Malignant Triton Tumor of the Head and Neck

Abstract

Malignant triton tumor is a rare, usually aggressive sarcoma consisting of a malignant schwannoma with rhabdomyoblastic differentiation. Although 20% of cases are located in the head and neck, this unusual tumor has not-to our knowledge-been described in the otolaryngology literature. We present the tenth reported case of triton tumor of the head and neck and the second involving the paranasal sinuses.'-9 An analysis of head and neck triton tumors suggests a possible subset of patients with lower-grade histology and improved survival, compared with triton tumors that occur elsewhere. CASE REPORTA 66-ye=-old woman went to her physician with a 1-year history of left nasal airway obstruction. She was treated with antibiotics for a diagnosis of sinusitis, with no improvement. A few months later, profuse left-sided epistaxis developed. A bleeding granular mass of the left nasal cavity was noted and subsequently underwent wide biopsy. Pathology suggested a malignant spindle-cell tumor. A CT scan revealed a large mass of the left nasal cavity involving the left ethmoid sinus and orbit (Fig. 1). She was referred to the Massachusetts Eye and Ear Infirmary.The patient's history was otherwise unremarkable. She had noted no anosmia or visual symptoms. Examination revealed a patent left nasal cavity status after wide biopsy. A detailed ophthalmologic evaluation revealed 2.5 mm of proptosis of the left eye, but was otherwise normal. Review of the pathology specimen revealed polypoid spindle cells with foci consistent with skeletal muscle differentiation and rare mitoses, suggestive of an unusual benign neoplasm.A left external, extended ethmoidectomy approach was performed to excise what was thought to be a benign or lowgrade malignant tumor. A fleshy grey mass was found, centered on the left posterior ethmoid sinus, displacing, thinning, and eroding the lamina papyracea and floor of the anterior cranial fossa. Dura and periorbiturn abutted tumor, but were From the Departments of Otolaryngology and Pathology, The Mas-

Malignant triton tumor of the nasal cavity

“…4,5 One of these cases showed welldifferentiated histology and low mitotic activity 5 ; the other case was also low-grade sarcoma with a high degree of skeletal differentiation with focal cytological atypia without necrosis or significant mitotic activity. 4 Our case also showed moderate cellular pleomorphism and low mitotic activity. These findings are unlike those of MTT in other sites, which are histologically high-grade with large numbers of mitoses.…”

“…3 It shows rapid growth, early metastasis and poor prognosis. 4 The tumor was first described by Masson 5 in 1932. A review of the literature yielded 48 reported cases, and only 10 cases of MTT of head and neck have been reported.…”

“…Of these, only two cases involved the nasal cavity and paranasal sinuses. 4,6 It was called a ÔÔTritonÕÕ tumor from LocatelliÕs experiments in which the cut ends of the sciatic nerve implanted subcutaneously in the back of triton salamander caused the growth of supernumerary limbs containing neural and muscular elements. 7 We present a case of sinonasal MTT and analyze its biological behavior by reviewing the two cases in the literature.…”

Sinonasal Malignant Triton Tumor: A Case Report with Historical and Surgical Review

“…Only two patients had died at the time of manuscript publication, one from an unrelated malignancy with no evidence of MTT recurrence, and the other a patient who had non-resectable tumor at initial presentation. All other patients were treated surgically, with eight receiving adjuvant radiotherapy and two adjuvant chemotherapy as summarized in Table 1 [6][7][8][9][10][11][12][13][14][15][16].…”

Rhabdomyoblastic Differentiation in Head and Neck Malignancies Other Than Rhabdomyosarcoma

“…[26] The majority of malignant Triton tumors occur in the setting of NF1, and as a result, the affected patient is typically young [22,23]. About a third of malignant Triton tumors affect the head and neck where they can involve virtually any anatomic subsite [27][28][29]. The rhabdomyoblasts in malignant Triton tumors are typically focal, and they often stand out at low power as their abundant eosinophilic cytoplasm is distinctly different than the pale background Schwannian cells (Fig.…”