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Granular cell tumors (GCTs) are characterized by abundant eosinophilic cytoplasmic granules. Based on the hypothesis that canine intracranial GCT is a subtype of meningioma and its cytoplasmic granules are formed through autophagy processes, histopathological and immunohistochemical examination were performed on biopsy samples from 7 cases of canine intracranial GCTs and 15 cases of conventional meningiomas. Histopathologically, 7/7 cases of GCTs involved the meninges; foci of meningothelial-like cells were observed in 3/7 cases; brain invasion was observed in 2/7 cases. Immunohistochemically, neoplastic cells of GCTs were positive for E-cadherin and negative for S100, cytokeratin, CD204, and β-catenin in 7/7 cases. Neoplastic cells of 15/15 cases of meningiomas were positive for E-cadherin, and negative for S100 and CD204. Immunoreactivity of meningiomas for cytokeratin and β-catenin was observed in 6/15 cases and 8/15 cases, respectively. Cytoplasmic granules of GCTs were positive for ubiquitin (5/7), p62 (5/7), and LC3 (7/7). Compared to GCTs, the ratios of ubiquitin (6/15) and p62 (3/15) positive cases were lower in meningiomas, and 15/15 cases were negative for LC3. These findings indicate that the biological natures of GCTs including anatomical location, histopathological features and immunoreactivity for E-cadherin are almost in conformity with those of meningiomas. The immunoreactivity for autophagy associated molecules may suggest the possible involvement of autophagy in cytoplasmic granule formation of canine intracranial GCTs.

Introduction and Objective: Granular cell tumour (GCT) is a benign neoplasm of neural/schwannian origin, usually presenting as a single asymptomatic lesion, mainly located in the dermis and subcutaneous tissue or submucosa, although multiple tumours may occur. Microscopically, GCTs are composed of large cells with abundant eosinophilic, granular cytoplasm arranged in sheets, nests, cords or trabeculae. Based on the cytological characteristics and the presence of necrosis, three types are recognized: benign, atypical and malignant. We aim to present the cytological and immunohistochemical characteristics of 12 granular cell tumours., Materials and Methods: 12 cases of GCT were selected from the consultation files of one of the authors (COH) The paraffin embedded tissue was processed for immunostaining with S-100 protein, calretinin, CD68, α-inhibin, PGP9.5, CD57 (Leu7), CD63 (NKI / C3), Gap43 (growth-associated protein-43), SOX10, TFE-3 and Ki-67., Results and Conclusions: 6 male and 6 female patients, with an average age of 40, made up the study group. The most frequent location for the tumours was in the subcutaneous soft tissues of the arms. There were no malignant cases. All tumours were positive for S-100, CD57, SOX10, calretinin, CD68, PGP9.5, α-inhibin and TFE-3, with a low Ki-67 (1-5%). Additionally, we reported, for the first time, the positive immunoreaction to Gap43 (growth-associated protein-43) in GCT., (Copyright © 2018 Sociedad Española de Anatomía Patológica. Publicado por Elsevier España, S.L.U. All rights reserved.)

