CHONDROSARCOMA OF THE NASAL SEPTUM EXTENDING INTO THE MAXILLARY SINUS SEAN
B.
635
PEPPARD, MD
BY INVIT"liON
GREGORY J. MATZ, MD CHICAGO, IlliNOIS
A case of chondrosarcoma of the nasal septum extending Into the maxillary sinus Is presented. These cartilaginous tumors are rare in the head and neck, with only 21 reported In the maxilla and four In the nasal septum. Careful histologic analysis Is required for correct diagnosis. The present case was treated with an en bloc ethmoidectomy and medial maxillectomy, followed by fast neutron radiation therapy. The therapeutic program is analyzed for opportunity to provide an excellent chance of cure.
CHONDROSARCOMA is rare in the facial bones but has been reported in the ethmoidosphenoid sinus and maxillary antrum.' A patient with chondrosarcoma arising in the nasal septum is presented, and the unusual location and atypical histologic appearance of the tumor are discussed. A unique therapeutic program of fast neutron therapy in addition to medial maxillectomy is reported.
CASE REPORT
A 33-year-old white man noted an increasing degree of nasal obstruction and multiple minor nosebleeds in December 1976. He consulted an otolaryngologist, who performed a
Submitted for publication Sept 12. 1978. From the Department of Surgery (Otolaryngology), University of Chicago. Presented in combination with the Committee on Surgery of the Head and Neck at the 1978 Annual Meeting of the American Academy of Otolaryngology, las Vegas. Sept 10-13. Reprint requests to Department of Otolaryngology, Wayne State University, S40 E Canfield, University Clinics Building. Detroit, MI 48201 (Dr Peppard).
septoplasty in February 1977 that did not relieve the nasal breathing difficulties. The patient also began to have an increasing amount of facial pain. Roentgenograms in late May disclosed an intranasal and left maxillary sinus mass, and the patient was referred to the University of Chicago Otolaryngology Clinic for further evaluation in early July. Examination disclosed 'that both sides of the nose were completely closed with a mucosalcovered mass; the sinuses failed to transilluminate. The left cheek was swollen, mildly erythematous, and tender. Oral, pharyngeal, and neck examinations were all normal. Ophthalmologic inspection showed full range of motion, pupillary reactivity, and normal visual acuity. Tomograms of the sinus revealed a mass destroying the septum and left medial wall of the maxillary sinus (Fig 1). The patient had a left Caldwell-luc procedure and biopsy. The sinus was filled with purulent material, and an extremely vascular tumor was causing destruction of the nasal wall. Pathologic examination showed a cartilaginous tumor that mimicked a chondroblastoma in appearance but proved to be a chondrosarcoma. The diagnosis was based on areas of nuclear atypia, occasional mitotic figures, and an angioinvasive character (Fig 2). Two weeks later, the patient had an en bloc ethmoidectomy, posterior septectomy, and medial maxillectomy (Fig 3). The tumor was well encapsulated and extended from the septum to the left ethmoid and maxillary sinus. The tumor did not invade the orbit, cribriform plate, or palatal tissues. Six weeks after uneventful healing, the patient received fast neutron radiation for a total dose of 2,100 neutron rads. The procedures were well tolerated, and to date, there is no recurrence of disease. A CAT scan of the orbital and sinus area at ten months after surgical treatment demonstrates a surgical defect but no evidence of tumor (Fig 4). The patient is presently 14 months postsurgery and works with minimal functional or cosmetic deformity.
Otolaryngol Head Neck Surg 87:635-639 (Sept-Oct) 1979
Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 9, 2016
636
PEPPARD AND MATl
Fig 1 .-Tomogram~ show ing destruction by tumor of medial wall of maxillary sinus .
