Malignant triton tumor below the peritoneal reflection: a case report

Abstract Malignant triton tumor (MTT) is a rare subtype of malignant peripheral nerve sheath tumors (MPNSTs) histologically defined by rhabdomyoblastic differentiation. MTTs are primarily found in the head, neck, extremities and trunk, but rare cases of MTT within the buttock, the mediastinum and the retroperitoneum have also been documented. We present the case of a 47-year-old male patient who initially presented with right flank pain and hematuria in July 2019, who was found to have a large pelvic mass below peritoneal reflection. Complete resection of the mass was performed, and pathology identified the mass as a MTT.

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Surgical Management of Sporadic Peripheral Nerve Schwannomas in Adults: Indications and Outcome in a Single Center Cohort

Cancers ◽

10.3390/cancers13051017 ◽

2021 ◽

Vol 13 (5) ◽

pp. 1017

Author(s):

Julian Zipfel ◽

Meizer Al-Hariri ◽

Isabel Gugel ◽

Alexander Grimm ◽

Volker Steger ◽

...

Keyword(s):

Peripheral Nerve ◽

Clinical Signs ◽

Complete Resection ◽

Adult Patients ◽

Neurological Deficits ◽

Primary Tumors ◽

Surgical Interventions ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath

Most sporadic peripheral nerve sheath tumors in adults are schwannomas. These tumors usually present with significant pain but can also cause neurological deficits. Symptomatology is diverse, and successful surgical interventions demand interdisciplinarity. We retrospectively reviewed 414 patients treated between 2006 and 2017 for peripheral nerve sheath tumors. We analyzed clinical signs, symptoms, histology, and neurological function in the cohort of adult patients with schwannomas without a neurocutaneous syndrome. In 144 patients, 147 surgical interventions were performed. Mean follow-up was 3.1 years. The indication for surgery was pain (66.0%), neurological deficits (23.8%), significant tumor growth (8.8%), and suspected malignancy (1.4%). Complete tumor resection was achieved on 136/147 occasions (92.5%). The most common location of the tumors was intraspinal (49.0%), within the cervical neurovascular bundles (19.7%), and lower extremities (10.9%). Pain and neurological deficits improved significantly (p ≤ 0.003) after 131/147 interventions (89.1%). One patient had a persistent decrease in motor function after surgery. Complete resection was possible in 67% of recurrent tumors, compared to 94% of primary tumors. There was a significantly lower chance of complete resection for schwannomas of the cervical neurovascular bundle as compared to other locations. The surgical outcome of sporadic schwannoma surgery within the peripheral nervous system is very favorable in experienced peripheral nerve surgery centers. Surgery is safe and effective and needs a multidisciplinary setting. Early surgical resection in adult patients with peripheral nerve sheath tumors with significant growth, pain, neurological deficit, or suspected malignancy is thus recommended.

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