Benign and malignant peripheral nerve sheath tumors can involve the breast, presenting as masses in the dermis, deep breast parenchyma or axillary soft tissue. Although the histologic features are frequently characteristic, diagnosis can be challenging on core needle biopsy, and the differential diagnosis includes a variety of other benign and malignant spindle cell lesions of the breast. Here, we review the key clinical and pathological features of breast schwannoma, neurofibroma, granular cell tumor, and malignant peripheral nerve sheath tumor., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Esophageal granular cell tumors (GCTs) are rare. Eosinophilic esophagitis (EoE) is an immune-mediated disease characterized by esophageal eosinophilia despite proton pump inhibitor (PPI) therapy. Given that GCTs occur at sites of scarring and inflammation, we sought to determine the prevalence of EoE in patients with esophageal GCTs. Our center's pathology database was searched for GCT specimens from 1995 to 2014. Slides were blindly rereviewed. GCTs were scored for atypical cytological features. Presence and number of eosinophils in the tumor and the surrounding esophageal epithelium and any EoE features were recorded. Medical records were reviewed. From >30,000 esophageal cases, 23 esophageal GCTs were identified, with 18 available for review (16 adult, 2 pediatric). Median patient age was 38.7 years. Four adults had esophageal intraepithelial eosinophilia (peak 38 to 68 eosinophils/high power field [HPF]); 2 confirmed to have EoE, 1 with PPI-responsive esophageal eosinophilia, and 1 had not received PPI therapy. Both pediatric cases had confirmed EoE (peak 24 and 34 eosinophils/HPF). In total, 12/18 GCTs had intratumoral eosinophilia (peak 1 to 16 eosinophils/HPF). All 6 cases with esophageal eosinophilia had intratumoral eosinophilia. Two GCTs displayed atypical cytologic features. Esophageal eosinophilia was present in 25% of adult and 100% of pediatric GCTs, the majority confirmed to have EoE. Overall, 67% of cases had intratumoral eosinophilia and 2 had atypical features. On the basis of these findings, we propose evaluating surrounding tissue for eosinophilia when esophageal GCT is diagnosed, and adding GCT as a potential complication of untreated EoE. Research for an immunologic link between EoE and esophageal GCTs is needed.

Granular cell tumor is a benign tumor of likely neural or neuroectodermal origin that occurs most commonly in the subcutaneous tissues of the trunk, breast, and extremities of adults. Congenital gingival lesions comprise the majority of the pediatric granular cell tumors. Granular cell tumors are generally small and asymptomatic, and while 1 in 10 patients has multiple tumors, recurrence and malignancy are very rare. Mediastinal granular cell tumors have been reported, most occurring in young adult or middle-aged women. We present a case of a 16-year-old asymptomatic boy with a large mediastinal granular cell tumor incidentally identified after a motor vehicle accident, and we review the intraoperative, microscopic, and ultrastructural features of this tumor. Both the patient's age and anatomical location are unusual for this tumor, which presented technical and diagnostic challenges to the patient care team.

Granular cell astrocytoma (GCA) is a rare type of infiltrative brain tumor with most reported cases occurring in the suprasellar region. A pineal localization is extremely rare, with only 4 previously reported cases in the literature. The authors describe the case of a 16-year-old boy who developed signs of increased intracranial pressure and Parinaud syndrome. Cranial CT and MRI revealed a well-demarcated and enhanced mass in the pineal region accompanied by obstructive hydrocephalus. Subtotal resection was performed via a subtemporal approach. A histological diagnosis of GCA was made. Three years after surgery, the patient was alive and well without adjuvant therapy, and serial MRI showed no signs of progression of a small residual tumor. After a thorough review of the different epidemiological, clinical, and imaging features; treatments; and prognoses of GCAs in other intracranial localizations, the authors analyzed features of this tumor in the pineal region.

Granular cell tumors (GCTs) are uncommon soft tissue tumors characterized by cytoplasmic granular appearance of the neoplastic cells. Malignant granular cell tumors (MGCTs) comprise less than 2% of GCTs and are mostly found in the subcutaneous soft tissues of the lower extremities, especially the thighs. Very few cases have been reported in the skin. The uncommon occurrence of cutaneous MGCTs and their histopathologic similarities with their benign counterpart make difficult the diagnosis of this particular malignancy. We describe a primary cutaneous MGCT that presented as a left posterior chest wall mass in a 51-year-old woman. Local excision was performed for the primary tumor, which was first interpreted as an atypical GCT, but 3 months later a left axillary mass appeared, and subsequent axillary lymph node dissection demonstrated metastatic disease in 4 of 12 excised lymph nodes. We report the immunophenotype of this primary cutaneous MGCT, which was studied with an ample panel of antibodies and compare our results with those of the few previously reported cases in the skin and subcutaneous soft tissues.