DISCUSSION Chondrosarcomas of the maxilla are rare tumors . The modern literature shows only 25 reported cases.t -n of which only four are bel ieved to have arisen from the nasal septum .2•5- 7 Clarification of chondrosarcoma as a distinct neoplasm was first made by Ewing,l1 who separated the cartilaginous from the osteogenic bone tumors. Lichtenstein and Jaffe lJ indicated that chondrosarcoma is often underdiagnosed and that some cases in the past must have been confused with benign lesions leading to faulty diagnosis and therapy. Lichtenstein and Jaffe 1 ) listed criteria for malignancy in a cartilaginous tumor as follows: (1) calcificat ion ; (2) many cells w ith plump nuclei or occasional cells with two nuclei; and (3) giant cells with clumped chromatin or either single or multiple nuclei. Batsakisv stated that chondrosarcomas, as opposed to benign chondromas and chondroblastornas, are richly cellular and manifest both cellular and nuclear irregularity. He added that cell division is amitotic, and thus, the scarcity or absence of mitotic figures does
not preclude a diagnosis of mal ignancy. The present case did show nuclear atypia, cellular irregularity, and occasional mitotic figures, and because chondroblastoma has never been noted in the maxilla , it was believed on histologic and anatomic grounds that the patient did have a chon drosarcoma, an important point in the plann ing of definitive therapy . Most authorities agree that wide surgical resection is necessary for control of chondrosarcoma .i' Batsakis and Dit0 1 have recommended a radical ex c ision of surrounding normal bone and soft tissues. The procedures of en bloc ethmoidectomy and med ial maxillectomy have been advocated by Sessions and Larson,' >since they sufficiently exenterate disease wh ile avoiding a larger mut ilating procedure on the one hand and a piecemeal removal on the other. The localized nature of the tumor allows complete removal with sparing of both orb its. To increase the chances for survival, the patient received postoperative radiation therapy with fast neutrons. Many authors believe that radiation is of no value.t > although others have noted oc-
Otolaryngol Head Neck Surg 87:635-639 (Sept-Oct) 7979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 9, 2016
637
CHONDROSARCOMA OF NASAL SEPTUM
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Fig 2.-Photomicrographs showing variable morphology of tumor in two different fields (hematoxylin-eosin,
x400l.
Oto/aryngol Head Neck Surg 87:635-639 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 9, 2016
638
PEPPARD AND MAll
Fig 3.-Tumor location and extent of resection (dotted lines) .
FiK 4.-CAT scans showing orbit free of disease lIeff) and surgical defect (right).
Otolaryngol Head Neck Surg 87:635-639 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 9, 2016
CHONDROSARCOMA OF NASAL SEPTUM casional responses of this tumor to conventional radiation therapy.16-18 Recent success with fast neutron radiation against a variety of tumors" encouraged its use against chondrosarcoma. Because of a greater tu mor penetration and destruction, neutron therapy may provide better control than conventional cobalt radiation in this rare head and neck tumor. The use of limited surgical treatment in addition to the neutron therapy may accomplish cure without destruction of vital structures.
639
8. Lapidot A, Ramm C, Fani K: Chondrosarcoma of the maxilla. J Laryngol Otol 80:743747, 1966. 9. Grewal BS, Nirola A, Lumba SP: Chondrosarcoma of the maxilla. J Laryngol Oto/86:741745, 1972. 10. Jones HM: Cartilaginous tumors of the head and neck. J Laryngol Otol 87:135-151, 1973. 11. Wolfowitz BL: Osteosarcoma and chondrosarcoma of the maxilla. J Laryngol Otol 87:409-415, 1973. 12. Ewing JA: A review of the classification of bone tumors. Surg Gynecol Obstet 68:971976, 1939. 13. lichtenstein L, laffe HL: Chondrosarcoma of bone. Am J Patho/19:553-589, 1943.
REFERENCES
1. Batsakis JG, Dito WR: Chondrosarcoma of the maxilla. Arch Oto/aryngol 75:69-75, 1962. 2. Lawson LJ: Intranasal chondrosarcoma. Arch Oto/aryngol 55:559-565, 1952.
3. Soboroff BJ, Lederer FL: Chondrosarcoma of the nasal cavity. Ann 0101 Rhinol Laryngol 64:718-727, 1955. 4. Sandler HC: Chondrosarcoma of the maxilla. Oral Surg 10:97-103, 1957. 5. Kragh LV, Dahlin DC, Erich IB: Cartilaginous tumors of the jaws and facial regions. Am J Surg 99:852-856, 1960.
14. Batsakis J: Tumors of the Head and Neck. Baltimore, Williams & Wilkins Co, 1974, pp 291293. 15. Sessions RB, Larson DL: En bloc ethmoidectomy and medial maxilleetomy. Arch Oto/aryngol 103:195-202, 1977. 16. Harmer WD: Treatment of malignant disease in the upper jaw. Lancet 1:129-133 1935. ' 17. Miles AEW: Chondrosarcoma of the maxilla. 8r Dent J 88:257-269, 1950.
Laryngol Otol 79:69-72, 1965.
18. lott 5, Bordley JE: A radiosensitive chondrosarcoma of the sphenoid sinus and base of skull. Report of a case. Laryngoscope 82:57-60 1972. '
7. Aretsky PI, Kantu K, Freund HR, et al: Chondrosarcoma of the nasal septum. Ann 0101 Rhinol Laryngol 79:382-387, 1970.
19. Catterall M, Von berg DO: Treatment of advanced tumors of head and neck with fast neutrons. 8r Med J 3:137-143, 1974.
6. Coyas AJ: Chondrosarcoma of the nose. J
Oto/aryngol Head Neck Surg 87:635-639 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 9, 2016
B.
