Aota Path. Jep. 27(1): 137-144, 1977
PRIMARY LEIOMYOSARCOMA ARISING IN THE TRUNK OF PULMONARY ARTERY
- A Case Report and Review of Literature Takashi HAYATAand Eiichi SATO The Secimd Department of Pdlwlogy, Faculty of Afedieine, Kagoshinuz University, Kagoshima (Received on May 14, 1976)
An autopsy case of a 46-year-old woman with primary leiomyosarcoma arisine in the pulmonary artery is presented. This case is the first description in Japan and the loth in the world literature as leiomyosarcoma in this part of the body. An electronmicroscopic observation of the postmortem material was useful to confirm the histoioeic diagnosis, in addition to some kinds of stainlnp method of histologic section. ACTA PATH. JAP. 27: 137-144,
1977.
Intro&.i&m Primary sarcoma of the trunk of the pulmonary artery is quite unusual; approximately 31 cues have been reported. They originate commonly from the inner layer of dorsal part of the pulmonary artery and form a polypoid tumor in the lumen, causing severe dyspnea. This report describes a case of leiomyosarcoma of the trunk of the pulmonary artery. Gtrossly, the tumor showed predominantly extraluminal growth between the aortic and pulmonary trunk and infiltrated all layers of the pulmonary artery, forming a polypoid tumorette in a cusp of the pulmonary valve. Microscopically, the tumor extended into the intrapulmonary branches of the pulmonary artery and caused multiple pulmonary infarcts. Case Report
A 46-year-old woman was admitted to the Kagoshima City Hospital on November 19, 1974, complaining of severe chest pain radiating to the left of the back and shoulder, malaise. and fever (37.237.8"C) for three months. On chest X-ray a tuberculous pericarditis and tuberculosis of the lung were suspected (Figs. 1, 2). Sixteen days after the admission when she got down the bed for urination, she suddenly fainted, fell into shock and died. Laboratory findings are shown in Table I. ~~
+EB
I@#
*-
The Second Department of Pathology, Faculty of Medicine, Kagoshime University, 1208-1, U d - o h o , Kagoehime, J a w 137
138
Ada Path. Jap.
LEIOMYOSARCOMA OF PULMONARY ARTERY
Table 1. Laboratory F b d h g 8
Date 11.20. '74
R.B.C. W.B.C. Hb Ht. Urine protein Blood preasure
12.3. '74
348 X lo4
SaO x lo4
QaoO
8Ooo 10.9
11.7
sa. a (-)
lM/M
91.8
(-1
108/70
I
Date 11.20. '74
~
Liver function 1.1. GOT GPT
a. 5 17 7 1.7
TTT Serum protein analysis
normal
L
Fig. 1
12.3. '74
-
Fig. 2
Autopsy Findings Autopsy 17 hours after death revealed a tumor located between the pulmonary and aortic trunk, adjacent to the left atrium and adventitia of the aorta, but it could be easily detached from them (Fig. 3). The tumor invaded all layers of the trunk of the pulmonary artery, slightly protruding into the lumen and formed a polyploid tumorette in the pulmonary posterior valve (Fig. 4). On cutting through the tumor, this showed a gray-white and partly necrotic surface with mixture of myxomatous appearance, especially in the central portion (Fig. 3). The margin of the tumor was apparently distinguished from the aorta and the atrium. The main branch of the right pulmonary artery was almost completely obstructed by the tumor. Consequently extensive hemorrhagic infarcts were caused in the middle and lower lobes of the right lung. Both lungs showed severe chronic passive congestion.
2711): 1977
T. HdYATA AND B. SAT0
139
Fig. 3. A tumor, 10x10 cm in size, was located between the pulmonary and aortic trunk, adjacent to the left atrium and adventitia of the aorta. Fig. 4. Tumorette, formed in the pulmonary posterior valve, 0 . 8 ~ 0 . 6cm in size.