Peripheral nerve sheath tumors are common neoplasms in daily practice. Diagnosis and classification of most conventional peripheral nerve sheath tumors are relatively straightforward for the experienced observer; but on occasion, they are diagnostically challenging (especially with locally aggressive and malignant tumors). This article aims to provide an update of the data (clinical, histological, immunohistochemistry and genomic) of benign, intermediate and malignant peripheral nerve sheath tumors, thanks to the latest WHO "Classification of Tumors of Soft Tissue and Bone", published in 2013, which includes a new chapter on "Nerve Sheath Tumors". Advances in molecular biology have provided new insights into the nature of the various peripheral nerve sheath tumors, and have begun to suggest novel targeted therapeutic approaches., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Background: To investigate the roles of autophagy in tumorigenesis, cytodifferentiation, and prognosis of odontogenic tumors, we analyzed the immunohistochemical expression of ATG7, LC3, and p62 in odontogenic tissues., Methods: Tissue specimens of nine dental follicles and 69 ameloblastomas were immunohistochemically examined with antibodies against ATG7, LC3, and p62., Results: Immunohistochemical reactivity for ATG7, LC3, and p62 was detected in many odontogenic epithelial cells and several endothelial cells and fibroblasts in dental follicles and ameloblastomas. ATG7 reactivity in ameloblatomas was significantly higher than that in dental follicles. Expression of ATG7, LC3, and p62 was found markedly in neoplastic cells near the basement membrane rather than central polyhedral cells in ameloblastomas. Reactivity for these molecules was significantly higher in unicystic ameloblastomas than in solid ameloblastomas. Granular cells in granular cell ameloblastomas showed obvious reactivity for the autophagy- related molecules, and LC3 reactivity in granular cell ameloblastomas was significantly higher than in other ameloblastoma variations. Recurrent ameloblastomas showed significantly lower reactivity of LC3 and p62 than primary ameloblastomas., Conclusions: Expression of ATG7, LC3, and p62 in dental follicles and ameloblastomas suggests that autophagy regulation might be affected by microenvironment alterations during tumorigenesis. The molecular machinery for autophagy is possibly involved in tissue architecture, neoplastic cell differentiation, and prognosis of the benign epithelial odontogenic tumor., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Pituicytomas are neoplasms that arise from pituicytes, which are specialized glia of the posterior pituitary. Pituicytes have 5 ultrastructural variants: light, dark, granular, ependymal, and oncocytic. Granular cell tumors of the pituitary gland are thought to arise from granular pituicytes. Spindle cell oncocytomas are considered to arise from folliculostellate cells, which are sustentacular cells of the adenohypophysis. Recent data suggest that, whereas pituicytes and all 3 tumor types are positive for TTF-1, folliculostellate cells are negative for TTF-1. We investigated 7 spindle cell oncocytomas, 4 pituicytomas, and 3 granular cell tumors for their genetic (BRAF(V600E) mutation and BRAF-KIAA fusion), immunohistochemical (GFAP, vimentin, S100 protein, olig2, IDH1-R132H, NF, galectin-3, chromogranin-A, CD56, EMA, CAM5.2, CD68, TTF-1, and bcl-2), and ultrastructural features to refine their classification. All tumors had nuclear positivity for TTF-1 and were negative for CAM5.2, chromogranin-A, and NF. GFAP, vimentin, S100, galectin-3, EMA, and CD68 were variably positive in the majority of the 3 tumor groups. Olig2 was only positive in 1 pituicytoma. Whereas granular cell tumors were negative for bcl-2 and CD56, pituicytomas and spindle cell oncocytomas showed variable positivity. All tumors were negative with the IDH1-R132H mutation-specific antibody, and none had evidence of BRAF alterations (BRAF(V600E) mutation and BRAF-KIAA fusion). Diffuse TTF-1 expression in nontumorous pituicytes, pituicytomas, spindle cell oncocytomas, and granular cell tumors indicates a common pituicyte lineage. The ultrastructural variants of pituicytes are reflected in these 3 morphologic variants of tumors arising from these cells. We propose the terminology "oncocytic pituicytomas" and "granular cell pituicytomas" to refine the classification of these lesions.

