Maxillary Chondrosarcoma Myers, MD, Stanley E. Thawley,

Ernest M.

is a malignant tuthat arises from cartilage. In the maxillary area, the tumors spread locally but may also invade blood vessels and metastasize systemically. Initial surgical resection is the treatment of choice. \s=b\ Chondrosarcoma

mor

(Arch Otolaryngol 105:116-118, 1979)

malignant tu¬ mor of connective tissue origin, arises from cartilage. Although it usually occurs in the long bones and ribs, 10% arise in the maxillofacial area, most commonly in the ethmoid and sphenoid sinuses and larynx.1 A

Chondrosarcoma,

a

male-to-female ratio of 10:1 has been reported with an average age of 50 years.

Kragh et al2 cited ten cases from the Mayo Clinic, Rochester, Minn, from

1907 to 1957. Batsakis and Dito' the literature of maxillary chrondrosarcoma. In the series of Kragh et al,2 80% arose from nasal structures (turbinâtes, septum), with only 20% found in the maxilla. They suggested maxillary involvement oc¬ curred by spread from the nasal cavi¬ ty. In 1974, Fu and Perzin1 found a total of 25 well-documented cases of chondrosarcoma in the literature that involved the nasal cavity, paranasal

reported and reviewed concerning 15 cases

sinuses,

or

nasopharynx.

To our knowledge, with the excep¬ tion of these articles, little has

appeared concerning chondrosarcoma, and nothing has appeared in the ear, Accepted

for publication July From the Department of

27,

1978.

Otolaryngology,

Washington University School of Medicine, St Louis.

Reprint requests to Department of Otolaryngology, Washington University School of Medicine, 517 S Euclid Ave, St Louis, MO 63110 (Dr Myers).

MD

nose, and throat

(ENT) literature.

surgical treatment is the exception, and most cases are inopera¬ ble. The following are two cases of Radical

maxillofacial

chondrosarcoma that diagnosed and surgically treated at Washington University School of Medicine, St Louis, by the Depart¬ ment of Otolaryngology. were

REPORT OF CASES Case 1.—A 45-year-old man had a leftsided nasal obstruction of ten years' dura¬ tion. Ear, nose and throat examination disclosed a mass in the left lateral nasal wall that extended to the septum and partially occluded the right nasal fossa. Multiple biopsy specimens showed acute and chronic inflammation, squamous meta¬ plasia, and granulomatous hyperplasia. Sinus x-ray films and tomograms showed a left anterior nasal mass that involved the lateral nasal wall, maxilla, and anterior ethmoid sinus (Fig 1 and 2). Erosion of the lateral nasal wall was demonstrated. Despite normal results of biopsies, the tumor was resected by performing a left vertical maxillectomy and a subtotal septectomy. Histopathologic examination showed a well-differentiated chondrosarco¬ ma (Fig 3 and 4). The surgical margins were clear. The patient has been followed up for two years without a recurrence. Case 2.-A 59-year-old man had a long history of chronic sinus drainage. The ENT examination disclosed a left nasal mass. Sinus films and tomograms showed a cloudy left maxillary sinus with question¬ able destruction of the medial wall and a bulging mass in the floor (Fig 5). A diag¬ nostic Caldwell-Luc procedure demon¬ strated a chondrosarcoma on the left side. One week later, a left total maxillectomy, ethmoidectomy, and excision of the poste¬ rior nasal septum and pterygoid plates was performed. The orbital floor was explored and found to have a defect posteromedially

without evidence of gross tumor. Examina¬ tion of the lateral wall of the maxillary sinus showed bone loss but no tumor. There was extensive tumor involvement of the entire left posterior ethmoids sinuses, as well as the posterior nasal fossa, with invasion of the nasal septum. Tumor was found to extend to the pterygoid plate

region. Pathologic examination of the specimens disclosed

chondrosarcoma in the maxil¬ and sphenoid sinuses. Focal areas of bone invasion were seen. The patient has been followed up for three years without evidence of recurrence. a

lary, ethmoid,

COMMENT

Chondrosarcomas are classified into two main types: primary and second¬

Primary chondrosarcomas arise from undifferentiated perichondrial cells.7"1 Usually, these neoplasms occur in younger patients; these are vascu¬ lar, extend to veins, and metastasize early. Secondary chondrosarcomas arise from altered cells either in a central chondroma or cartilaginous exostoses, and rarely in a patient with multiple osteochondromas. These oc¬ cur in older patients from 30 to 50 years of age. A third type, recently defined, is mesenchymal chondrosar¬ coma.71' It seems to arise from primi¬ tive mesenchymal cells, occurs pre¬ dominantly in women, is found largely in soft tissue, and rarely occurs in the head and neck. Lichenstein and Jaffe's1" criteria for the diagnosis of primary and secondary chondrosarcoma are (1) development from mature cartilage, (2) derivation from sarcomatous stro¬ ma (never osteoid tissue), and (3) cellular and nuclear irregularity. De¬ spite these seemingly clear-cut crite¬ ria, no sharp distinction can be drawn ary.

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between chondromas and secondary chondrosarcomas. ' ' Microscopically, the cartilage cells are grossly atypical (Fig 3 and 4). They are larger, irregu¬ larly arranged, and have large vac¬ uoles (Fig 4). The nuclei are plump, hyperchromatic, and occasionally multinucleated with visible mitosis (Fig 4). The intercellular substance is poor in fibers and variable in amount. Despite what seems to be clear-cut criteria, Dahlin and Henderson11 em¬ phasized that a chondrosarcoma is often underdiagnosed. Differentia¬ tion between the benign and malig¬ nant forms may be difficult. The distinction is largely based on clinical behavior, particularly progressive lo¬ cal destruction, invasion, and metastases.