Microswpic Findings The tumor was composed of a typical spindle shaped cells with round to oval nuclei which showed sometimes elongation and hyperchromasia (Fig. 5). The cytoplasm was rather eosinophilic. Mitotic figures were frequently observed. These tumor cells were arranged in a fascicular pattern, strands or sheets especially at the periphery of the tumor (Fig. 6). In the specimens taken from central portion, tumor cells frequently showed perinuclear vacuolation or vesiculation (Fig. 7). These vesicles were negative for PAS, alcian blue, mucicaramine and fat stain. The elmtica-Goldner stain revealed red cytoplasm, faint collagenous bundles and scarcity of elastic fibers. In the PTAH staining the cytoplasm was purple but cross striation was not demonstrated. The Gomori’s reticulin stain revealed fine silver network which surrounded the individual tumor cells. Sudan 111stain gave no fat granules in most of the tumor cells. Alcian blue stain was fairly positive in the stroma of the myxomatous area. These tumor cells invaded all layers of the pulmonary artery, destructing their elastic fibers (Fig. 8). In some major intrapulmonary branches of the right pulmonary artery the intima showed marked hyaline thickening and tumor cells were found in the inner layer of the intima (Fig. 9).
Eledrcmmicroswpic Findings Owing to the postmortem examination, the structual preservation of tumor cells in electronmicroscopy was not sufficient. However, in the cytoplasm of the tumor cells, micro-fibrillar structure with patchy condensation could be found in parallel
140
LEIOMYOSAWOM.4 OF PVLMONABY ARTERY
A& Path. Jap.
Fig. 6. Marked proliferation of cltypical spindle-shapd cells. H. & E., x 320. Fig. 6. Periphery of the tumor. The cells are arranged in a fascicular pattern, strands or sheets. H. & E. x 320. Fig. 7. Central portion of the tumor. Note perinuclear vecuolation of tumor cells. H. & E., x 320. Fig. 8. Deetruction of elastic fibers of pulmonary artery by tumor invasion. eleetica-Goldner,
x 69.
27(1): I977
T. HhYATA AND 19. SAT0
141
Fig. 9. Hyaline thickening and tumor cells (arrow) in intrapulmonary branchee of the right pulmonary artery. elmtica-Goldner, x 69. Fig. 10. Electron microscopy reveals microfibrillar structure with patchy condensation in the cytoplasm of tumor cells. x 12.200.
142
Acta Path. Jap.
LF4IOMYOSARCQMA OF PULMONARY hRTBRY
arrangement to the long axis of the cells (Fig. 10). These fibrils were made of two components, 60 A and 160 A in width. Collagenous bundles were present surrounding some of the cells. Basal lamina was not apparent. On the basis of the above findings the tumor was diagnosed as leiomyosarcoma.
Diswsion Primary leiomyosarcoma of the pulmonary artery is extremely rare. As shown in Table 2, approximately 31 cases of malignant tumors have been reported in the available literature. The diagnosis of leiomyosarcoma was found in only 9 cases and the present case was regarded as the 10th following them. We could not find a report of this type of tumor in the Japanese literatures. The only case reported by U M E H A R A ~ was benign hemangiomas located a t the surface of the left ventricle and of the pulmonary artery. Among tumors reported the involvement of pulmonary valve was found in Table 2. Primary flasareomn of the Pulmonary Artery ~
PT*
7. 8. 9. 10. 11.
1923 1928 1928 1936 1936 1939 1941 1942 1946 1961 1959
Mendeletsmmxo Fr~boeee~ Eechbech' Kudlich & Sohuh18 coedel* Martin et alal Haythorn e t all' V.d. Lindele -in & Ingleby4 Moegenxa Elphinetone & Spectors
12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 26. 26. 27. 28. 29. 30. 31. 32.