Granular cell tumor (GCT) was first described by Abrikossoff in 1926. GCT is a rarely seen soft tissue tumor and is generally benign. While the tumor can be seen in all parts of the body it is generally located on the head and neck region, and especially on the tongue. GCT is rarely seen in the anal-perianal region. In accordance with literature this case was reported because it was thought to be the 27th anal-perianal located GCT case. In this case report, approximately 0,5-1 cm pedunculated polypoid lesion was determined in the perianal region during the physical examination of a 23 year old female patient who applied with palpable mass complaint in the perianal region. Lesion in the patient was totally excited with healthy skin-subcutaneous tissue under local anesthesia. A benign granular cell tumor was detected in the histopathological examination. Positive staining was monitored immunohistochemically with S-100 and neuron specific enolase (NSE). GCT is a rarely seen tumor in the anal-perianal region and its malign transformation rate is very low. Even lesions seen in the perianal region have clinically a benign appearance, a histopathological examination should be conducted and also GCT should be kept in mind during diagnosis. Malign-benign separation of these lesions is difficult so histopathological examination should be conducted with great care. Large local excision in the treatment provides curative treatment. But for those presenting malign transformation further examination must be performed for metastasis. After the treatment local recurrence and metastasis should be considered carefully. Prognosis of metastatic disease is very bad.

Background: Granular cell tumor is an uncommon benign neoplasm with a predisposition for upper aerodigestive tract, skin and soft tissue involvement. Malignant and atypical granular cell tumors account for less than 2% of the lesions and in the pediatric population they are extremely rare and atypia has not been previously reported., Case: We present a case of a rapidly growing granular cell tumor of the vulva of a 12-year-old girl exhibiting atypical histology. The lesion demonstrated prominent Ki-67 proliferation index (up to 20%), localized areas of spindling of tumor cells, scattered apoptotic bodies and p53 overexpression., Conclusion: The current histologic diagnostic criteria of atypical granular cell tumors are evaluated while physician awareness and the need for follow-up of patients for potential recurrences of this rare entity are emphasized., (Copyright © 2013 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

Classic granular cell tumors (GCTs) stain strongly and uniformly positive for S100 protein and are believed to show Schwann cell derivation. Polypoid cutaneous tumors composed of cells with large nuclei and abundant granular cytoplasm that do not stain for S100 protein or show apparent Schwannian differentiation have been reported by several groups under names including "primitive polypoid granular cell tumors," "dermal nonneural granular cell tumor," and "primitive nonneural granular cell tumors of skin." We report a polypoid tumor composed of S100-negative epithelioid cells with abundant eosinophilic granular cytoplasm that meets diagnostic criteria for (primitive polypoid dermal) nonneural GCT but also meets criteria for a granular cell variant of epithelioid cell histiocytoma. We have identified a single previous report of a similar lesion. We report the immunohistochemical characteristics of these lesions and address how they are best classified.

Granular cell tumors (GCTs) are uncommon benign neoplasms deriving from Schwann cells of the peripheral nerve fibers. Although these tumors can be found anywhere in the body, the most frequent site is the tongue, followed by the chest wall and the arm. The abdominal wall is an extremely rare site for GCTs. These tumors are generally asymptomatic and have a slow growth rate. Today, thanks to their immunoreactivity to S-100 and CD68, the differential diagnosis is more straightforward than in the past. We report on a young patient affected by a GCT located in the upper third of the right rectus abdominis muscle. En bloc excision through a diamond-shaped skin incision allowed us to make a correct histological diagnosis, which was confirmed by the immunohistochemical findings. GCT, which is very rare in abdominal wall muscles, should be considered in the differential diagnosis, and surgical excision is the treatment of choice.

