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Journal of Cancer Research and Therapeutics, Vol. 7, No. 2, April-June, 2011, pp. 192-194 Case Report A fatal case of mesenchymal chondrosarcoma of the mandible Arvind Krishnamurthy1, Anitha Vaidhyanathan1, Satish Srinivas2, Urmila Majhi3 1 Department of Surgical Oncology, Cancer Institute (WIA), 36, Sardar Patel Road, Adyar, Chennai-600 020, India Code Number: cr11045 PMID: 21768711 Abstract A 34-year-old man presented with an expansile, erosive tumor involving the left side of the mandible, with secondary invasion into the maxilla, measuring 13 Χ 7 cm. Microscopic analysis revealed a malignant small round cell neoplasm with focal cartilaginous differentiation. Immunohistochemical analysis revealed positivity for vimentin, NSE and CD99 with primitive small round cells, and S100 positivity with neoplastic chondrocytes. To the best of our knowledge, this is perhaps the largest reported case of mesenchymal chondrosarcoma of the maxillofacial region. Diagnosed as inoperable, he was treated with radiation and chemotherapy only to die within a few months. Keywords: Head and neck tumors, mesenchymal chondrosarcoma, prognosis Introduction Lichtenstein, in his review, described a heterogeneous group of 25 unusual chondroid tumors of bone. However, two tumors were described as notably different from the remainder. These tumors possessed a combination of comparatively benign-appearing round-oval chondroid islands and highly anaplastic intervening stroma. Given the appearance, separation of this group from other bone tumors was accomplished. The investigators believed them to be multicentric in origin--derived from cartilage-forming mesenchyme, thus using mesenchymal chondrosarcoma (MC) to describe the tumor. [1] MC can occur in all other sites of the craniofacial compartment where cartilage is found-the maxillofacial skeleton, base of skull, and nasopharynx. [2],[3],[4] Unique in terms of its presentation and rapid, aggressive biological behavior, our case is perhaps the largest reported of MC of the mandible. Case Report A 34-year-old man without any comorbid illnesses was referred for evaluation of a rapidly growing necrotic, expansible, ulceroproliferative lesion, measuring 13 x 7 cm on the left side of the mandible and maxilla of two-month duration. Clinical examination revealed a large foul-smelling ulcerated tumor occupying the left half of the face and protruding through the oral cavity. Marked facial asymmetry along with bicortical expansion was noted on the left side [Figure - 1]a. There was left mandibular nerve paresthesia, with no significant lymphadenopathy. Intraorally, the swelling appeared diffuse but lobulated with an ulcerated surface and extended from the right mandibular parasymphysis to the left retromolar trigone. Computed tomography (CT) scans revealed a large heterodense irregular mass obliterating the oral aperture. Bony destruction of the mandibular alveolus was evident with infiltration to the ipsilateral maxilla, the hard palate, and also infiltrating the soft palate submucosally, as evidenced by a bulge in the left lateral wall of the oropharynx. Serum alkaline phosphatase, chest CT, and bone marrow aspirate were normal [Figure - 2]. Microscopic examination revealed a cartilaginous tumor with a characteristic bimorphic histological appearance, with areas of well-differentiated cartilage along with abnormal neoplastic chondrocytes and small primitive-appearing round cells with scanty cytoplasm and round to oval hyperchromatic nuclei. Mitotic activity was increased and varied from 1-3/hpf. Chondromyxoid areas were also seen [Figure - 3]. Immunohistochemically, the small cells of the cartilaginous component demonstrated positive immunostaining with S100, vimentin, CD99, and neuron-specific enolase [Figure - 4]. The histological features were suggestive of MC. The lesion was deemed inoperable in view of its rapid progression and extensive skin and soft tissue involvement. He was hence planned for radiotherapy and single agent doxorubicin chemotherapy, with an intention for intensification based on his response. An elective tracheostomy was fashioned prior to initiation of radiotherapy and a nasogastric tube was placed to ensure adequate nutrition. He developed Grade 4 neutropenia following the