作者：HongBin Lü
作者单位：Department of Ophthalmology, the Affiliated Hospital of Luzhou Medical College, Luzhou 646000, Sichuan Province, China Department of Ophthalmology, West China Hospital of Sichuan University, Chengdu 610041, Sichuan Province, China
【摘要】
AIM: To report a rare case of mesenchymal chondrosarcoma in the orbit and to explore its clinic manifestations, pathologic characters, management and prognosis.
METHODS: We report a case of mesenchymal chondrosarcoma of the orbit. The clinical materials, including ophthalmological examination, computed tomography scan of the orbit, histopathology and immunohistochemistry of the biopsy specimen was reported, and its pertinent literatures were reviewed.
RESULTS: A 36yearold female was seen with proptosis and decreased vision. Histopathology demonstrated an admixture of undifferentiated mesenchymal cells and islands of mature hyaline cartilage. Immunohistochemical studies revealed positivity for vimentin and S100, which was consistent with the diagnosis of mesenchymal chondrosarcoma.
CONCLUSION: Mesenchymal chondrosarcoma in the orbit is extremely rare malignant tumor. Multimodality treatments (surgery, chemotherapy and radiotherapy) may lead to longterm survival.
KEYWORDS: mesenchymal chondrosarcoma; histopathology; immunohistochemistry
【关键词】 mesenchymal chondrosarcoma histopathology immunohistochemistry
INTRODUCTION
Mesenchymal chondrosarcoma is a rare malignant tumor derived from primitive mesenchymal tissue with potential chondroblast. It can occur anywhere in the body, both in skeletal and extraskeletal sites, including the limbs, orbit, vertebra, pelvis, meninges or brain. However, it tends to occur 2 to 3 times more commonly in bones than in extraskeletal sites. It has a variable clinical course with frequent recurrence and occasional distant osseous and visceral metastatic spread [1]. It was first described in the bone in 1959 by Lichtenstein and Bernstein [2]. Mesenchymal chondrosarcoma in the orbit is extremely rare. Till now there have been only 18 cases overseas [1] and eight cases in China [38] reported on mesenchymal chondrosarcoma of the orbit. In these studies, there were no detailed case reports. In the present article, we report a case of orbital mesenchymal chondrosarcoma in detail, and summarize all the cases in Chinese literature, focusing on its clinical course, pathologic characters, management and prognosis in the literature.
CASE REPORT
A 36yearold woman, without past medical history, complained of proptosis and decreased vision in the right eye from August 2001, and admitted to our dhospital in May 2003. Computed tomography (CT) scan of the orbit revealed a firm lesion in right musclecone, and tumor resection was done in June 2003. The postoperative pathological diagnosis was hemangiopericytoma of the right orbit. After operation, the patient was not given further chemotherapy and/or radiotherapy. She was readmitted to our hospital with the same symptom in June 2005. On examination, uncorrected vision of the right eye was 1/50. The right eyeball protruded forward with mild limitation of mobility of the eyeball. On palpation, there was an unclear margin, middlefirmed mass, absence of tenderness and fluctuation (Figure 1). Fundus examination showed papilledema that projected 1D. The regional lymph nodes were not enlarged. Visual acuity (VA) of the left eye was normal. The laboratory examination was normal. CT scan of the orbit revealed a right intraorbital 30 20mm ovalshape, high density, partially calcified and welldefined, local recurrence lesion, without bone destruction (Figure 2). The preoperative diagnosis was hemangiopericytoma of the right orbit. The patient was operated under general anesthesia through tracheal cannula and via lateral orbit rim approach on June 7, 2005. We discovered a relatively well encapsulated, and partially calcified lesion in right musclecone, with mild adhesion to circumference tissue. The lesion deviated from the optic nerve without invading and the bony orbit appeared intact. We performed a macroscopically complete resection. Macroscopically, the size of the tumor was 30mm×28mm×20mm, and the cut surface was graywhite in color, rubbery in texture, and smooth in contour, with focally calcified areas. Histopathology showed a malignant tumor with an admixture of undifferentiated mesenchymal ovalshape and fusiform cells and islands of mature hyaline cartilage with some vascular channels (Figure 3). The mesenchymal cell nucleus chromatin was scarce, and had obvious nucleolus, clear atypia and mitotic figures. Islands of mature cartilage with mild atypia of cartilage cells were seen (Figure 4). Immunohistochemistry analysis was negative for desmin but positive for vimentin and S100 (Figure 5). The pathological diagnosis was mesenchymal chondrosarcoma of the right orbit. The patient was discharged on 14 June 2005. The proptosis had regressed, and the right eye vision was slightly improved (1/25). Postoperative radiation therapy was performed.
Table 1 Characteristics of patients with mesenchymal chondrosarcoma of the orbit（略）
DISCUSSION
Mesenchymal chondrosarcoma in the orbit is extremely rare. On the basis of an Internet search of the literature through Chinese Biomedicine Disc (CBMDISC), Chinese Medical Current Contents (CMCC), Chinese Periodical Fulltext Data Base (CJFD) and VIP Information (VIP), etc. from 1978 to 2005, only eight cases have been reported in China (Table 1) [38]. Outside China, Tuncer et al[1] searched for only 17 cases, except for his own case. He reported a case of a 5dayold girl with a lesion in the right lower tarsal conjunctiva present from birth, whose final diagnosis of orbital mesenchymal chondrosarcoma was made by immunocytochemical analysis at the third histologic examination when she was 5 months old. The first pathologic diagnosis was nodular fascitis at 5 days old and the second pathologic diagnosis was not definitive but suspicious of malignancy at 80 days old.
According to the foreign literature [1, 913], the mean age at diagnosis of orbital mesenchymal chondrosarcoma is approximately 23 years (ranged from 5 months to 84 years), with a female predominance (72%). The youngest patient (a 5monthold girl) with orbital mesenchymal chondrosarcoma was reported by Tuncer et al [1]. While in the Chinese literature [38], the mean age is 27.75 years (ranged from 20 to 36 years), with the female predominance of 37.5%. The reason for our lower occurrence rate in female may be that the cases are less (Table 1).
Figure 1 The right eyeball slightly protruded, and fissura palpebra widen（略）
Figure 2 CT scan showed an ovalshape, high density, partially calcified and welldefined local recurrence lesion without bone destruction（略）
Orbital mesenchymal chondrosarcoma is a malignant tumor with proptosis and decreased VA as the primary symptoms, accompanied by various degrees of limitation of eye ball mobility and optic nerve compression, no evident delimitation with surrounding soft tissue. Some patient complained of pain in eyes and headache. With progression of disease, it can invade the orbit soft tissue and local bone. Only one case had extended to the intracranial region reported by Khouja et al[13]. Distant metastasis is rare.
Figure 3 An admixture of undifferentiated mesenchymal ovalshape and fusiform cells and islands of mature hyaline cartilage with some vascular channels The mesenchymal cell nucleus chromatin was scarce, and had obvious nucleolus, clear atypia and mitotic figures (HE×248.16)（略）
Figure 4 Islands of mature cartilage with mild atypia of cartilage cells were seen (HE×454.08)（略）
Figure 5 Immunohistochemistry showed vimentin (++) (×503.36)（略）
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