Congenital granular cell lesion of the newborn, also known as congenital epulis, is a rare benign oral cavity tumor presenting at birth. Usually, it appears as a solitary mass arising in the mouth and originates from the anterior alveolar ridge. The objective of the present article is to report a case of congenital granular cell lesion in an 8-day-old female newborn. The patient presented four intraoral pedunculated lesions. Diagnosis, treatment, microscopic and immunohistochemical characteristics are also discussed.

Granular cell tumors (GCT) are infrequent tumors first described by Abrikossoff in 1926. Gastrointestinal involvement occurs in about 6% of GCT, the esophagus being the most frequent location. These tumors are usually benign and asymptomatic and are usually found incidentally when an upper gastrointestinal endoscopy is carried out for another reason, showing an isolated polyp or sessile submucosal nodule, covered by intact yellowish mucosa and with firm consistency. Endoscopic ultrasonography has significantly improved the diagnosis of these lesions. Nowadays endoscopic mucosectomy is the treatment of choice of esophageal GCT with a low frequency of complications. Histologic analysis of the surgical specimen shows specific characteristics such as positivity for S-100 protein. We present two new cases of esophageal GCT that were diagnosed recently and discuss the most relevant features of this infrequent disease., (Copyright © 2011 Elsevier España, S.L. All rights reserved.)

Fanburg-Smith et al. classified granular cell tumors (GCTs) using six criteria with high Ki-67 and p53 in malignant cases. We aim to refine their classification and reproduce their immunohistochemical findings. We, first, classified our 48 cases according to Fanburg-Smith criteria (37 benign, seven atypical, and four malignant), and performed Ki-67 and p53 on a sample of tumors. Then, we reclassified them into 44 benign and four with uncertain malignant potential (GCT-UMP) using only necrosis and/or mitoses. (1) According to Fanburg-Smith criteria: Malignant cases were significantly younger than benign and atypical ones; occurred predominantly in males; were significantly larger in size; and showed a higher Ki-67 expression but an insignificant difference in p53 staining. (2) Comparative findings: The four malignant cases according to Fanburg-Smith corresponded to our four cases with UMP. The seven atypical cases and our benign group shared similar means, except for age. None of these atypical cases recurred or metastasized. Despite its small number, our preliminary study showed similar selectivity of two more reproducible criteria (vs six) in the classification of cases of GCT with potential aggressive behavior, preserving a role for Ki-67 in difficult cases. However, metastases remain the sole definite criterion for malignancy., (Published by Elsevier GmbH.)

Pediatric airway tumors are uncommon. A 4-year-old girl with history of stridor since the first year of life underwent rigid laryngotracheal endoscopy revealing a left posterolateral subglottic mass occluding 80% of lumen. Complete tumor resection by open approach was undertaken. Histopathologic and immunohistochemical studies revealed granular cells tumor. The similarity of the clinical and endoscopic presentation of this tumor to the much more frequent subglottic hemangioma that usually requires a different therapeutic approach was of interest. Granular cell tumor must be considered in the differential diagnosis of upper airway obstructive symptoms in infancy and childhood., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Background: Cutaneous granular cell tumors have only rarely been examined for Bcl-2, a marker that is expressed by granular cell tumors from other parts of the body., Objective: We retrospectively studied three cases of cutaneous granular tumors from our archives., Methods: We immunohistochemically tested for neuron-specific enolase (NSE), CD68 (KP1), Bcl-2, and S-100. In Cases 1 and 3, we also tested for CD34, HMB-45, and melan-A. In Case 3, we additionally tested for smooth muscle actin, CD68-PGM1, cytokeratin AE1-AE3, epithelial membrane antigen, desmin, CD1a, and CD117., Results: None of our cases presented any of the histological markers traditionally considered to be indicators of potential malignancy in granular cell tumors. All cases strongly expressed S-100, CD68, NSE, and Bcl-2. Case 3 also expressed CD68-PGM1. The rest of the markers were not expressed by the tumors., Conclusions: Bcl-2 is expressed by cutaneous granular cell tumors.

