extrapleural thoracic gutter [1 ]. Males and females are equally affected, most commonly between 30 and 50 years of age [2]. Eight-five percent of paravertebral meningoceles are seen in patients with neurofibromatosis [3]. Moreover, thoracic paraspinal masses are more likely to be meningoceles than neurofibromas in these patients [1 46]. The majority (70%) of lateral thoracic meningoceles are right sided [1]. They frequently occupy a single neural foramen in the upper thoracic region (T3-T7), usually T5-T6 [7]. Their size varies from almost undetectable to nearly the size of the hernithorax itself. In neonates, large meningoceles may cause significant respiratory compromise [2]. Clinically, patients with lateral thoracic meningoceles may have a cough or dyspnea, but more commonly they are asymptomatic. Some patients have pain (23%); minor neurologic symptoms including sensory deficits, weakness, and hyperreflexia (1 0%); or severe paresis (9%) [8]. The acute symptoms in this patient were unrelated to his known diagnosis of neurofibrornatosis. Chest films of patients with lateral thoracic meningoceles usually exhibit a short focal scoliosis of the upper thoracic spine, convex toward the lesion. However, kyphosis may be more prominent than is scoliosis [9]. The neural foramina are enlarged, with thinning of adjacent pedicles, focal increase in ,
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interpediculate distance, thinned lamina, and scalloping of the dorsal surfaces of adjacent vertebral bodies [7]. The adjacent intercostal space may be widened, with erosion of contiguous rib margins [10]. Fused or hypoplastic ribs and vertebrae may coexist [2, 3, 1 0]. Adjacent ventral roots may be hypoplastic or absent on the ipsilateral or contralateral side of the meningocele [2]. MR signal intensity of CSF within the meningocele may appear brighter than that within the spinal canal because of dampened CSF pulsations [1 1]. Douglas T. Gibbens Nicolas Argy REFERENCES 1 . Bunner
A. Lateral
intrathoracic meningocele. Acta Radio/ i959;51 : 1-9 EE. Lateral intrathoracic meningocele: case report with preoperative diagnosis. AJR 1963:90:1216-1219 3. Erkulvrawatr S. El Gammal T, Hawkins J, et al. Intrathoracic meningoceles and neurofibromatosis. Arch Neurol i979;36: 557-559
2. Chandler A, Herzberger
4. Kent EM, Blades B, Valle AR, Graham EA. Intrathoracic neurogenic tumors. J Thorac Surg 1944; 13:116-161 5. Blades B. Mediastinal tumors: report of cases treated at Army thoracic centers in the United States. Ann Surg 1946;1 23:749-765 6. Hagelstam L. On the deformities of spine and multiple neurofibromatosis (von Aecklinghausen). Acta Chir Scand i946;93: 169-193 7. Naidich TP, McLone DG, Harwood-Nash DC. Arachnoid cysts, paravertebral meningoceles, and perineurial cysts. In: Newton TH, Potts DG, eds. Computed tomography of the spine and spinal cord. San Anselmo, CA: Clavadel Press, 1983 :388-390 8. Miles J, Pennybacker J, Sheldon P. Intrathoracic meningocele: its development and association with neurofibromatosis. J Neurol Neurosurg Psychiatr 1969;32:99-1 10 9. Healy JF, Wells MV, Carlstrom T, Rosenkrantz H. Lateral thoracic meningocele demonstrated by computerized tomography. Comput Tomogr i980;4: 159-163 1 0. Shealy CN, Le May M. Intrathoracic meningocele: two additional cases of this rare entity. J Neurosurg 1964:21 : 880-883 1 1 . Flannigan-Sprague BD, Medic MT. The pediatric spine: normal anatomy and spinal dysraphism. In: Medic MT. Masaryk TJ, Ross JS, eds. Magnetic resonance imaging of the spine. Chicago: Year Book Medical, 1989:244
1295
Chest Case of the Day David J. Eschelman,1 John F. O’Connor
Douglas
T. Gibbens,
Case 1: Tuberculous
Empyema
Necessitatis
Julia
R. Fielding,
loss.
Romo,
Daryl
R. Parker,
Nicolas
Argy,
ninth
and
mass
1 2th
1: Tuberculous
empyema necessitatis. A, Chest radiograph shows an area of fibronodular scarring in left upper lobe with retraction of left hilum. Pleural collection is present in right costophrenic angle with an adjacent chest wall mass. Periosteal reaction is noted in anterior aspect of 10th and 11th ribs on right side. B, Chest CT scan at lung win-
dow setting shows fibronodular scarring
in left upper
lobe.
