A rare case of intracranial teratocarcinosarcoma: Case report and review of literature

Abstract

Background:Teratocarcinosarcoma (TCS) is a rare malignant neoplasm with epithelial and mesenchymal components such as fibroblasts, cartilage, bone and smooth muscle. With less than 100 total reported cases, this malignant neoplasm is rarely encountered by neurosurgeons because it primarily involves the nasal cavity and paranasal sinuses.Case Description:A 55-year-old male with chronic frontal headaches was found to have a frontal mass with involvement of nasal sinus and right ethmoid sinus. The patient underwent preoperative embolization of tumor followed by bilateral frontal craniotomy for near total resection of the tumor. Patient did well postoperatively without new neurological deficits.Conclusion:Although cases with intracranial involvement are scarce, treatment with surgical resection with or without adjuvant treatments of chemotherapy and radiation therapy is the most widely accepted with goal for gross total resection. In our case, we achieved near total resection and the patient continues to do well without any gross neurological deficits.

A Unique Case of Sinonasal Teratocarcinosarcoma Presenting as Foster Kennedy Syndrome

“…4 Given the histologic diversity of SNTCS adequate tissue sampling is required for diagnosis and therefore biopsy is frequently inadequate. 2,4 In our case, the aforementioned tissue characteristics were discovered after thorough histologic analysis of the resected tumour. The differentiation between the two entities is important as esthesioneuroblastoma is typically a slow-growing, low-grade malignant tumour, while SNTCS is generally a high-grade lesion with a poorer prognoses.…”

“…Sinonasal teratocarcinosarcoma (SNTCS) is a rare neoplasm with under 100 cases described in the literature. 1,2 In 1984, Heffner and Hyams 3 first described the clinicopathologic characteristics of 20 cases and proposed the term "teratocarcinosarcoma". The lesion is typically an aggressive neoplasm that arises in the paranasal sinuses and occasionally extends intracranially.…”

“…1 The current recommended treatment course for SNTCS includes a combination of surgery, chemotherapy and radiation. 1,2 The prognosis for SNTCS is poor with overall mortality and recurrence rates of 30% and 37%, respectively. 1 In this report, we describe a case of SNTCS with unique symptomatology consistent with Foster Kennedy syndrome (FKS) secondary to intracranial extension of the tumour arising from the nasal cavity.…”

“…2 The most common presenting symptoms are nasal congestion and epistaxis, which are reported in 75-90% of patient cases. 1,2 Other commonly reported signs and symptoms include headache, anosmia, visual changes, proptosis/exophthalmos, frontal lobe signs and airway obstruction. 1,2 Most notably, our patient presented with FKS.…”

“…1,2 Other commonly reported signs and symptoms include headache, anosmia, visual changes, proptosis/exophthalmos, frontal lobe signs and airway obstruction. 1,2 Most notably, our patient presented with FKS. First described in 1911, FKS refers to a constellation of symptoms that include anosmia, compressive optic atrophy causing unilateral visual loss, and contralateral papilledema secondary to increased intracranial pressure.…”