We report a 20-year-old woman who underwent complete resection of a granular cell tumor (GCT). On chest computed tomography (CT) scan, a mass with a maximum diameter of 36 mm at the lower bronchus with atelectasis of the right lower lobe was noted. Bronchoscopic examination revealed a whitish mass in the truncus intermedius, and the middle and lower bronchus were unable to be seen. A cytopathological examination of the mass revealed GCT. A right middle and lower lobectomy was performed via a posterolateral thoracotomy. Microscopically, the tumor was composed of polygonal cells with oxyphilic granular cytoplasm and small ovoid nuclei. The cytoplasm of the neoplastic cells was positive for S-100 protein and neuron-specific enolase. The patient's postoperative course was uneventful, and she was asymptomatic after 4 months. A large bronchial GCT is rare, which is why we report this case.

Introduction: Granular cell tumors (GCT) are uncommon soft tissue neoplasms of presumed neural origin that rarely involve the male external genitalia. Penile lesions are distinctly uncommon with less than 20 cases reported till now., Objective: In the present paper we describe the clinicopathological and immunohistochemical findings in a case of GCT of the penis shaft in a 31-years-old man., Results and Conclusions: On physical examination the patient was found to have a small ovoid mass, 20 x 10 mm in diameter, at the left postero-lateral area of the penis' base. The mass was firm on palpation with no fixation on the neighboring tissues. The lesion was completely excised under loco-regional anesthesia. The surgical specimen was an ovoid, gray-white, elastic mass, of 10 x 5 mm. Microscopically, the tumor was moderately cellular and was composed of polygonal-shaped cells with abundant granular eosinophilic cytoplasm. Tumor cells were disposed in nests, cords, and trabeculae and showed perineural invasion. The tumor presented bland cytological features with only focal slight nucleo-megaly. Mitotic activity was undetectable. The tumor cells showed diffuse immunohistochemical expression for S100 protein. At 6 month after surgery the patient was free of persistent/recurrent disease or metastatic spread of the tumor. We discuss the clinical, histo-immunohistochemical and therapeutical features of this unusual penile tumor, the single one encountered in the Department of Pathology from Timisoara County Hospital and, to our knowledge, the only one reported in the Romanian medical literature.

Background: Granular cell tumor (GCT), also known as Abrikossoff tumor, is an uncommon benign neoplasm, probably of neural origin derived from Schwann cells. It presents clinically as a solitary asymptomatic nodule and its pathogenesis has been the subject of much debate in the literature., Objectives: We aimed to analyze the clinical, histologic, and immunohistochemical characteristics associated with this tumor and to determine whether these findings correspond to those reported to date in the literature., Methods: In this retrospective study of 34 patients with histologic diagnosis of GCT, we analyzed clinical characteristics (site, age, sex, duration, and suspected diagnosis), histological findings (border, cell atypia and mitoses, involvement of adnexal structures, pseudoepitheliomatous hyperplasia, and presence of the recently described pustulo-ovoid bodies), and immunohistochemical findings (S-100 staining in 16 randomly selected cases)., Results: In total, 58.82 % were men and 41.18 % were women, and the mean age was 31.74 years. The most common site was the oral cavity (61.76 %). The most frequently suspected clinical diagnosis was fibroma (17.65 %). The lesion was poorly defined and diffuse in 85.29 %. Pseudoepitheliomatous hyperplasia was present in 58.82 %. Nuclear atypia was found in 29.41 % and mitoses in 20.59 %. One case was considered malignant (2.94 %) and 2 atypical (5.88 %). Pustulo-ovoid bodies were present in 47.06 % of the cases and S-100 staining was positive in all cases analyzed., Conclusions: Our series confirms the characteristics described previously for GCT, except for certain peculiarities, and supports the presence of pustulo-ovoid bodies as an additional histologic finding for diagnosis of this tumor.