1980 1960 1960 1961 1962 1962 1964 1966 1966 1967 1968 1969 1969 1969 1969 1969 1970 1970 1972 1974 1976
Wolf et aP0 Jacques & Barclaylb Jacques & Barclaylb Hegstrtimll Chatelanata , K o v h et all7 Green e t allo Ali & Lee1 Munk et ala4 Oleby et a P Friedman & Smith' Wsckers et al*B Caha Gain* Cab* Nalbat et alas Hirachl4 MacOnnell*a Sethi*' Heyea et all* H a y 8 h & Sat0
1. 2. 3. 4. 5. 6.
*pT:
68M 64F 68M 27M 47M 46F 51F 23F 63F 70M 61M 63F MF 45M 69F 81M 56M 47M 36F
KOF 23M 63F 69M 72F 62F 32M 52F
K3F KOF 62F 46F
rs
--- ___
pindle cell sarcoma Fibmarcoma Leiomyosarcoma Leiomyossrcoma Angioplastic earcoma Polymorphic sarcoma Fibromyxoesrcoma Leimoyosercoma Rhabdomyosercoma Malignant mesenchymal mixed tumor Fibmarcoma Fibroearcoms Anaplastic sarcoma Malignant tumor Malignant megenchymoma Fibromyxosercoma Fibmrcoma Fibromyxosarcoma Leiomyoaercoma Malignant megenchymoma Primary melignant myoma Leiomyoearcoma Leiomyoe&rcoma Myxomatous giant cell sarcoma Polymorphic sarcoma Fibroearcoma Primary malignant tumor Primary sarcoma Osteoaercoma Sugg. Leiomyosarcoma Leiomyoarrrcoma Leiomyosarcoma
+ + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + +
pulmonary trunk. *PV: Pulmonary valves. *OFT: Outflow tract.
++ + +
++ + +
+
-
-
-
-
+-
--
-
-
f
+ +
-
-
+ + + + -- ++-
-
+ -
-
+-
+ + +
+
+ + f +-
+ +
-
+ +
+
+
-
f
+ +
-
f
+
f
-
-
- + - + + + +
+
+ + +
27(1): 1977
T. RAYATA AND E. SAT0
143
15 cases and metastasis in 17 cases (Table 2). Most of the tumors were discovered at autopsy, some cases were diagnosed antemortem and a few received operative treatment.14.16.22.2' Reviewing the description of tumors arising in large arteries and veins, K E V O R K I A N ~ ~ reported that the origin of the neoplasm was most frequent in large veins, such as inferior vena cava, followed by pulmonary artery and large systemic artery. Histologically the present tumor showed fascicular pattern of spindle cells with eovinophilic cytoplasm. But in some areas the tumor cells had round to oval hyperchromatic nuclei, perinuclear vacuoles in the cytoplasm and indistinct cell border. The latter findings may suggest a pattern of bizarre leiomyoblastoma. This histology, however, should be considered as secondary modification. The tumorette invading the pulmonary valve showed myxomatous change, not myxoma, for the fact that histologic findings including alcian blue stain revealed no apparent substance in the matrix.
References 1. ALI, M.Y. and LEE, G.S.: Sarcoma of the pulmonary artery. Cancer 17: 1220-1224, 1964. 2. CAIN,H.: Uber primlire Sarkome des Hauptatammea der Arteria pulmonalis. Dtsch. Med. Wochenschr. 94: 1807-1614, 1969. F.: Fibromyxoeercome dea valvulea pulmonaires et son extension dans lea 3. CHATELANAT, poumons. Pathol. Microbiol. 25: 366371, 1962. H.: Primary sarcoma of the pulmonary artery. Clinics 5: 1824. DURQIN,B. and INQLEBY, 189, 1946. 6. ELPEINSTONE, R.H. and SPIPCTOR, R.G.: Sarcoma of the pulmonary artery. Thorax 14: 333-340, 1969. H.: Ueber ein malignes Leiomyom dea Endocsrds mit Veretopfung der Lung6. ESCHBACH, enschlagader. Ziegl. Betir. Path. 80: 672-681, 1928. H.M. and SMITH,C.K.: Leiomyoesrcoma of the pulmonary artery. J.A.M.A. 7. FRIEDMAN, 203: 809, 1968. U. FROBOESE, C. : Emboliformea Sarkom dea Hauptatammea der Pulmonaearterie. Zentralbl. Allg. Pathol. 44: 148-163, 1928. 9. GOEDEL,A. : Zur Kenntnis des primliren Lungenschlagedersarkoms. Frankfurt. Z. Path. 49: 1-9, 1936. L.E. and SEANKLIN, D.R.: Fibromyxoeercoma of the pulmonary 10. GREEN,J.R. JR.,CREVASSE,
11. 12. 13. 14.
