Typical and Atypical Granular Cell Tumors of Soft Tissue

Malignant granular cell tumor (MGCT) is a rare high-grade mesenchymal tumor of Schwann cell origin. MGCTs commonly affect thigh, extremity, and trunk; however, involvement of the abdominal wall is quite rare. It has poor prognosis with 39% mortality rate in 3-year interval. We report a 50-year-old female who had MGCT arising in the anterior abdominal wall and developed massive metastatic deposits in both lungs and in the right inguinal lymph nodes, with prolonged survival for 11 years. A brief review of the literature is presented.

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“…Most patients (64%) were females. There was a predilection for the upper extremities and the upper trunk [40].…”

Section: Discussionmentioning

confidence: 99%

Metastasizing Malignant Granular Cell Tumor (Abrikossoff Tumor) of the Anterior Abdominal Wall, with Prolonged Survival

Alnashwan

Ali²,

Amr

2019

Case Reports in Pathology

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“…Awareness of stromal desmoplasia and consequential cytological changes in a cutaneous GCT is perhaps more important from a prognostic perspective. This is because several classification schemes employ features such as spindling of the tumor cells to support a diagnosis of atypical or malignant GCT . In 1998, Fanburg‐Smith et al proposed a system for selection of atypical and malignant GCT based on 6 histopathologic criteria: (a) nuclear pleomorphism, (b) increased nuclear‐cytoplasmic ratio, (c) vesicular nuclei with macronucleoli, (d) tumor necrosis, (e) mitotic rate >2/10 high‐power fields, and (f) spindling of tumor cells.…”

Section: Discussionmentioning

confidence: 99%

Desmoplastic stromal changes in cutaneous neural granular cell tumors: An under‐recognized histopathologic feature of diagnostic and prognostic importance

et al. 2019

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Background: Granular cell tumors (GCTs) are uncommon mucocutaneous and soft tissue neoplasms with distinctive histopathologic appearance but controversial histogenesis. Herein, we report a variant of cutaneous GCT featuring extensive desmoplastic stroma which may result in diagnostic difficulties with mesenchymal proliferations, particularly a dermatofibroma.Methods: Following a recent case of GCT with prominent stromal desmoplasia, we reviewed all cases diagnosed as GCT during the past 10 years accessioned at the dermatopathology unit in a tertiary university hospital.Results: Three additional cases with a similar excessive connective tissue were identified out of a total of 49 GCTs. Cytoplasmic granularity was often subtle and focal, S100 expression was weak, and nuclei had a tendency to show spindling in tumor cells entrapped within the desmoplastic areas. Of note, nuclear spindling is one of the criteria used to diagnose an atypical/malignant GCT. Conclusion:We propose the term "desmoplastic GCT" for these tumors, which not only appropriately addresses the stromal changes but also raises an awareness of GCT being one of the cutaneous tumors which may show stromal desmoplasia. Differential diagnostic difficulties apart, awareness of this phenomenon is important so that desmoplasia and resultant spindling are not linked with potential aggressive behavior. K E Y W O R D Scutaneous neoplasms, desmoplasia, diagnostic criteria, granular cell tumor, prognosis

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“…Granular cell tumors are benign neuroectodermal tumors composed of large round to oval cells with abundant and distinctly granular cytoplasm [66]. The tumor can arise at any age, but most cases occur in the fourth to sixth decades of life.…”

Section: Granular Cell Tumormentioning

confidence: 99%

“…The tumor can arise at any age, but most cases occur in the fourth to sixth decades of life. Granular cell tumor commonly arises in the extremities, head and neck, and trunk [66]. Loss-of-function mutations in ATP6AP1 and ATP6AP2 are present in about 70% of cases [67].…”

Section: Granular Cell Tumormentioning

confidence: 99%

Epithelioid Cutaneous Mesenchymal Neoplasms: A Practical Diagnostic Approach

Choi

2020

Diagnostics

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Epithelioid cells are rounded or polygonal cells with abundant eosinophilic or clear cytoplasm and ovoid to round nuclei, superficially resembling epithelial cells. Cutaneous mesenchymal neoplasms composed predominantly or exclusively of epithelioid cells are relatively uncommon and can cause considerable diagnostic difficulties due to overlapping histologic features among heterogeneous groups of tumors. Familiarity with practical diagnostic approaches and recognition of key histopathologic features are important for correct diagnosis and management. This review summarizes the histologic features of epithelioid cutaneous mesenchymal neoplasms and discusses their differential diagnoses from malignant melanomas and carcinomas.

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Typical and Atypical Granular Cell Tumors of Soft Tissue

Cited by 29 publications

References 8 publications

Metastasizing Malignant Granular Cell Tumor (Abrikossoff Tumor) of the Anterior Abdominal Wall, with Prolonged Survival

Metastasizing Malignant Granular Cell Tumor (Abrikossoff Tumor) of the Anterior Abdominal Wall, with Prolonged Survival

Desmoplastic stromal changes in cutaneous neural granular cell tumors: An under‐recognized histopathologic feature of diagnostic and prognostic importance

Epithelioid Cutaneous Mesenchymal Neoplasms: A Practical Diagnostic Approach

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