Abrikosoff's tumor (AT), or granular cell tumor (GCT), is relatively rare in the gastrointestinal tract, where the most common site is the esophagus. This tumor is usually found incidentally when an upper gastrointestinal endoscopy is carried out for another reason. Endoscopically, GCT appears as a small, yellow and submucosal lesion covered by normal mucosa. Endoscopic ultrasonography shows a homogeneous hypoechoic lesion with well defined margins. The definitive diagnosis is histological. The origin of GCT is neurogenic and the tumor is composed of eosinophilic granular cytoplasm and PAS-positive cells, which show the S-100 protein on immunohistochemistry. Although GCT is usually clinically and histologically benign, some malignant cases have been reported. Consensus is lacking on the treatment and follow-up of this tumor. Currently, endoscopic mucosal resection is a safe and effective technique to treat submucosal esophageal lesions, allowing subsequent histologic analysis. We present three patients with esophageal CGT, who were definitively treated with endoscopic mucosal resection.

Hybrid peripheral nerve sheath tumors (PNSTs) are recently recognized, rarely reported entities. Very few hybrid PNSTs with elements of perineurioma have been reported. We report a case of a 47-year-old man who presented with multiple (>20) cutaneous nodules on all 4 extremities, present since childhood. The patient and his family had no history of other PNSTs, other neural-origin tumors, or genetic disorders, including neurofibromatosis. Five excised lesions are well circumscribed but unencapsulated nodular tumors composed of cytologically bland spindled cells with tapering nuclei and indistinct cytoplasm arranged in a storiform pattern in a collagenous matrix. Scattered among these cells are large granular eosinophilic cells. The spindled cells are epithelial membrane antigen-positive and S-100 protein negative. The cytoplasm of the granular cells contains abundant S-100 protein, NK1/C3, and CD68-positive granules. Sparse nerve fascicles contain neurofilament-positive axons. Each tumor also contains poorly marginated areas that histologically resemble pure granular cell tumor, and superficial biopsies of these tumors can histologically resemble pure granular cell tumors. Considering a Schwannian origin for both perineurial and granular cells can provide a logical explanation for the pathogenesis of this case. To the best of our knowledge, our case is the first report of multiple hybrid granular cell tumor-perineurioma.

The authors report on a case of a granular cell tumor of the parotid gland, which constitutes an extremely rare neoplasm. The infiltrative growth of the tumor may pose the question of malignancy, especially on frozen section. The objective of this report was to function as a reminder of this entity for every pathologist that deals with a biopsy from a lesion of the parotid gland, and furthermore, to keep the unwary away from a possible diagnostic pitfall. A meticulous search of the literature revealed only 4 similar cases.

Nonneural granular cell tumors (NNGCTs) are rare benign neoplasms originally described in 1991 by Leboit et al. Typical granular cell tumors (GCTs) are commonly encountered in the oral cavity, but NNGCTs, unlike GCTs, are S-100 negative and may display cytologic atypia, allowing for misdiagnosis as a more aggressive lesion. We report a case of a 43-year-old male with a lesion of the mandible that we believe to be the first intraoral example of an NNGCT.

Granular cell tumor of the urinary bladder is an exceedingly rare disease, with only 10 cases reported so far in literature. We report a case of granular cell neoplasm of the bladder incidentally found in a 44-year-old woman during an abdominal ultrasound examination for evaluation of stress urinary incontinence. The patient underwent a transurethral resection of the mass and histological examination revealed the presence of a granular cell tumor. Immunohistochemical staining for neuron-specific enolase and S100 protein was positive. At a 6-month follow-up the patient is free of bladder recurrence.