artery, associated with syncope, intractable heart failure, polycythemia and thrombocytopenia. Am. J. Cardiol. 13: 547662, 1964. HAGSTMM,L.: Malignant meaenchymoma in pulmonary artery and right ventricle. Acta Pathol. Microbiol. Scsnd. 51: 87-94, 1961. HAYES,W.L., FARHA, 8.5. and BROWN,R.L.: Primary leiomyosarcoma of the pulmonary artery. Am. J. Cardiol. 34: 616-617, 1974. HAYTHORN, S.R., RAY,W.B. and WOLFF,R.A.: Primary fibromyxosarcoma of the heart and the pulmonary artery. Am. J. Path. 17: 261-271, 1941. HIRSCH,A., CHRI~TIENE,J., RENAULT,P., GALEY,J.J., ROBILLARD, M., DUROUX,P., GAJDOS,Ph. and BROUET,G.: Sarcome primitif de I'artAre pulmonaire chez onc jeune femme. Mcidive rapide a p r b exhrhe. DBcb. J. Franc. Med. Chir. Thorac. 24: 147-168,
1970. 16. JACQUES, J.E. and BARCLAY, R.: The solid sarcomatous pulmonary artery. Brit. J. Dis. Chest 54: 217-220, 1980. 16. KEVORKIAN, J. and CENTO,D.P.: Leiomyoesrcoma of large arteries and veins. Surgery 73: 390-400, 1973. 17. KovAcs, K., ILLYSS,E., S Z A B ~K. , and WALLACEER, L: Primary sarcoma of the pulmonary artery. ORV. HETIL. 103 (841-844) 1962 cited by Excerpta Medica sect. 5
144
LBLOXYOBAWOMA OF PULMONIBY AE-Y
Acfo Path. Jap.
(1963) 16, 37-38. 18. KUDIJCH, H. and SCWH,W.: 19. 20. 21. 22. 23. 24. 26.
Ein Beitrag zum myoplaetischen Sarkom der Lungenschlagader. Virchowa Arch. Pathol. Anat. 294: 113-119, 1936. LINDE,v.d., H.M.: Sarooom van de longslagader. Ned. Tijdschr. Geneeakd. 86: 1049-10M), 1942. cited by Wackers, F.J. MANDELBTAMM, M.: Ueber primaere Neubildungen dea Henen. Virchowa Arch. Pathol. Anat. 245: 43-64, 1923. MAETIN, W.C.,TUOFKY, E.L. and WILL,C.: Primary tumor of the heart (Entrance of the pulmonary artery). Am. Heart J. 17: 728-734, 1939. MCICONNIDLL,T.H.: Bony and cartilagenoua tumors of the heart and great vessels. Report of an osteowcmme of the pulmonary artery. Cancer 25: 611-817, 1970. MOEGEN, P.: Ueber einen primiiren wrkornatdmn Tumor der Pulmonalarterie mit auagedehnten Metatesen in der reohten Lunge. Z. Kceisl. -Forsch. 40: 1W180, 1961. MUNK, J., GBWFEL,B. and KOGAN,J.: Primary menenchymoma of the pulmonary artery -Radiological featucea. Br. J. Radiol. 38: 104-111, 1986. NALBAT,A.S. and LYBKOVTBEVA, T.E.: A rare c888 of angiowrcoma of the pulmonary artery (Rueeian). Arkh. Pat. (Moek.) 31: 71-74,1869. cited by Excerpts Medica sect. 5 (19sQ)
