|LETTER TO THE EDITOR
|Year : 2018 | Volume
| Issue : 6 | Page : 1446-1448
Mesenchymal chondrosarcoma of the parapharyngeal space
Department of Surgical Oncology, Cancer Institute (WIA), Chennai, Tamil Nadu, India
|Date of Web Publication||28-Nov-2018|
Department of Surgical Oncology, Cancer Institute (WIA), 38, Sardar Patel Road, Adyar, Chennai - 600 036, Tamil Nadu
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Krishnamurthy A. Mesenchymal chondrosarcoma of the parapharyngeal space. J Can Res Ther 2018;14:1446-8
Chondrosarcomas are malignant tumors composed of transformed cells that produce neoplastic cartilage and have a predilection to affect the axial skeleton. Nearly 6%–12% of chondrosarcomas are reported to originate in the head-and-neck region, with the larynx, sinonasal tract, and skull base being the common sites. The parapharyngeal space is believed to be a very rare site for chondrosarcomas; only about ten cases have been reported in Medline literature thus far, and only two of them have been reported to be mesenchymal chondrosarcomas (MCs).,
A 23-year-old male presented to our center for further evaluation of a slowly growing painless swelling in the right parotid region for 6 months [Figure 1]. Clinical examination revealed a 7 cm × 6 cm firm swelling in the right parotic region, with an intact facial nerve function and no cervical lymphadenopathy. Cranial nerve examination, however, revealed paralysis of the cranial nerves IX and X. A magnetic resonance imaging scan revealed a 7 cm × 6 cm × 5 cm lobulated mass in the parapharyngeal space with the destruction of the petrous part of the right temporal bone and extension onto the middle and the inner ear. Further skull base destruction was seen in the right occipital bone including the basiocciput with intracranial extension into the posterior cranial fossa [Figure 2]a and [Figure 2]b. An initial fine-needle aspiration cytology was not contributory; a subsequent Trucut biopsy from the mass suggested a cartilaginous neoplasm. The patient was taken up for a radical excision of the tumor, which entailed a craniofacial resection through a postauricular transtemporal approach. A dural breach was encountered during the resection on the intracranial portion and the neurosurgical team repaired the same with a patch of temporalis fascia. The resultant soft-tissue defect was closed using the abdominal fat and by a temporalis muscle transfer [Figure 3]a, [Figure 3]b, [Figure 3]c. The final histopathology was suggestive of a poorly differentiated malignant tumor which on IHC correlation was suggestive of a MC excised with free margins [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d, [Figure 4]e, [Figure 4]f. The patient developed transient facial paresis which recovered following facial physiotherapy; in addition, he needed swallowing and speech rehabilitation. The patient, however, declined any further adjuvant treatment (chemo/radiotherapy) and was hence kept on regular follow-up. He finally succumbed to his cancer close to 3½ years after his definitive surgery due to progressive lung metastatic disease.
|Figure 2: (a and b) An magnetic resonance imaging scan revealed a 7 cm × 6 cm × 5 cm lobulated mass in the parapharyngeal space with destruction of the petrous part of the right temporal bone and extension onto the middle and the inner ear. Further skull base destruction was seen in the right occipital bone including the basiocciput with intra-cranial extension into the posterior cranial fossa|
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|Figure 3: (a) Intraoperative photograph of the patient following radical excision of the tumor, which entailed a craniofacial resection through a postauricular transtemporal approach. (b) The resultant defect was closed using the abdominal fat and by a temporalis muscle transfer (c) Postoperative clinical photograph|
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|Figure 4: (a) Section shows a malignant neoplasm composed of intermingled areas of chondromyxoid stroma. The chondrocytes appear atypical with bi and multinucleated forms. Few osteoblast type of giant cells are seen. Focal lymphovascular invasion is also seen (H and E, ×20). (b) Strong positivity for vimentin, (c) strong positivity for S-100p, (d) moderate positivity for chromogranin, (e) moderate positivity for synaptophysin (IHC, ×40), (f) 30% of cells showing nuclear positivity for Ki-67|
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Head-and-neck chondrosarcomas are reported to occur 10–20 years earlier than those in other locations, with a peak incidence around the fourth decade of life and with a slight male preponderance. MC is a rare variant of chondrosarcoma that accounts for up to 3%–9% of all chondrosarcomas. Further, compared to conventional chondrosarcomas which are very rarely extraskeletal and occur later in life, MCs frequently have an extraskeletal location and present at an earlier age.
Although the pathogenesis of MC is not entirely clear, the transition areas between the mesenchymal cells and the cartilage seem to suggest a precartilage mesenchymal origin. Complete surgical resection, either open/endoscopic, is the gold treatment for all subtypes of chondrosarcomas., There is no general agreement on whether additional chemotherapy, radiotherapy, or both are necessary after surgery., Although chondrogenic tumors have been traditionally believed to be relatively radioresistant, MCs, with a higher fraction of dividing cells, have shown benefit from radiotherapy., MCs tend to have an aggressive clinical course with a higher tendency for late recurrences and distant metastasis; the overall disease prognosis is reported to be poor with 5-year survivals ranging from 30% to 50%.
In conclusion, our report adds to the limited literature and will guide oncologists to make better-informed choices with regard to the management of chondrosarcomas of the parapharyngeal space.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]