Retroperitoneal malignant peripheral nerve sheath tumor treated with laparotomy approach: A case report

TL;DR

This case report describes a rare retroperitoneal nerve sheath tumor in a woman, highlighting diagnostic and treatment challenges.

Contribution

The study presents a rare clinical case of retroperitoneal MPNST and emphasizes the diagnostic difficulties in distinguishing it from other sarcomas.

Findings

Retroperitoneal MPNST is rare, accounting for only 1% of all MPNST cases.

Accurate diagnosis of MPNST requires immunohistochemistry to differentiate it from similar tumors like liposarcoma.

Complete surgical removal with clear margins is a key treatment approach for MPNST.

Abstract

Malignant peripheral nerve sheath tumors (MPNST) are sporadic neoplasms that present significant diagnostic challenges, particularly in retroperitoneal locations. While these aggressive tumors most commonly occur in the head, neck, and upper extremities, retroperitoneal cases represent a mere 1 % of all instances. This case study examines a specific instance of retroperitoneal MPNST diagnosed and treated through laparotomy, with the primary objective of enhancing medical professionals' understanding of this uncommon tumor's diagnostic complexities, treatment approaches, and potential prognostic implications. By highlighting such a rare clinical scenario, the research seeks to raise awareness among clinicians about the nuanced considerations required when encountering these challenging and infrequent malignancies in unusual anatomical regions.

During a medical investigation of abdominal pain in a 44-year-old female patient, advanced imaging revealed a complex mass located in the left adrenal gland. Computed tomography scans demonstrated significant anatomical involvement, with the tumor compressing adjacent structures, including the pancreas and spleen superiorly, the left kidney and renal vasculature inferiorly, and positioned adjacent to the abdominal aorta. Surgical intervention was undertaken with the objective of complete tumor removal, successfully achieving unambiguous surgical margins. Subsequent immunohistochemical analysis confirmed the diagnosis of an MPNST, providing critical insights into the nature of the patient's complex medical condition.

MPNST represents a complex and challenging neoplasm characterized by its highly invasive and rapidly progressing nature, originating from neural tissue. The diagnostic process for MPNST is intricate, primarily due to the absence of definitive histological criteria and a distinctive immune profile. Critical diagnostic challenges emerge from the significant morphological similarities between MPNST and other tumors, such as fibrosarcomas and leiomyosarcomas. In this specific case, pathological anatomy initially suggested a liposarcoma lesion; however, immunohistochemistry testing revealed a negative Desmin result, effectively eliminating the liposarcoma diagnosis and underscoring the nuanced complexity of accurate tumor classification.

This case report highlights the diagnostic difficulty in identifying divergent differentiation in sarcomas, using MPNST and liposarcoma as examples.

•MPNST are rare and aggressive tumors. Moreover, retroperitoneal instances are challenging to diagnose.•Distinguishing MPNST and other differential diagnosis, such as fibrosarcomas and leiomyosarcomas, is crucial.•This study highlights the diagnostic difficulty of MPNST and attempts to raise awareness regarding its diagnosis, treatment, and prognosis.

MPNST are rare and aggressive tumors. Moreover, retroperitoneal instances are challenging to diagnose.

Distinguishing MPNST and other differential diagnosis, such as fibrosarcomas and leiomyosarcomas, is crucial.

This study highlights the diagnostic difficulty of MPNST and attempts to raise awareness regarding its diagnosis, treatment, and prognosis.

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