635
PEPPARD, MD
BY INVIT"liON
GREGORY J. MATZ, MD CHICAGO, IlliNOIS
A case of chondrosarcoma of the nasal septum extending Into the maxillary sinus Is presented. These cartilaginous tumors are rare in the head and neck, with only 21 reported In the maxilla and four In the nasal septum. Careful histologic analysis Is required for correct diagnosis. The present case was treated with an en bloc ethmoidectomy and medial maxillectomy, followed by fast neutron radiation therapy. The therapeutic program is analyzed for opportunity to provide an excellent chance of cure.
CHONDROSARCOMA is rare in the facial bones but has been reported in the ethmoidosphenoid sinus and maxillary antrum.' A patient with chondrosarcoma arising in the nasal septum is presented, and the unusual location and atypical histologic appearance of the tumor are discussed. A unique therapeutic program of fast neutron therapy in addition to medial maxillectomy is reported.
CASE REPORT
A 33-year-old white man noted an increasing degree of nasal obstruction and multiple minor nosebleeds in December 1976. He consulted an otolaryngologist, who performed a
Submitted for publication Sept 12. 1978. From the Department of Surgery (Otolaryngology), University of Chicago. Presented in combination with the Committee on Surgery of the Head and Neck at the 1978 Annual Meeting of the American Academy of Otolaryngology, las Vegas. Sept 10-13. Reprint requests to Department of Otolaryngology, Wayne State University, S40 E Canfield, University Clinics Building. Detroit, MI 48201 (Dr Peppard).
septoplasty in February 1977 that did not relieve the nasal breathing difficulties. The patient also began to have an increasing amount of facial pain. Roentgenograms in late May disclosed an intranasal and left maxillary sinus mass, and the patient was referred to the University of Chicago Otolaryngology Clinic for further evaluation in early July. Examination disclosed 'that both sides of the nose were completely closed with a mucosalcovered mass; the sinuses failed to transilluminate. The left cheek was swollen, mildly erythematous, and tender. Oral, pharyngeal, and neck examinations were all normal. Ophthalmologic inspection showed full range of motion, pupillary reactivity, and normal visual acuity. Tomograms of the sinus revealed a mass destroying the septum and left medial wall of the maxillary sinus (Fig 1). The patient had a left Caldwell-luc procedure and biopsy. The sinus was filled with purulent material, and an extremely vascular tumor was causing destruction of the nasal wall. Pathologic examination showed a cartilaginous tumor that mimicked a chondroblastoma in appearance but proved to be a chondrosarcoma. The diagnosis was based on areas of nuclear atypia, occasional mitotic figures, and an angioinvasive character (Fig 2). Two weeks later, the patient had an en bloc ethmoidectomy, posterior septectomy, and medial maxillectomy (Fig 3). The tumor was well encapsulated and extended from the septum to the left ethmoid and maxillary sinus. The tumor did not invade the orbit, cribriform plate, or palatal tissues. Six weeks after uneventful healing, the patient received fast neutron radiation for a total dose of 2,100 neutron rads. The procedures were well tolerated, and to date, there is no recurrence of disease. A CAT scan of the orbital and sinus area at ten months after surgical treatment demonstrates a surgical defect but no evidence of tumor (Fig 4). The patient is presently 14 months postsurgery and works with minimal functional or cosmetic deformity.
Otolaryngol Head Neck Surg 87:635-639 (Sept-Oct) 1979
Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 9, 2016
636
PEPPARD AND MATl
Fig 1 .-Tomogram~ show ing destruction by tumor of medial wall of maxillary sinus .
DISCUSSION Chondrosarcomas of the maxilla are rare tumors . The modern literature shows only 25 reported cases.t -n of which only four are bel ieved to have arisen from the nasal septum .2•5- 7 Clarification of chondrosarcoma as a distinct neoplasm was first made by Ewing,l1 who separated the cartilaginous from the osteogenic bone tumors. Lichtenstein and Jaffe lJ indicated that chondrosarcoma is often underdiagnosed and that some cases in the past must have been confused with benign lesions leading to faulty diagnosis and therapy. Lichtenstein and Jaffe 1 ) listed criteria for malignancy in a cartilaginous tumor as follows: (1) calcificat ion ; (2) many cells w ith plump nuclei or occasional cells with two nuclei; and (3) giant cells with clumped chromatin or either single or multiple nuclei. Batsakisv stated that chondrosarcomas, as opposed to benign chondromas and chondroblastornas, are richly cellular and manifest both cellular and nuclear irregularity. He added that cell division is amitotic, and thus, the scarcity or absence of mitotic figures does
not preclude a diagnosis of mal ignancy. The present case did show nuclear atypia, cellular irregularity, and occasional mitotic figures, and because chondroblastoma has never been noted in the maxilla , it was believed on histologic and anatomic grounds that the patient did have a chon drosarcoma, an important point in the plann ing of definitive therapy . Most authorities agree that wide surgical resection is necessary for control of chondrosarcoma .i' Batsakis and Dit0 1 have recommended a radical ex c ision of surrounding normal bone and soft tissues. The procedures of en bloc ethmoidectomy and med ial maxillectomy have been advocated by Sessions and Larson,' >since they sufficiently exenterate disease wh ile avoiding a larger mut ilating procedure on the one hand and a piecemeal removal on the other. The localized nature of the tumor allows complete removal with sparing of both orb its. To increase the chances for survival, the patient received postoperative radiation therapy with fast neutrons. Many authors believe that radiation is of no value.t > although others have noted oc-
Otolaryngol Head Neck Surg 87:635-639 (Sept-Oct) 7979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 9, 2016
637
CHONDROSARCOMA OF NASAL SEPTUM
I
f
." '.
