Skeletal Radiol (1991) 20:299-301
Case report 670" F. Martinez-Madrigal, M.D. 1, D. Vanel, M.D. 2, B. Luboinski, M.D. 3, and Ph. Terrier, M.D. 1 Departments of t Histopathology, 2 Radiology, and 3 Head and Neck Surgery, Institut Gustav-Roussy, Villejuif, France
Clinical information A 14-year-old female patient developed left nasal obstruction, epistaxis, e x o p h t h a l m o s and diplopia o f 2 m o n t h s ' duration. Facial plain roentg e n o g r a m s showed an opacity o f the left maxillary sinus. O n C T study, there was a partially calcified t u m o r o f the left nasal cavity, maxillary and e t h m o i d sinuses (Fig. 1). Differential diagnosis included cartilaginous tumor, benign ( c h o n d r o m a ) or malignant ( c h o n d r o s a r c o m a ) , and fibrous dysplasia with cartilaginous metaplasia. A n increased u p t a k e was noted on the b o n e scan. N o l a b o r a t o r y abnormalities were detected. A biopsy o f the lesion was performed, but no conclusion could be drawn. The t u m o r was r e m o v e d by surgery.
Fig. 1. ACT, axial and B coronal views. Mass in the left maxillary sinus, nasal cavity, and ethmoid. The walls of the sinus are displaced but not destroyed. The hard palate is partially destroyed. The tumor contains heterogeneous calcifications suggesting a cartilaginous tumor
* Presented at the closed meeting of the International Skeletal Society, New York, September 1989 Address reprint requests to: D. Vanel, De-
partment of Radiology, Institut GustaveRoussy, 39, rue Camille Desmoulins, F94805 Villejuif Cedex, France 9 1991 International Skeletal Society
F. Martinez-Madrigal et al. : Case report 670
Diagnosis: Chondroblastoma maxillary sinus The pathological specimen showed a well-defined, non-encapsulated tumor of rubbery consistency. The cellularity was variable. Cellular areas showed both mononucleated and giant multinucleated cells of benign appearance (Fig. 2A). Mononuclear cells were found in sheets of round or polyhedral cells with distinct cytoplasmic border. Multinucleated cells were isolated or in small groups in variable proportions. Less cellular areas exhibited an interstitial matrix with chondroid differentiation. Scattered calcifications and focal areas of ossification were noted (Fig, 2B). No mitosis or cellular atypia were present.
Discussion Chondroblastoma is a benign and distinct bone tumor that almost invariably arises in the epiphysis of long bones . This is a rare tumor comprising less than 1% of all bone tumors . Chondroblastoma usually occurs in individuals between 5 and 25 years of age and more frequently in men . Histogenetically, it is thought to derive from cartilage cells of epiphyseal cartilage . This theory is supported by its usual location in the epiphyses of children and young adults. Although the long tubular bones are the ones most commonly involved, a number of cases with facial and skull locations have been reported [1, 4]. These patients are, in general, older than patients with chondroblastoma of the long bones . The most frequent site is the temporal bone. A tumor mass in the ear and loss o f hearing are the most frequent complaints . Only one case of chondroblastoma of the maxilla has been reported . In our case the tumor was found in the maxillary sinus, an exceptional location for chondroblastoma, and to our knowledge, no other case has been described to date. In this peculiar location, the differential diagnosis includes the true giant-cell tumors and the giant-cell
Fig. 2. A Histological section from the tumor. Typical chondroblastoma showing areas of chondroid substance and round cells with abundant cytoplasm (H&E x 25). B Cellular areas, showing mono- and multinucleated cells, both of benign appearance. Note focal areas of ossification (H & E • 100)
reparative granulomas. Chondroid differentiation and/or calcifications must be identified for the correct diagnosis. Our case showed all the histological features characteristic of typical chondroblastoma. Roentgenographic findings were consistent with a slow-growing cartilaginous tumor, but, as in other series , they were not suggestive of the diagnosis
of chondroblastoma. A slow-growing cartilaginous tumor (chondroma or chondrosarcoma) or fibrous dysplasia with chondroid metaplasia was suggested. No histological signs o f malignancy were found in our case. In summary, a benign chondroblastoma of the left maxillary sinus developed in a 14-year-old girl.
F. Martinez-Madrigal et al. : Case report 670 Roentgenographic examination s h o w e d a n e x p a n d i n g , well-defined, calcified t u m o r . F a c i a l a n d skull c h o n d r o b l a s t o m a s m a y be r e l a t e d to p r i m a r y o r s e c o n d a r y centers o f enc h o n d r a l ossification, a n d t h e r e f o r e t h e y m a y derive f r o m the c a r t i l a g e o f g r o w t h p l a t e s . Initial b i o p s y was inconclusive, b u t a f t e r surgical removal a histological diagnosis was made.
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4. Kurt AM, Unni KK, Sim FH, McLeold RA (1989) Chondroblastoma of bone. Hum Pathol 20 : 965 5. Levine DG, Bensch KG (1972) Chondroblastoma. The nature of the basic cell. A study by means of histochemistry, tissue culture, electron microscopy and autoradiography. Cancer 29 : 1546 6. Spjut H J, Dorfman HD, Fechner RE, Ackerman LV (1971) Tumors of bone and cartilage. Armed Forces Institute of Pathology, Washington DC, p 33 (Atlas of tumor pathology, fasc. 5)