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Metastatic Granular Cell Tumor: A Rare Entity.

Dimosthenis Zylis¹, Stefania Kokkali^1,2, Michail Sofopoulos³

¹First Medical Oncology Clinic, Saint-Savvas Cancer Hospital, Athens, Greece.

International Journal of Surgical Pathology

|February 7, 2024

Summary

A rare atypical granular cell tumor showed aggressive behavior after 30 years, recurring locally and metastasizing to the lungs. Despite treatment, the patient had a fatal outcome, highlighting the need for better management strategies for this rare soft tissue tumor.

Keywords:

chemotherapy granular cell tumor soft tissue sarcoma

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Area of Science:

Oncology
Pathology
Soft Tissue Neoplasms

Background:

Granular cell tumors (GCTs) are rare soft tissue neoplasms with uncertain lineage, often recapitulating Schwann cell phenotype.
GCTs are histologically classified as benign, atypical, or malignant, with most cases showing indolent behavior.
The biology, clinical course, and optimal management of GCTs remain poorly understood due to their rarity.

Purpose of the Study:

To describe the clinical course of an atypical granular cell tumor with a long indolent period followed by aggressive recurrence and metastasis.
To highlight the challenges in managing recurrent and metastatic GCTs, given the scarcity of data.

Main Methods:

Case report detailing a patient with an atypical granular cell tumor of the skin.
Description of the tumor's evolution over 30 years, including surgical removal, recurrence, and metastatic spread.
Administration of systemic therapy based on soft tissue sarcoma guidelines for metastatic disease.

Main Results:

An atypical granular cell tumor recurred locally and metastasized to the lungs after a 30-year indolent period.
Systemic therapy guided by soft tissue sarcoma protocols resulted in disease progression.
The patient experienced a fatal outcome, underscoring the aggressive potential of advanced GCT.

Conclusions:

Recurrent and/or metastatic granular cell tumor is a rare, potentially life-threatening condition with unknown therapeutic responses.
Atypical and malignant GCTs exhibit aggressive behavior distinct from benign GCTs, warranting clinical vigilance and comparison to soft tissue sarcomas.
Further research is needed to explore the role of adjuvant chemotherapy and radiation therapy in preventing recurrence.