22, 486 J., KULIO,A. and P o w , J.: Pierwotny miesniak zlosliwy tetnicy plucnej. Pol. 26. OLEKBY, Arch. Med. Wewn. 38: 663-668, 1967. G.K., SLAVEN,J.E., KEPIDB, J.J., PUOH,D. and THAL,A.P.: Primary sarcoma of 27. SETHI, the pulmonary artery. J. Thorac. Cardioveec. Surg. 63: 687493, 1972. Y.: Hemangiomee of the heart and pulmonary artery surface -A case report 28. UMEHAIU, Acts pathol. Jap. 9: 821-822, 1969. F.J., VAN DEBSOHOOT, J.B. and HAMPE,J.F.: Sarcoma of the pulmonary 29. WACKEBB, trunk m c i a t e d with hemorrhagic tendency. A w e report and review of the literature. Cancer 23: 339-361, 1060. R.C. and LANGBTON, J.D. : Fibrowrooma of the pulmonary artery, 30. Worn.R.L. DIOK~N?~AN, masqueradii an a pheochromooytoma. Amer. J. Clin. Path. 34: 148-164, 1960.
-.
PRIMARY LEIOMYOSARCOMA ARISING IN THE TRUNK OF PULMONARY ARTERY
- A Case Report and Review of Literature Takashi HAYATAand Eiichi SATO The Secimd Department of Pdlwlogy, Faculty of Afedieine, Kagoshinuz University, Kagoshima (Received on May 14, 1976)
An autopsy case of a 46-year-old woman with primary leiomyosarcoma arisine in the pulmonary artery is presented. This case is the first description in Japan and the loth in the world literature as leiomyosarcoma in this part of the body. An electronmicroscopic observation of the postmortem material was useful to confirm the histoioeic diagnosis, in addition to some kinds of stainlnp method of histologic section. ACTA PATH. JAP. 27: 137-144,
1977.
Intro&.i&m Primary sarcoma of the trunk of the pulmonary artery is quite unusual; approximately 31 cues have been reported. They originate commonly from the inner layer of dorsal part of the pulmonary artery and form a polypoid tumor in the lumen, causing severe dyspnea. This report describes a case of leiomyosarcoma of the trunk of the pulmonary artery. Gtrossly, the tumor showed predominantly extraluminal growth between the aortic and pulmonary trunk and infiltrated all layers of the pulmonary artery, forming a polypoid tumorette in a cusp of the pulmonary valve. Microscopically, the tumor extended into the intrapulmonary branches of the pulmonary artery and caused multiple pulmonary infarcts. Case Report
A 46-year-old woman was admitted to the Kagoshima City Hospital on November 19, 1974, complaining of severe chest pain radiating to the left of the back and shoulder, malaise. and fever (37.237.8"C) for three months. On chest X-ray a tuberculous pericarditis and tuberculosis of the lung were suspected (Figs. 1, 2). Sixteen days after the admission when she got down the bed for urination, she suddenly fainted, fell into shock and died. Laboratory findings are shown in Table I. ~~
+EB
I@#
*-
The Second Department of Pathology, Faculty of Medicine, Kagoshime University, 1208-1, U d - o h o , Kagoehime, J a w 137
138
Ada Path. Jap.