C, CT scan of upper abdomen shows right-sided collection extending
pleural fluid into overly-
ing soft tissues.
I
All authors:
Department
AJR 156:1295-1300,
June
of Radiology,
Boston
University
1991 0361-803X/91/1566-1295
Medical
Center,
© American
and
in the right lateral chest wall between the ribs. The right side of the lower thorax was dull to percussion, and breath sounds were diminished at the lung base.
man had an enlarging, painless mass in the lateral chest wall. He had also noted a recent weight Physical examination revealed a soft, nontender, subFig. 1.-Case
Vitale
cutaneous
A 24-year-old right
Laura
88 E. Newton
Roentgen
St., Boston,
Ray Society
MA 021 18. Address
reprint
requests
to J. F. OConnor.
Downloaded from www.ajronline.org by 197.255.205.211 on 10/12/15 from IP address 197.255.205.211. Copyright ARRS. For personal use only; all rights reserved
1296
ESCHELMAN
A posteroanterior chest radiograph (Fig. 1 A) showed a right-sided, homogeneous, mass lesion obscuring the costophrenic angle and involving the adjacent extrathoracic soft tissues. Periosteal reaction was seen in the anterior aspects of the right 1 0th and 1 1 th ribs. Fibronodular scarring, especially in the left upper lobe, was noted in addition to retraction ofthe left hilum. CT of the chest (Fig. 1 B) revealed fibronodular scarring, mainly in the upper lobe of the left lung. CT of the upper abdomen (Fig. 1 C) showed a large, right-sided, wellencapsulated, pleural, low-attenuation collection with extension through the parietal pleura into the adjacent soft tissues of the inferolateral chest wall. A needle biopsy yielded acidfast bacilli. Subsequent culture was positive for Mycobacterium tuberculosis. Empyema necessitatis (a cold abscess necessitating drainage) is a collection of inflammatory tissue that usually extends directly from the pleural cavity into the thoracic wall, forming a mass in the extrapleural soft tissues [1 2]. It is a rare complication of a chronic pleural empyema, usually becoming clinically apparent many years after the acute infection. Although accounting for only 1 0% of pleural empyemas, M. tuberculosis is responsible for 73% of empyema necessitatis [3]. Rarer causes of empyema necessitatis include malignancy, other pyogenic lung abscesses, blastomycosis, and actinomycosis [1 4]. Tuberculous empyema is usually the result of a subpleural caseous focus rupturing into the pleural space [5]. Rarely, it is due to hematogenous spread, extension from thoracic lymph nodes, or extension from a subdiaphragmatic focus [3, 6]. If tuberculous empyema is inadequately treated, it may spontaneously decompress or require operative drainage. After spontaneous pleural perforation, extension usually follows fascial planes. Other sites of empyema extension include the vertebral column, paravertebral soft tissues, retroperitoneum, and more rarely the esophagus, flank, groin, and pericardium [1 -3, 7, 8]. Clinically, patients with isolated pleural empyema are frequently asymptomatic. In empyema necessitatis, patients can have pleuritic chest pain worsening with deep inspiration and rarely a nonproductive cough. Most commonly they have an enlarging, painful mass in the chest wall. Radiologically, CT findings of a thick-walled, well-encapsulated, pleural mass associated with an extrapleural mass in the chest wall are highly suggestive of tuberculous empyema necessitatis. Although direct communication between the pleural and chest wall collections is rarely demonstrated, CT is helpful in the detection and characterization of this complication of pleural tuberculosis [1 9]. Douglas T. Gibbens Nicolas Argy ,
,
,
REFERENCES 1 . Bhatt 2.
3. 4.
5.
GM, Austin HM. CT demonstration of empyema necessitatis. J Comput Assist Tomogr 1985;9: 1108-1109 Peterson MW, Austin JHM, Yip CK, McManus AP, Jaretzki A. CT findings in transdiaphragmatic empyema necessitatis due to tuberculosis. J Comput Assist Tomogr i987;1 1 :704-706 Sindel EA. Empyema necessitatis. Q Bull Sea View Hosp 1940:6:1-49 Felson B. The extrapleural space. Semin Roentgenol i977;12:327-333 Berger HW, Mejia E. Tuberculous pleurisy. Chest i973;63:88-92
ET AL.
