A Rare Case of a Metastatic Malignant Abrikossoff Tumor

Abrikossoff tumor, also called granular cell tumor (GCT), is a neoplasm of the soft tissues which is most commonly a solitary, painless, and benign tumor. However, 2% of Abrikossoff tumors can be malignant. We report here the case of a 75-year-old male who presented a local recurrence of Abrikossoff tumor of the left thigh. The anatomopathological analysis concluded to a malignant GCT, and the F-18 fluorodeoxyglucose positron emission tomography showed multiple lesions in the lymph nodes and bones. The potential conversion to malignancy should alert practitioners because of the extremely poor prognosis. The diagnosis of malignant granular cell tumor should be based on a bundle of clinical and histological features and not solely on histologic features because of the challenging distinction between malignant and benign tumors due to the lack of well-defined criteria for the diagnosis of malignancy. Large size and recurrence are the most important clinical features predicting malignant behavior. Patients with a history of Abrikossoff tumor should be followed closely to monitor recurrence and malignant transformation. The apparent originality of our observation – which could lie in the evolution of a GCT tumor, initially considered as benign, to a malignant form – has to be challenged regarding the issue of classifying some cases according to the classical “benign” and “malignant” dichotomy.

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.

Malignant granular cell tumor of the arm – case report

Granular cell tumor (GCT) is a rare form of soft tissue cancer that is usually benign. Its malignant evolution is encountered in less than 2% of cases, having a more rapid and unfavorable evolution. Clinical presentation betraying malignant features could be increased tumor size, rapid growth, deep localization, and female gender. This paper presents the case of a 52-year-old patient with a hard, rapidly evolving tumor in the left arm. The diagnosis of granular cell tumor was made based on histopathological examination using the Fanburg and Smith criteria to differentiate the formation as malignant, but with certainty this was subsequently confirmed by the existence of a metastasis. Surgical excision was performed and the evolution was favorable. Evolution and treatment differ depending on the benign or malignant form, but surgical treatment with wide local excision is recommended. This may be followed by chemotherapy or radiotherapy, and follow-up of patients for the rest of their lives is mandatory.

Bladder malignant granular cell tumor with EP300 gene mutation: a case report and literature review

Background Malignant granular cell tumor (GCT) is extremely rare. Malignant GCT with EP300 gene mutation in the bladder has not been reported in the literature. Case presentation We report a special case of 45 years old female with malignant GCT of the bladder. Physical examination found a pelvic mass in the patient. Magnetic resonance imaging showed a huge mass between the posterior wall of the bladder, the cervix, and the anterior wall of the vagina. Pathological examination showed that the mass was 11×11×4.5cm in size, involved in the bladder's posterior wall. Under the microscope, the tumor cells were arranged in the shape of a nest or cord to infiltrate the bladder's wall. The tumor cells were pleomorphic, red-stained granular within the cytoplasm, with increased nuclear/cytoplasmic ratio, vacuolar nuclei, and obvious nucleoli. The tumor cells were showed obvious nuclear atypia, and the mitosis was more than 5/50HPF. Coagulative necrosis was widely showed within the tumor. Immunohistochemistry(IHC) showed that S-100, NSE, CD68, CR, α-AT, and TFE-3 were strongly positive, and the Ki-67 proliferation index was around 15%. The next-generation high throughput sequencing indicated that EP300 gene was missense mutated (c.457A>G) with 33% mutation abundance, and genes of DPYD(c.1627A>G),ERCC1( c.354T>C),NQO1(c.559C>T),TPMT(c.719A>G) and XRCC1(c.1196A>G) were polymorphic mutated. The patient died after three months of the second surgical treatment. Conclusions We report for the first time a primary bladder malignant GCM accompanied by mutations in special driving genes such as EP300. We also conducted a comprehensive literature review and an in-depth discussion.

Bladder Malignant Granular Cell Tumor With EP300 Gene Mutation: A Case Report and Literature Review

Background Malignant granular cell tumor (GCT) is extremely rare. Malignant GCT with EP300 gene mutation in the bladder has not been reported in the literature.Case presentation We report a special case of 45 years old female with malignant GCT of the bladder. Physical examination found a pelvic mass in the patient. Magnetic resonance imaging showed a huge mass between the posterior wall of the bladder, the cervix, and the anterior wall of the vagina. Pathological examination showed that the mass was 11×11×4.5cm in size, involved in the bladder's posterior wall. Under the microscope, the tumor cells were arranged in the shape of a nest or cord to infiltrate the bladder's wall. The tumor cells were pleomorphic, red-stained granular within the cytoplasm, with increased nuclear/cytoplasmic ratio, vacuolar nuclei, and obvious nucleoli. The tumor cells were showed obvious nuclear atypia, and the mitosis was more than 5/50HPF. Coagulative necrosis was widely showed within the tumor. Immunohistochemistry(IHC) showed that S-100, NSE, CD68, CR, α-AT, and TFE-3 were strongly positive, and the Ki-67 proliferation index was around 15%. The next-generation high throughput sequencing indicated that EP300 gene was missense mutated (c.457A>G) with 33% mutation abundance, and genes of DPYD(c.1627A>G),ERCC1( c.354T>C),NQO1(c.559C>T),TPMT(c.719A>G) and XRCC1(c.1196A>G) were polymorphic mutated. The patient died after three months of the second surgical treatment. Conclusions We report for the first time a primary bladder malignant GCM accompanied by mutations in special driving genes such as EP300. We also conducted a comprehensive literature review and an in-depth discussion.