LEIOMYOSARCOMA OF PULMONARY ARTERY
Table 1. Laboratory F b d h g 8
Date 11.20. '74
R.B.C. W.B.C. Hb Ht. Urine protein Blood preasure
12.3. '74
348 X lo4
SaO x lo4
QaoO
8Ooo 10.9
11.7
sa. a (-)
lM/M
91.8
(-1
108/70
I
Date 11.20. '74
~
Liver function 1.1. GOT GPT
a. 5 17 7 1.7
TTT Serum protein analysis
normal
L
Fig. 1
12.3. '74
-
Fig. 2
Autopsy Findings Autopsy 17 hours after death revealed a tumor located between the pulmonary and aortic trunk, adjacent to the left atrium and adventitia of the aorta, but it could be easily detached from them (Fig. 3). The tumor invaded all layers of the trunk of the pulmonary artery, slightly protruding into the lumen and formed a polyploid tumorette in the pulmonary posterior valve (Fig. 4). On cutting through the tumor, this showed a gray-white and partly necrotic surface with mixture of myxomatous appearance, especially in the central portion (Fig. 3). The margin of the tumor was apparently distinguished from the aorta and the atrium. The main branch of the right pulmonary artery was almost completely obstructed by the tumor. Consequently extensive hemorrhagic infarcts were caused in the middle and lower lobes of the right lung. Both lungs showed severe chronic passive congestion.
2711): 1977
T. HdYATA AND B. SAT0
139
Fig. 3. A tumor, 10x10 cm in size, was located between the pulmonary and aortic trunk, adjacent to the left atrium and adventitia of the aorta. Fig. 4. Tumorette, formed in the pulmonary posterior valve, 0 . 8 ~ 0 . 6cm in size.
Microswpic Findings The tumor was composed of a typical spindle shaped cells with round to oval nuclei which showed sometimes elongation and hyperchromasia (Fig. 5). The cytoplasm was rather eosinophilic. Mitotic figures were frequently observed. These tumor cells were arranged in a fascicular pattern, strands or sheets especially at the periphery of the tumor (Fig. 6). In the specimens taken from central portion, tumor cells frequently showed perinuclear vacuolation or vesiculation (Fig. 7). These vesicles were negative for PAS, alcian blue, mucicaramine and fat stain. The elmtica-Goldner stain revealed red cytoplasm, faint collagenous bundles and scarcity of elastic fibers. In the PTAH staining the cytoplasm was purple but cross striation was not demonstrated. The Gomori’s reticulin stain revealed fine silver network which surrounded the individual tumor cells. Sudan 111stain gave no fat granules in most of the tumor cells. Alcian blue stain was fairly positive in the stroma of the myxomatous area. These tumor cells invaded all layers of the pulmonary artery, destructing their elastic fibers (Fig. 8). In some major intrapulmonary branches of the right pulmonary artery the intima showed marked hyaline thickening and tumor cells were found in the inner layer of the intima (Fig. 9).
Eledrcmmicroswpic Findings Owing to the postmortem examination, the structual preservation of tumor cells in electronmicroscopy was not sufficient. However, in the cytoplasm of the tumor cells, micro-fibrillar structure with patchy condensation could be found in parallel
140
LEIOMYOSAWOM.4 OF PVLMONABY ARTERY
A& Path. Jap.
Fig. 6. Marked proliferation of cltypical spindle-shapd cells. H. & E., x 320. Fig. 6. Periphery of the tumor. The cells are arranged in a fascicular pattern, strands or sheets. H. & E. x 320. Fig. 7. Central portion of the tumor. Note perinuclear vecuolation of tumor cells. H. & E., x 320. Fig. 8. Deetruction of elastic fibers of pulmonary artery by tumor invasion. eleetica-Goldner,
x 69.
27(1): I977
T. HhYATA AND 19. SAT0
141
Fig. 9. Hyaline thickening and tumor cells (arrow) in intrapulmonary branchee of the right pulmonary artery. elmtica-Goldner, x 69. Fig. 10. Electron microscopy reveals microfibrillar structure with patchy condensation in the cytoplasm of tumor cells. x 12.200.
142
Acta Path. Jap.