AJA:156,
6. Sibley JC. A study of 200 cases Rev Tuberculosis i950;62:314-323 7. Tees FJ. Empyema necessitatis.
8. Marks i970;101
MI, Eickhoff
of tuberculous Arch
TC. Empyema
Surg
pleurisy
June 1991
with effusion.
i923;7:321-331 Am
necessitatis.
Rev
Respir
Am
Dis
:759-761
9. Glicklich M, Mendelson DS, Gendal ES, Teirstein AS. Tuberculous empyema necessitatis: computed tomographic findings. Cl/n Imaging i990;14:23-25
Case 2: Cutaneous Breasts
T-CelI Lymphoma
Involving
Both
This 61 -year-old woman had a 1 0-year history of progressive skin ulcerations diagnosed by skin biopsy in 1 982 as cutaneous T-cell lymphoma (CTCL), previously known as mycosis fungoides. She complained to her dermatologist at this time of dysphagia and groin swelling. Physical examination revealed diffuse lymphadenopathy and ulcerating, nodular skin lesions of the trunk and extremities. Multiple, bilateral breast masses also were palpated. Results of a barium swallow were normal. A CT study was performed to delineate the extent of the lymphadenopathy (Figs. 2A and 2B). CT scans through the chest showed extensive axillary adenopathy, left internal mammary adenopathy, and multiple soft-tissue masses in both breasts. The breast masses were further evaluated by mammography (Figs. 2C and 2D), which revealed several well-defined, round lesions in both breasts without associated microcalcifications or skin changes. Biopsy of each breast was performed, and the masses were found to be T-cell lymphoma. CTCL encompasses mycosis fungoides, S#{233}zary syndrome, reticulum cell sarcoma, lymphoma cutis, and most T-ceII leukemias [1]. First described in 1806 by Jean Louis Alibert, CTCL is a malignant lymphoma that begins in the skin. The annual incidence in the United States is increasing and now surpasses that of Hodgkin disease [2]. Prevalence is estimated at 40,000 to 50,000 cases [3]. CTCL occurs twice as often in males as in females and is the only lymphoma more common in blacks than whites. It is usually diagnosed in the fifth decade of life. The causative agent is unknown; however, there is an association with human T-cell lymphoma virus (HTLV) [1 , 3]. There is no cure for the disease, but many palliative therapies are in use. Cutaneous lesions are treated with topical steroids, nitrogen mustard, ultraviolet light, and electron beam therapy. Extracutaneous disease requires systemic chemotherapy, radiation therapy, or photophoresis, sometimes in combination. The mean survival time after diagnosis is 3 to 1 0 years. CTCL progresses through three clinical stages, termed patch or premycotic, plaque, and tumor [1 3]. The macular lesions in the patch stage resemble several benign dermatoses and are localized to the trunk and extremities. Plaques may develop from patch lesions or arise de novo in any area of the body. They are raised, scaly, and pruritic. Painless tumors or nodules generally originate in plaques and often ulcerate. Extracutaneous disease eventually occurs in 50% of all patients. Spread occurs hematogenously and via the lymphatics, although not in an organized fashion. The liver is the organ most often affected. Gastrointestinal, cardiac, pulmonary, renal, and central and peripheral nervous system ,
Downloaded from www.ajronline.org by 197.255.205.211 on 10/12/15 from IP address 197.255.205.211. Copyright ARRS. For personal use only; all rights reserved
AJA:156,
CHEST
June 1991
Fig. 2.-Case cell
2: Cutaneous
lymphoma
breasts. A, Transaxial
involving
CASE
OF
THE
1297
DAY
Tboth
CT scan at level
of aortic arch shows bilateral axillary lymphadenopathy, greater on left side than on right side. B, Transaxial CT scan at a level 5 cm below carina shows multiple soft-tissue masses in right breast
and left internal mammary lymphadenopathy. C and D, Bilateral mammograms (mediolateral-oblique
views) show multiple round, welldefined
breasts,
masses
cations, and lymphadenopathy.
