Metastatic malignant peripheral nerve sheath tumor. A diagnostic surprise

Abstract

Malignant peripheral nerve sheath tumours (MPNSTs) are rare soft tissue tumors that arise from pre-existing plexiform neurofibromas or within a normal peripheral nerve. They are aggressive tumors with high rates of recurrence and distant metastases, the most common sites of metastasis being the lung followed by bone.A 46 year old gentleman presented with breathlessness and chest pain three years after post amputation of left thumb for an ulcerative growth. CECT thorax showed a left upper lobe mass with pleural and pericardial effusion. Within a month of presentation he worsened and succumbed to the disease. Antemortem biopsy of the left hand ulcerative growth showed features suggestive of malignant peripheral nerve sheath tumour- epithelioid variant and post mortem liver and lung biopsy showed metastasis of MPNST. The diagnosis was a malignant peripheral nerve sheath tumor with lung, liver and cardiac metastasis. This case report aims to highlight the importance of upfront aggressive multimodality local therapy for achieving local disease control in patients presenting with localised MPNST and regular follow up for early detection of relapse and metastasis.

Investigation of Ultrasound Parameters for the Differential Diagnosis of Malignant and Benign Peripheral Nerve Sheath Tumors

“…Malignant peripheral nerve sheath tumors (MPNSTs) are relatively rare, representing 3%–10% of all soft‐tissue sarcomas 4,5 . MPNSTs are soft tissue tumors with a poor prognosis, with a reported 5‐year survival rate of 20%–50%, local recurrence rate of 40%–65%, and metastasis rate of 30%–60% 4–8 . Therefore, early detection of MPNSTs and differentiation from BPNSTs are of great clinical value, as the preferred treatment is surgical resection with a negative resection border, which is the only way to completely control the disease 9–11 …”

“…4,5 MPNSTs are soft tissue tumors with a poor prognosis, with a reported 5-year survival rate of 20%-50%, local recurrence rate of 40%-65%, and metastasis rate of 30%-60%. [4][5][6][7][8] Therefore, early detection of MPNSTs and differentiation from BPNSTs are of great clinical value, as the preferred treatment is surgical resection with a negative resection border, which is the only way to completely control the disease. [9][10][11] The gold standard for the diagnosis and differentiation of BPNSTs and MPNSTs should be pathology; however, because MPNSTs are relatively rare, routine biopsy is impractical.…”

Hemotórax paraneoplásico metastático de schwannoma maligno: um relato de caso

“…O objetivo do trabalho é apresentar um caso sobre uma apresentação clínica rara, de um portador de TMBNP, que apresentou uma hemotórax maciço cuja intervenção cirúrgica solucionou o hemotórax, proporcionou alta hospitalar e possibilitou cuidados paliativos por uma equipe especialista na área. Metástase pulmonar de TMBNP também foi descrita por Bai 7 em 2021, entretanto não houve hemotórax associado 7 .…”