LF4IOMYOSARCQMA OF PULMONARY hRTBRY
arrangement to the long axis of the cells (Fig. 10). These fibrils were made of two components, 60 A and 160 A in width. Collagenous bundles were present surrounding some of the cells. Basal lamina was not apparent. On the basis of the above findings the tumor was diagnosed as leiomyosarcoma.
Diswsion Primary leiomyosarcoma of the pulmonary artery is extremely rare. As shown in Table 2, approximately 31 cases of malignant tumors have been reported in the available literature. The diagnosis of leiomyosarcoma was found in only 9 cases and the present case was regarded as the 10th following them. We could not find a report of this type of tumor in the Japanese literatures. The only case reported by U M E H A R A ~ was benign hemangiomas located a t the surface of the left ventricle and of the pulmonary artery. Among tumors reported the involvement of pulmonary valve was found in Table 2. Primary flasareomn of the Pulmonary Artery ~
PT*
7. 8. 9. 10. 11.
1923 1928 1928 1936 1936 1939 1941 1942 1946 1961 1959
Mendeletsmmxo Fr~boeee~ Eechbech' Kudlich & Sohuh18 coedel* Martin et alal Haythorn e t all' V.d. Lindele -in & Ingleby4 Moegenxa Elphinetone & Spectors
12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 26. 26. 27. 28. 29. 30. 31. 32.
1980 1960 1960 1961 1962 1962 1964 1966 1966 1967 1968 1969 1969 1969 1969 1969 1970 1970 1972 1974 1976
Wolf et aP0 Jacques & Barclaylb Jacques & Barclaylb Hegstrtimll Chatelanata , K o v h et all7 Green e t allo Ali & Lee1 Munk et ala4 Oleby et a P Friedman & Smith' Wsckers et al*B Caha Gain* Cab* Nalbat et alas Hirachl4 MacOnnell*a Sethi*' Heyea et all* H a y 8 h & Sat0
1. 2. 3. 4. 5. 6.
*pT:
68M 64F 68M 27M 47M 46F 51F 23F 63F 70M 61M 63F MF 45M 69F 81M 56M 47M 36F
KOF 23M 63F 69M 72F 62F 32M 52F
K3F KOF 62F 46F
rs
--- ___
pindle cell sarcoma Fibmarcoma Leiomyosarcoma Leiomyossrcoma Angioplastic earcoma Polymorphic sarcoma Fibromyxoesrcoma Leimoyosercoma Rhabdomyosercoma Malignant mesenchymal mixed tumor Fibmarcoma Fibroearcoms Anaplastic sarcoma Malignant tumor Malignant megenchymoma Fibromyxosercoma Fibmrcoma Fibromyxosarcoma Leiomyoaercoma Malignant megenchymoma Primary melignant myoma Leiomyoearcoma Leiomyoe&rcoma Myxomatous giant cell sarcoma Polymorphic sarcoma Fibroearcoma Primary malignant tumor Primary sarcoma Osteoaercoma Sugg. Leiomyosarcoma Leiomyoarrrcoma Leiomyosarcoma
+ + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + +
pulmonary trunk. *PV: Pulmonary valves. *OFT: Outflow tract.
++ + +
++ + +
+
-
-
-
-
+-
--
-
-
f
+ +
-
-
+ + + + -- ++-
-
+ -
-
+-
+ + +
+
+ + f +-
+ +
-
+ +
+
+
-
f
+ +
-
f
+
f
-
-
- + - + + + +
+
+ + +
27(1): 1977
T. RAYATA AND E. SAT0
143
15 cases and metastasis in 17 cases (Table 2). Most of the tumors were discovered at autopsy, some cases were diagnosed antemortem and a few received operative treatment.14.16.22.2' Reviewing the description of tumors arising in large arteries and veins, K E V O R K I A N ~ ~ reported that the origin of the neoplasm was most frequent in large veins, such as inferior vena cava, followed by pulmonary artery and large systemic artery. Histologically the present tumor showed fascicular pattern of spindle cells with eovinophilic cytoplasm. But in some areas the tumor cells had round to oval hyperchromatic nuclei, perinuclear vacuoles in the cytoplasm and indistinct cell border. The latter findings may suggest a pattern of bizarre leiomyoblastoma. This histology, however, should be considered as secondary modification. The tumorette invading the pulmonary valve showed myxomatous change, not myxoma, for the fact that histologic findings including alcian blue stain revealed no apparent substance in the matrix.