involvement
both
macrocalcifi-
bilateral
axillary
been observed [1 ]. To our knowledge, no described involvement of the breast. The bones are usually spared. When present, lesions are generally osteolytic. The presence of extracutaneous disease mandates the use of systemic therapy and has a poorer prognosis. The diagnosis of CTCL is generally made by punch biopsy of a skin lesion in the patch or plaque stage. Imaging defines the extent of extracutaneous disease and can be used to monitor the response to therapy. CT is useful in identifying lymphadenopathy and visceral involvement. Chest radiographs may reveal pulmonary nodules, patchy infiltrates, and hilar lymphadenopathy in the late stages of the disease [1]. Lymphorna manifests itself in the breast parenchyrna in many ways. Mammography may show a single nodule, multiple masses, an increased interstitial pattern, or diffuse increase in density [4, 5]. Massive axillary lymphadenopathy is rarely seen with breast carcinoma and should raise the suspicion of lymphorna [6]. In identifying the presence of extracutaneous disease, the radiologist assists in defining appropriate therapy for CTCL for prolonging patients’ survival. Julia A. Fielding prior
reports
have
within
scattered
have
REFERENCES 1 . Knoble AM, Edelson AL. Cutaneous T cell lymphorna. Med C/in North Am 1986;70: 109-138 2. Weinstock MA, Horm JW. Mycosis fungoides in the United States. JAMA
1988;260:42-46 3. Worobec-Victor S. Cutaneous T cell lymphoma. N J Med 1989:86: 395-400 4. Meyer JE, Kopans DB, Long JC. Mammographic appearance of malignant lymphoma of the breast. Radiology 1980: 1 35 : 623-626 5. Aubanel D, Bruneton JN. Gynecologic lymphomas. In: Bruneton JN, Schneider M, eds. Radiology of lymphomas. New York: Springer-Verlag, 1986:99-102 6. Kopans DB. Breast imaging. Philadelphia: Lippincott, 1989:305-307
Case
3: Endodermal
Sinus
Tumor
of the Mediastinum
This 28-year-old man had a 3-month history of pain in the right shoulder and a 1 -month history of hemoptysis, dysphagia, and night sweats. He had an elevated serum level of the beta subunit of human chorionic gonadotropin and alphafetoprotein (AFP). Chest radiographs (Figs. 3A and 3B) showed a vaguely defined anterior mediastinal mass with
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1298
ESCHELMAN
confluent densities in the upper, mid, and lower right hemithorax. Chest CT (Figs. 3C and 3D) revealed a large, heterogeneous anterior mediastinal mass with irregular borders. The tumor extended into the right hemithorax, displacing the right middle lobe bronchus, with growth continuing to the anterior chest wall through the upper sternum and into the middle mediastinum with obliteration of the fat planes between the mass and the right cardiac border, suggesting invasion. A second necrotic-appearing mass was present next to the major fissure on the right side within the right lower lobe. Findings on scrotal sonography (not shown) were normal. Abdominal CT, MR imaging of the thoracic and lumbar spine, and bone scanning (not shown) revealed hepatic, vertebral, sternal, and calvarial metastases. A pathologic specimen obtained by open biopsy was diagnostic of an endodermal sinus tumor of the mediastinum. Endodermal sinus tumors are the rarest of the germ cell tumors. Germ cell tumors are divided into the serninomas and the nonserninomas, namely the endodermal sinus tumors, teratomas, choriocarcinomas, and embryonal carcinomas. Although this group of tumors is classically associated with the
ET AL.