r
,
. o
,
.. •
.•
-
••
,.
, ,.
'. .........
co
. . ..;.. ,~
•
.."
~
,,,
.
, "o
",
.I
r
'.
Fig 2.-Photomicrographs showing variable morphology of tumor in two different fields (hematoxylin-eosin,
x400l.
Oto/aryngol Head Neck Surg 87:635-639 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 9, 2016
638
PEPPARD AND MAll
Fig 3.-Tumor location and extent of resection (dotted lines) .
FiK 4.-CAT scans showing orbit free of disease lIeff) and surgical defect (right).
Otolaryngol Head Neck Surg 87:635-639 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 9, 2016
CHONDROSARCOMA OF NASAL SEPTUM casional responses of this tumor to conventional radiation therapy.16-18 Recent success with fast neutron radiation against a variety of tumors" encouraged its use against chondrosarcoma. Because of a greater tu mor penetration and destruction, neutron therapy may provide better control than conventional cobalt radiation in this rare head and neck tumor. The use of limited surgical treatment in addition to the neutron therapy may accomplish cure without destruction of vital structures.
639
8. Lapidot A, Ramm C, Fani K: Chondrosarcoma of the maxilla. J Laryngol Otol 80:743747, 1966. 9. Grewal BS, Nirola A, Lumba SP: Chondrosarcoma of the maxilla. J Laryngol Oto/86:741745, 1972. 10. Jones HM: Cartilaginous tumors of the head and neck. J Laryngol Otol 87:135-151, 1973. 11. Wolfowitz BL: Osteosarcoma and chondrosarcoma of the maxilla. J Laryngol Otol 87:409-415, 1973. 12. Ewing JA: A review of the classification of bone tumors. Surg Gynecol Obstet 68:971976, 1939. 13. lichtenstein L, laffe HL: Chondrosarcoma of bone. Am J Patho/19:553-589, 1943.
REFERENCES
1. Batsakis JG, Dito WR: Chondrosarcoma of the maxilla. Arch Oto/aryngol 75:69-75, 1962. 2. Lawson LJ: Intranasal chondrosarcoma. Arch Oto/aryngol 55:559-565, 1952.
3. Soboroff BJ, Lederer FL: Chondrosarcoma of the nasal cavity. Ann 0101 Rhinol Laryngol 64:718-727, 1955. 4. Sandler HC: Chondrosarcoma of the maxilla. Oral Surg 10:97-103, 1957. 5. Kragh LV, Dahlin DC, Erich IB: Cartilaginous tumors of the jaws and facial regions. Am J Surg 99:852-856, 1960.
14. Batsakis J: Tumors of the Head and Neck. Baltimore, Williams & Wilkins Co, 1974, pp 291293. 15. Sessions RB, Larson DL: En bloc ethmoidectomy and medial maxilleetomy. Arch Oto/aryngol 103:195-202, 1977. 16. Harmer WD: Treatment of malignant disease in the upper jaw. Lancet 1:129-133 1935. ' 17. Miles AEW: Chondrosarcoma of the maxilla. 8r Dent J 88:257-269, 1950.
Laryngol Otol 79:69-72, 1965.
18. lott 5, Bordley JE: A radiosensitive chondrosarcoma of the sphenoid sinus and base of skull. Report of a case. Laryngoscope 82:57-60 1972. '
7. Aretsky PI, Kantu K, Freund HR, et al: Chondrosarcoma of the nasal septum. Ann 0101 Rhinol Laryngol 79:382-387, 1970.
19. Catterall M, Von berg DO: Treatment of advanced tumors of head and neck with fast neutrons. 8r Med J 3:137-143, 1974.
6. Coyas AJ: Chondrosarcoma of the nose. J
Oto/aryngol Head Neck Surg 87:635-639 (Sept-Oct) 1979 Downloaded from oto.sagepub.com at NANYANG TECH UNIV LIBRARY on June 9, 2016