References 1. ALI, M.Y. and LEE, G.S.: Sarcoma of the pulmonary artery. Cancer 17: 1220-1224, 1964. 2. CAIN,H.: Uber primlire Sarkome des Hauptatammea der Arteria pulmonalis. Dtsch. Med. Wochenschr. 94: 1807-1614, 1969. F.: Fibromyxoeercome dea valvulea pulmonaires et son extension dans lea 3. CHATELANAT, poumons. Pathol. Microbiol. 25: 366371, 1962. H.: Primary sarcoma of the pulmonary artery. Clinics 5: 1824. DURQIN,B. and INQLEBY, 189, 1946. 6. ELPEINSTONE, R.H. and SPIPCTOR, R.G.: Sarcoma of the pulmonary artery. Thorax 14: 333-340, 1969. H.: Ueber ein malignes Leiomyom dea Endocsrds mit Veretopfung der Lung6. ESCHBACH, enschlagader. Ziegl. Betir. Path. 80: 672-681, 1928. H.M. and SMITH,C.K.: Leiomyoesrcoma of the pulmonary artery. J.A.M.A. 7. FRIEDMAN, 203: 809, 1968. U. FROBOESE, C. : Emboliformea Sarkom dea Hauptatammea der Pulmonaearterie. Zentralbl. Allg. Pathol. 44: 148-163, 1928. 9. GOEDEL,A. : Zur Kenntnis des primliren Lungenschlagedersarkoms. Frankfurt. Z. Path. 49: 1-9, 1936. L.E. and SEANKLIN, D.R.: Fibromyxoeercoma of the pulmonary 10. GREEN,J.R. JR.,CREVASSE,
11. 12. 13. 14.
artery, associated with syncope, intractable heart failure, polycythemia and thrombocytopenia. Am. J. Cardiol. 13: 547662, 1964. HAGSTMM,L.: Malignant meaenchymoma in pulmonary artery and right ventricle. Acta Pathol. Microbiol. Scsnd. 51: 87-94, 1961. HAYES,W.L., FARHA, 8.5. and BROWN,R.L.: Primary leiomyosarcoma of the pulmonary artery. Am. J. Cardiol. 34: 616-617, 1974. HAYTHORN, S.R., RAY,W.B. and WOLFF,R.A.: Primary fibromyxosarcoma of the heart and the pulmonary artery. Am. J. Path. 17: 261-271, 1941. HIRSCH,A., CHRI~TIENE,J., RENAULT,P., GALEY,J.J., ROBILLARD, M., DUROUX,P., GAJDOS,Ph. and BROUET,G.: Sarcome primitif de I'artAre pulmonaire chez onc jeune femme. Mcidive rapide a p r b exhrhe. DBcb. J. Franc. Med. Chir. Thorac. 24: 147-168,
1970. 16. JACQUES, J.E. and BARCLAY, R.: The solid sarcomatous pulmonary artery. Brit. J. Dis. Chest 54: 217-220, 1980. 16. KEVORKIAN, J. and CENTO,D.P.: Leiomyoesrcoma of large arteries and veins. Surgery 73: 390-400, 1973. 17. KovAcs, K., ILLYSS,E., S Z A B ~K. , and WALLACEER, L: Primary sarcoma of the pulmonary artery. ORV. HETIL. 103 (841-844) 1962 cited by Excerpta Medica sect. 5
144
LBLOXYOBAWOMA OF PULMONIBY AE-Y
Acfo Path. Jap.
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