AJR:156,
June
1991
gonads, extragonadal sites such as the mediastinum are primary sites of occurrence. On the whole, germ cell tumors account for approximately 20% of all mediastinal tumors and cysts. Like their gonadal counterparts, mediastinal germ cell tumors also are derived from primitive germ cell elements [1]. The presence of germ cell elements in the mediastinum has been a topic of great debate. Originally, it was proposed that these mediastinal tumors were metastatic from gonadal tissue. In the absence of definable gonadal tumor, it was theorized that there must be an undefinable regressed focus. Today, however, the most favored explanation is failure of germ cells to migrate along the urogenital ridge to the gonads [2]. Whereas serninomas affect males only, the nonserninomatous tumors also affect females, although a male predilection is maintained. Germ cell tumors affect persons in the 20-30 year age range. Mediastinal endodermal sinus tumors, embryonal carcinomas, and choriocarcinomas are not only more aggressive lesions than their seminomatous counterparts, but they are also more aggressive when compared with their
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AJA:156,
June 1991
CHEST
CASE
identical histologic testicular counterparts. The single most favorable prognostic factor for mediastinal endodermal sinus tumors is complete resectability. Endodermal sinus tumors secrete AFP, and tumor activity is strongly correlated with the serum level. Human chorionic gonadotropin is detectable in some cases of endodermal sinus tumor; however, it is generally accepted that this endocrinopathy is due to small foci of choriocarcinorna or embryonal carcinoma within the endoderrnal sinus tumor [3, 4]. As do all germ cell tumors, endodermal sinus tumors arise within the anterior mediastinum. Extension into the middle or posterior mediastinum and the lung may occur. Bony invasion by direct extension, particularly of the sternum, is not uncommon. Chest radiographs are used in making initial observations. In the last decade, CT has been found to better define the tissue characteristics and extent of tumor invasion of endodermal sinus tumors and germ cell tumors as a whole. CT has been very useful in differentiating between the nonseminomatous and seminomatous germ cell tumors. Seminomas have been described as large, sharply demarcated masses that are usually of homogeneous attenuation. Chest wall invasion is rare, as is the development of pleuropericardial effusions. In contrast, the nonseminomatous tumors have spiculated borders and are of heterogeneous attenuation. The areas of lower attenuation within the tumors are due to necrosis and hemorrhage. A strong tendency for bony invasion and fat plane obliteration exists. Calcification and thick capsules that enhance after IV administration of contrast material have been described. Pleural effusions are more common [5-8]. Although CT may be helpful in differentiating between the two subtypes of germ cell tumors, its use in differentiating germ cell tumors from other tumors of the anterior mediastinum is more questionable. Thymomas, carcinoid tumors, lymphoma, and mediastinal metastases must also be considered in the differential diagnosis of heterogeneous anterior mediastinal masses by CT. Although benign thymomas are usually round or oval masses of homogeneous attenuation, thymomas that undergo cystic degeneration gain irregular borders along with the tendency to invade fat planes. Some may also calcify. CT features do not differentiate these thymomas from the nonseminomatous germ cell tumors [5, 7, 8]. In addition, cystic forms of Hodgkin disease or mediastinal lymph nodes involved by metastatic disease may also resemble the nonseminomatous germ cell tumors [5, 6]. Carcinoid, although usually a well-circumscribed mass, may show fat plane obliteration and calcification [5]. In conclusion, some advances have been made to better characterize germ cell tumors of the anterior mediastinum. Difficulty still exists, however, in differentiating germ cell tumors from other anterior mediastinal tumors. Germ cell tumors should always be considered in the differential diagnosis of an anterior mediastinal mass in a young patient. Laura Vitale Romo Daryl R. Parker
REFERENCES 1 . Rosai J. Ackerman s surgical pathology. St. Louis: Mosby. 1989 :367 2. Luna MA, Valenzuela-Tamariz J. Germ-cell tumors of the mediastinum:
OF
THE
DAY
postmortem 3. Knapp RH, mediastinum.
1299
findings. Am J C/in Pathol 1976;65:450-454 Hurt AD, Payne 5, et al. Malignant germ cell tumors J Thorac Cardiovasc Surg 1985;89:82-89
4. Truong LD, Hams L, Mattioli C, et al. Endodermal 5.
6.
7. 8.
of the
sinus tumor of the
mediastinum. Cancer i986;58: 730-739 Lee KS, Im JG, Han CH. Han MC, Kim CW, Kim WS. Malignant primary germ cell tumors of the mediastinum: CT features. AJR i989;1 53: 947-951 Blomlie V, Lien H, Fossa SD, Jacobsen AB, Stenwig AE. Computed tomography in primary non-seminomatous germ cell tumors of the mediastinum. Acta Radiol i988;29:289-292 Levitt AG, Husband JE, Glazer HS. CT of primary germ-cell tumors of the mediastinum. AJR i984;142:73-78 Mon K, Eguchi K, Moriyama H, Miyazawa N, Kodama T. Computed tomography of anterior mediastinal tumors: differentiation between thymoma and germ cell tumor. Acta Radiol 1987:28: 395-398