first cycle of doxorubicin, and hence further chemotherapy was deferred. He went on to complete 60 Gy of radiation. He demonstrated partial response and was planned for reassessment after six weeks [Figure - 1]b. He presented a month after the completion of his radiation with 2-day-old paraplegia. There was no appreciable change in the dimensions of the lesion. A magnetic resonance imaging of his spine revealed an extradural mass compressing the spinal cord at the second dorsal vertebra [Figure - 5]. Thereafter, he was considered for only palliative radiotherapy, 30 Gy was delivered over 10 days. His general condition further deteriorated and he succumbed to disease within a month. Discussion The clinical course of MC has been known to exhibit great variability. Duration of symptoms is controversial, with time frames ranging from 4 days to 7 years in reviews involving MC in all areas of the body. [2] Nakashima et al.[2] documented the age range as between 5 to 74 years, with 60% diagnosed in the second and third decade. There has been no gender predilection. Radical surgery with clear margins appears to be the most effective treatment for MC, as is with other chondrosarcomas occurring in the jaws and should be considered in all potentially operable cases. [2],[3],[4],[5],[6] Similar to predicaments regarding the management of chondrosarcomas, [6] the role of chemoradiation appears to be unclear in the management of MC. Huvos et al.[5] subdivided a group of 13 MC patients into the better-differentiated hemangiopericytomatoid (eight patients) and undifferentiated small cell variants (five patients). In the small cell undifferentiated group, the two patients still alive had been treated with preoperative irradiation and preoperative and postoperative chemotherapy. One of these two patients had a clinical cure at 27-month follow-up. Conversely, five of the eight patients in the hemangiopericytomatoid group had no evidence of disease at 8 to 49 months of follow-up. Their treatment was not uniform, but all four of the patients who received initial chemotherapy with or without irradiation or surgery, followed by more chemotherapy, had no evidence of disease at follow-up. Their chemotherapeutic regimens included high-dose methotrexate, doxorubicin, and a combination of bleomycin, cyclophosphamide, and dactinomycin. Nooij et al.[7] prospectively observed one of two good pathological responses in the primary tumor after preoperative doxorubicin and cisplatin combination therapy, but only a limited effect in four patients treated with the same regimen in the metastatic setting. Tumors with a high percentage of small cells and limited cartilage content are thought to be most sensitive to chemotherapy and radiotherapy, as with other small cell sarcomas. [5] Patients with MC of the jaws have variable clinical course following treatment. This may range from complete tumor response and long-term survival, to rapid local tumor progression with widespread metastasis and demise within months. It is accepted that recurrence following initial tumor response may occur within short or long periods of disease-free status for chondrosarcomas as well as MC. [2],[3],[4],[5],[6] Saito et al., [6] in their review of chondrosarcomas of the jaws, report overall actuarial survival at 5, 10, and 15 years to be as high as 80.7, 65.3, and 56%, respectively, which is perhaps in stark comparison with the overall 5- and 10-year survival for patients with MC. Nakashima et al.[2] found a 5-year survival rate of 54.6% and 10-year survival rate of 27.3%. Salvador et al.[3] reported 5- and 10-year survival rates for patients with MC to be 55 and 27%, respectively. Huvos et al.[5] found a l0-year survival rate of 28%. At no instance has treatment for MC been randomized; hence, comparisons of treatment modalities and subsequent results remain limited. Collective experience allows broad conclusions to be drawn. Ablative surgery appears to be the most effective treatment for MC and the benefit of chemotherapy and radiotherapy is unclear. References
Copyright 2011 - Journal of Cancer Research and Therapeutics The following images related to this document are available:Photo images[cr11045f2.jpg] [cr11045f1.jpg] [cr11045f4.jpg] [cr11045f3.jpg] [cr11045f5.jpg] |
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