Although

tumors is

the

etiology of these

unknown, two theories have been proposed111: (1) a malignant

neoplasm that arises in tissue that is known to form cartilage, and (2) a malignant neoplasm that arises in bone and preceded by a cartilage model. Jones1 suggested that these tumors are remnants of the embryo¬ nal cartilaginous skeleton that es¬ caped résorption during endochondral ossification. In the skull, the sphenoid, ethmoids, and nasal capsule pass through a cartilaginous phase. The primitive mesenchymal cell is believed to be the precursor of the chondroblast. These cells have the potential to ultimately form bone or cartilage. This may explain cartilage and bone tumors that form in tissues, such as dura, falx, or even membranous bones like the maxilla. Since the diagnosis of chondrosarco¬ ma is not always easy, Dahlin and Henderson11 and Batsakis and Dito'

Fig 1.—Sinus

x-ray film

demonstrating

chondrosarcoma in left nasal

cavity.

therapeutic aggressive¬ emphasize that the pathologic diagnosis of a chondro¬

encourage ness.

These authors

in a facial tumor dictates radical treatment. This philosophy greatly ma

influenced management of our re¬ ported cases. Although there have been isolated reports of favorable response to radiation therapy,12 Kragh et al2 in the Mayo Clinic series concluded that radical en bloc surgery is the treatment of choice. This is not always easy, particularly in the maxil¬ lofacial area. The intricate anatomy

Fig 2.—Sinus x-ray film demonstrating chondrosarcoma in left nasal fossa and maxillary wall. Note posterior extent of tumor and incidental ocular prosthesis.

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medial

Fig 3.—Chondrosarcoma showing intercellular substance with few fibers and atypical cartilage cells (hematoxylin-eosin, X150).

Fig 4.—Chondrosarcoma showing large vacuoles and multinucleated hyperchromatic nuclei (hematoxylin-eosin, 350). with destruction of the base of the skull. Recurrences arose only in patients with histologie or clinical

positive surgical margins. Careful followup is mandatory. Five-year survival does not apply to these patients. This is an insufficient period of time. The patients must be followed up for

a

lifetime.

References

Fig 5.—Tomogram demonstrating

mass

may preclude complete eradication with a negative margin. If a conserva¬ tive course is adopted, one exposes the patient to recurrence. The tumor may spread beyond resectability, invade the blood vessels, and metastasize to the lungs. Maxillofacial chondrosarco¬ ma requires radical surgery. The prognosis of patients with chondrosarcoma depends on the fol-

in left ethmoid and medial

lowing factors: (1)

maxillary

areas.

site and extent of the tumor, (2) tumor differentiation, and (3) adequate surgical margins. Tumors in surgically difficult areas, such as the nasopharynx, posterior nasal cavity, and sphenoid sinuses, have a poor prognosis. Case 2 probably fits in this category. In the series by Fu and Perzin,4 the four patients who died had uncontrolled local growth

1. Jones HM: Cartilaginous tumors of the head and neck. J Laryngol Otol 87:135-151, 1973. 2. Kragh LV, Dahlin DC, Erich JB: Cartilaginous tumors of the jaws and facial regions. Am J Surg 99:852-856, 1960. 3. Batsakis JG, Dito WR: Chondrosarcoma of the maxilla. Arch Otolaryngol 75:55-61, 1962. 4. Fu Y, Perzin KH: Non-epithelial tumors of the nasal cavity, paranasal sinuses and nasopharynx: A clinicopathological study. Cancer 34:453\x=req-\ 463, 1974. 5. Soboroff BJ, Lederer FL: Chondrosarcoma of the nasal cavity. Ann Otol Rhinol Laryngol 64:718-727, 1955. 6. Lawson LJ: Intranasal chondrosarcoma: Report of a case. Arch Otolaryngol 55:559-565, 1952. 7. Lichtenstein L, Bernstein D: Unusual benign and malignant chondroid tumors of the bone: A survey of some mesenchymal cartilage tumors and malignant chondroblastic tumors, including a few multicentric ones, as well as many atypical benign chondroblastomas and chondromyoid fibromas. Cancer 12:1142-1157, 1959. 8. Dahlin DC, Henderson ED: Mesenchymal chondrosarcoma: Further observations on a new entity. Cancer 15:410-417, 1962. 9. Salvador AH, Beabout JW, Dahlin DC: Mesenchymal chondrosarcoma: Observations on 30 new cases. Cancer 28:605-615, 1971. 10. Lichtenstein L, Jaffe HI: Chondrosarcoma of bone. Am J Pathol 19:553-589, 1942. 11. Dahlin DC, Henderson ED: Chondrosarcoma, a surgical and pathological problem. Bone Joint Surg Br 38A:1025-1038, 1956. 12. Paddison GM, Hanks GE: Chondrosarcoma of the maxilla: Report of a case responding to supervoltage irradiation and review of the literature. Cancer 28:616-619, 1971.

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J

Maxillary chondrosarcoma.

Maxillary Chondrosarcoma Myers, MD, Stanley E. Thawley, Ernest M. is a malignant tuthat arises from cartilage. In the maxillary area, the tumors spr...
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