Case 4: Lateral Neurofibromatosis
Thoracic
Meningocele
in a Patient
with
A 52-year-old man came to the emergency department with shortness of breath and wheezing. A posteroanterior chest radiograph (Fig. 4A) revealed a round, well-circumscribed, left paravertebral, soft-tissue mass measuring 6 cm next to the T5-T6 vertebral bodies. A short focal scoliosis convex toward the lesion, cutaneous neurofibromas in the extrathoracic soft tissues on the right side, and rib notching also were seen. A lateral chest radiograph (Fig. 4B) showed a posterior mediastinal mass in the upper thoracic region with enlarged neural foramina and scalloping of the dorsal surfaces of the T5-T8 vertebral bodies. Lateral view of myelogram (Fig. 4C) showed a rounded meningocele corresponding to the posterior mediastinal soft-tissue mass on chest radiographs, as well as widening of the subarachnoid space with extension of contrast material into scalloped vertebral bodies. Lateral paravertebral meningoceles are an uncommon anomaly of the meninges manifested by lateral extrusion of the dura and arachnoid through an enlarged intervertebral foramen into the extrapleural thoracic gutter [1 ]. Males and females are equally affected, most commonly between 30 and 50 years of age [2]. Eight-five percent of paravertebral meningoceles are seen in patients with neurofibromatosis [3]. Moreover, thoracic paraspinal masses are more likely to be meningoceles than neurofibromas in these patients [1 46]. The majority (70%) of lateral thoracic meningoceles are right sided [1]. They frequently occupy a single neural foramen in the upper thoracic region (T3-T7), usually T5-T6 [7]. Their size varies from almost undetectable to nearly the size of the hernithorax itself. In neonates, large meningoceles may cause significant respiratory compromise [2]. Clinically, patients with lateral thoracic meningoceles may have a cough or dyspnea, but more commonly they are asymptomatic. Some patients have pain (23%); minor neurologic symptoms including sensory deficits, weakness, and hyperreflexia (1 0%); or severe paresis (9%) [8]. The acute symptoms in this patient were unrelated to his known diagnosis of neurofibrornatosis. Chest films of patients with lateral thoracic meningoceles usually exhibit a short focal scoliosis of the upper thoracic spine, convex toward the lesion. However, kyphosis may be more prominent than is scoliosis [9]. The neural foramina are enlarged, with thinning of adjacent pedicles, focal increase in ,
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interpediculate distance, thinned lamina, and scalloping of the dorsal surfaces of adjacent vertebral bodies [7]. The adjacent intercostal space may be widened, with erosion of contiguous rib margins [10]. Fused or hypoplastic ribs and vertebrae may coexist [2, 3, 1 0]. Adjacent ventral roots may be hypoplastic or absent on the ipsilateral or contralateral side of the meningocele [2]. MR signal intensity of CSF within the meningocele may appear brighter than that within the spinal canal because of dampened CSF pulsations [1 1]. Douglas T. Gibbens Nicolas Argy REFERENCES 1 . Bunner
A. Lateral
intrathoracic meningocele. Acta Radio/ i959;51 : 1-9 EE. Lateral intrathoracic meningocele: case report with preoperative diagnosis. AJR 1963:90:1216-1219 3. Erkulvrawatr S. El Gammal T, Hawkins J, et al. Intrathoracic meningoceles and neurofibromatosis. Arch Neurol i979;36: 557-559
2. Chandler A, Herzberger
4. Kent EM, Blades B, Valle AR, Graham EA. Intrathoracic neurogenic tumors. J Thorac Surg 1944; 13:116-161 5. Blades B. Mediastinal tumors: report of cases treated at Army thoracic centers in the United States. Ann Surg 1946;1 23:749-765 6. Hagelstam L. On the deformities of spine and multiple neurofibromatosis (von Aecklinghausen). Acta Chir Scand i946;93: 169-193 7. Naidich TP, McLone DG, Harwood-Nash DC. Arachnoid cysts, paravertebral meningoceles, and perineurial cysts. In: Newton TH, Potts DG, eds. Computed tomography of the spine and spinal cord. San Anselmo, CA: Clavadel Press, 1983 :388-390 8. Miles J, Pennybacker J, Sheldon P. Intrathoracic meningocele: its development and association with neurofibromatosis. J Neurol Neurosurg Psychiatr 1969;32:99-1 10 9. Healy JF, Wells MV, Carlstrom T, Rosenkrantz H. Lateral thoracic meningocele demonstrated by computerized tomography. Comput Tomogr i980;4: 159-163 1 0. Shealy CN, Le May M. Intrathoracic meningocele: two additional cases of this rare entity. J Neurosurg 1964:21 : 880-883 1 1 . Flannigan-Sprague BD, Medic MT. The pediatric spine: normal anatomy and spinal dysraphism. In: Medic MT. Masaryk TJ, Ross JS, eds. Magnetic resonance imaging of the spine. Chicago: Year Book Medical, 1989:244
