Malignant granular cell tumor of the ulnar nerve: A case report of long-term follow-up and literature review

Background: The incidence of malignant granular cell tumor, an extremely rare Schwann cell-derived tumor with a poor prognosis, is reported to be approximately 0.2% of malignant soft tissue tumors. We report a case of a malignant granular cell tumor originating from the ulnar nerve.Case presentation: A 71-year-old woman presented with a mass in her right forearm. Magnetic resonance imaging showed a tumor with homogenous intensity of T1 and heterogeneous hyperintensity of T2, continuous with the ulnar nerve. Incisional biopsy revealed a malignant granular cell tumor, and marginal excision of the tumor was performed. Histologically, the tumor size was 9.2 cm and consisted of eosinophilic, granular polygonal to round and spindle-shaped cells, with vesicular and prominent nucleoli, and increased mitosis. Immunohistochemically, the tumor cells were positive for S-100 protein, CD68, H3K27me3, TFE3, and SOX10 and negative for smooth muscle alpha-actin, desmin, cytokeratin AE1/3, epithelial membrane antigen, and synaptophysin. The Ki-67 positivity rate was 12%. These findings were consistent with those of malignant granular cell tumors. In addition, no metastasis or recurrence was observed 15 years after the excision.Conclusion: Surgical resection is the standard treatment option. In our case, the diagnostic criteria for malignant granular cell tumors were histologically met. Patients with malignant granular cell tumors have a poor prognosis. However, no metastasis or recurrence was observed in this case 15 years after the surgery.

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

Case Reports in Pathology ◽

10.1155/2019/9576487 ◽

2019 ◽

Vol 2019 ◽

pp. 1-8

Author(s):

Yara A. Alnashwan ◽

Khaled A. H. Ali ◽

Samir S. Amr

Keyword(s):

Abdominal Wall ◽

Poor Prognosis ◽

Granular Cell Tumor ◽

Anterior Abdominal Wall ◽

Granular Cell ◽

Cell Tumor ◽

Prolonged Survival ◽

Review Of The Literature ◽

Malignant Granular Cell Tumor ◽

The Right

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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Malignant granular cell tumor of the ulnar nerve with novel cytogenetic and molecular genetic findings

Cancer Genetics and Cytogenetics ◽

10.1016/j.cancergencyto.2009.01.011 ◽

2009 ◽

Vol 191 (1) ◽

pp. 46-50 ◽

Cited By ~ 18

Author(s):

Dionysios J. Papachristou ◽

Alka Palekar ◽

Urvashi Surti ◽

Kathleen Cieply ◽

Richard L. McGough ◽

...

Keyword(s):

Ulnar Nerve ◽

Granular Cell Tumor ◽

Molecular Genetic ◽

Granular Cell ◽

Cell Tumor ◽

Malignant Granular Cell Tumor

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Multifocal Abrikossoff's granular cell tumor of the oesophagus: Case report

Srpski arhiv za celokupno lekarstvo ◽

10.2298/sarh0810533r ◽

2008 ◽

Vol 136 (9-10) ◽

pp. 533-537 ◽

Cited By ~ 1

Author(s):

Tomislav Randjelovic ◽

Zorica Stojsic ◽

Jasna Gacic ◽

Darko Babic ◽

Miodrag Stojiljkovic ◽

...

Keyword(s):

Tumor Cells ◽

Granular Cell Tumor ◽

Pathological Finding ◽

Granular Cell ◽

Soft Tissue Tumors ◽

Posterior Mediastinum ◽

Cell Tumor ◽

Ki 67 ◽

Luminal Narrowing ◽

Operative Specimen

INTRODUCTION Granular cell tumors, relatively uncommon soft tissue tumors, have been a matter of debate among pathologists regarding histogenesis for a long time. Less common locations are in the aerodigestive tract including the oesophagus. CASE OUTLINE We have recently treated a rare case, a 37-year old male, who was admitted due to dysphagia and a painful swallow with occasional pharyngo-nasal regurgitation followed with a mild loss of weight. Standard clinical examination including X-ray chest, ECG and laboratory tests did not show pathological findings. Barium contrast oesophagography demonstrated multiple ovoid defects in the wall of the oesophagus. CT scan of the chest confirmed luminal narrowing owing to the tumor of the upper oesophagus. Upper endoscopy showed unusual multifocal nodular lesions alongside the oesophageal axis covered by smooth mucosa. A primary biopsy specimen taken from the largest nodules confirmed an unusual pathological finding of the granular cell tumor. Subtotal, transpleural oesophagectomy was performed and reconstruction was derived by long colon segment interposition through the posterior mediastinum. The postoperative course was uneventful. The operative specimen consisted of four ovoid tumors alongside the oesophagus (the greatest diameter 0.5-1.8, average 1.25). All verified tumors histologicaly consisted of a spindle-shaped or polygonal cells containing small and large eosinophilic granules and central nuclei. Most tumor cells showed strongly positive immunohistochemical staining for S-100 protein. These tumor cells were partially positive for p-53 and Ki-67. No lymph node metastases were detected histologically. CONCLUSION Multifocal granular cell tumor of the oesophagus is an unusual finding with low incidence, and rarely caused symptoms. Pathological features and multiplicity of such tumors emphasized malignant predisposition requiring surgical resection of the oesophagus.

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