A case report of a rare intramuscular granular cell tumor

Abstract

BackgroundGranular cell tumors (GCTs) were firstly described by Weber in 1854 and 70 years later by Abrikossoff and classified as benign tumors. Originally considered muscle tumors, they have been identified as neural lesions, due to their close association with nerve and to their immunohystochemical characteristics.GCTs are uncommon tumors and they may arise in any part of the body; they have been mainly observed in tongue, chest wall and upper extremities; less frequent sites are larynx, gastrointestinal tract, breast, pituitary stalk and the female anogenital region.Here we report a case of GCT showing an uncommon localization such as the upper third of the right rectus muscle of the abdominal wall.Case presentationA 45 year-old woman of Caucasian origin presented to the surgeon with a 6-month history of light pain in the upper third of the abdominal wall.Radiological exams (Ultrasonography, Computed Tomography and Contrast magnetic resonance imaging) showed a localized in the right rectus abdominis muscle.After excision, histological and immunohystochemical analysis, with the support of electron microscopy, allowed making diagnosis of granular cell tumor.DiscussionAfter fist description by Abrikosoff in 1926 of GCT like mesenchymal tumor of unknown origin, in recent years immunohystochemical techniques definitely demonstrated the histogenetic derivation of GCT from Schwann cells.Granular cell tumors are rare, small, slow-growing, solitary and painless subcutaneous nodules which behave in a benign fashion, but can have a tendency to recur; in rare cases they can metastasize, when they became malignant; there are some clinical and histological criteria to suspect the malignance of this tumor.ConclusionIt is important that clinicians, radiologists and pathologists are aware of the clinical presentation and histopathology of GCT for appropriate management, counselling and follow-up. In our case we had a complete radiological, morphological and immunohystochemical characterization of the lesion and a definitive diagnosis of benignity confirmed by electron microscopy.

Granular cell tumor of the trunk of the facial nerve

“…Although this is not always possible because of lacking a surrounding capsule or proximity to structures such as nerves or vessels, surgical excision with a safe and clean margin is the treatment of choice for this tumor. [10,11]…”

Synchronous subcutaneous granular cell tumours, a rare presentation

“…Multifocal disease is present in approximately 5-16% of cases and although this does occur in benign disease, it can be a sign of malignancy or associated with PTPN11 and RAF1 gene mutation syndromes, such as LEOPARD or Noonan. [1][2][3][4][5] LEOPARD is an acronym for an autosomal dominant genetic syndrome. Lentingines, skin discolouration marked by multiple spots ranging in colour from dark brown to black (L).…”

Tumor de células granulares de laringe en el niño. Caso clínico

“…3 La tomografía computada y la resonancia magnética pueden ser útiles en el diagnóstico de este tumor benigno, pero el diagnóstico definitivo es histológico. 7,12 En el estudio histológico, el rasgo más característico es la presencia de células poligonales con abundante citoplasma con gránulos eosinofílicos en su interior que corresponden a lisosomas en distintos estados de fragmentación. 2 Los gránulos citoplasmáticos son positivos para el ácido peryódico de Schiff (PAS) y el Sudan Black.…”

“…2,3 El análisis inmunohistoquímico revela reacción positiva a la proteína S-100, enolasa neuronal y proteínas mielínicas, como P0 y P2. 8,[11][12][13] L a s v a r i a n t e s m a l i g n a s d e l T C G s o n extraordinariamente infrecuentes y presentan características que evidencian su naturaleza agresiva, como la existencia de necrosis, intensa actividad mitótica, pleomorfismo nuclear, tamaño mayor de 4 cm, crecimiento rápido, infiltración a estructuras adyacentes y recurrencia. 1,8,14,15 También han sido presentados casos de aparición sincrónica de carcinoma de células escamosas y TCG.…”