Background: Cutaneous granular cell tumor (GCT) may present with extension into the junctional region of the epidermis and thus may mimic melanocytic neoplasms., Methods: We reviewed three cases of cutaneous GCT where a melanocytic neoplasm was either initially diagnosed or considered in the differential diagnosis. Histopathology was evaluated in regards to features associated with melanocytic neoplasms. Immunohistochemistry was performed to delineate a panel useful in the distinction of these and other entities., Results: All cases consisted of spindle and epithelioid cells with granular cytoplasm and bland nuclei and were centered in the superficial dermis with extension into the epidermis. Two cases resembled Spitz nevi and one case demonstrated lentiginous growth. All cases stained positively with calretinin and inhibin. Two of the three cases stained diffusely with S100 and 2/2 cases with CD56. HAM56 and CD68 were positive in one case and another showed positivity for NSE and PGP9.5. HMB-45, tyrosinase, and Melan-A were non-reactive in all cases tested., Conclusions: GCT may involve the epidermis and has a growth pattern similar to melanocytic neoplasms. An immunohistochemical (IHC) panel including S100, Melan-A, tyrosinase, HMB-45, CD56, CD68, calretinin, inhibin, and PGP9.5 may aid in the distinction and may spare the patient from unnecessary morbidity.

Primitive nonneural granular cell tumor was recognized in an 82-year-old man, mimicking a granuloma in the scalp. Despite its disturbing cytomorphology, this histogenetically enigmatic tumor with unusual immunoprophile (NK1-C3, CD 68 and NSE positivity; S-100 protein negativity) behaves indolently.

We present a case of granular cell tumor (GCT) occurring in the esophagus 7 years after operation for bronchial GCT. A 59-year-old Japanese man complained of epigastralgia, and endoscopic examination of the upper digestive tract disclosed a submucosal tumor in the lower esophagus. Histological examination of the endoscopic mucosal resection of the esophageal tumor showed a proliferation of neoplastic cells with an eosinophilic and granular cytoplasm. The cytoplasm of the neoplastic cells was histochemically positive for PAS stain and immunohistochemically positive for S-100. This tumor did not fulfill any of the diagnostic criteria for malignancy at either the macroscopic or microscopic level. I believe that this is the first case of GCT occurring metachronously in the respiratory and digestive tracts. Clinicians and pathologists should bear in mind that GCT may arise metachronously in the respiratory and digestive tracts.

We report a rare case of granular cell tumor (GCT) arising within the thyroid parenchyma. The differential diagnosis of GCT in thyroid includes all oncocytic/Hürthle cell lesions of the thyroid, medullary carcinoma and metastasis. The classic histomorphologic features and immunohistochemistry are helpful in arriving at the correct diagnosis.

The granular cell tumor is an uncommon tumor that usually appears as a solitary small nodular growth and runs a benign course. It occurs widely throughout the body, but is rarely described in the abdominal wall. The authors report a case of malignant granular cell tumor which was arising in anterior abdominal wall of a 67-year-old woman. Malignant variant is rare and the abdominal wall site is extremely uncommon. Regarding this clinical case and the literature the authors purpose to review the criteria of malignancy.

Granular cell tumors are generally benign oncocytoid lesions of schwannian origin that are often incidental findings in many locations. Gastrointestinal stromal tumors occur in older adults and express the c-Kit protein (CD117). Both of these tumors have been described in association with many other entities; however, they have never been reported to occur jointly. This report is prompted by the simultaneous appearance of 2 granular cell tumors, a gastrointestinal stromal tumor, and a gastric adenocarcinoma in a 65-year-old woman with a history of breast carcinoma and granular cell tumor. To our knowledge, this is the first case report of these tumors occurring simultaneously.

We report on two patients with granular cell atypical fibroxanthoma. Both neoplasms were solitary, light-tan, dome-shaped papules on sun-exposed areas of the head in two elderly white men. Microscopically, these neoplasms showed a dermal proliferation of pleomorphic granular cells with irregular hyperchromatic nuclei, multinucleated cells, and scattered mitoses. Immunohistochemical stains were positive for CD68 and vimentin and negative for Melan-A or human melanoma black (HMB)-45, S-100 protein, pancytokeratin, and actin, consistent with atypical fibroxanthoma. The differential diagnosis of granular cells in neoplasms containing cytological pleomorphism is challenging in view of the many different neoplasms that may present with granular cytoplasm. These include the conventional granular cell tumor and its malignant form, leiomyoma, leiomyosarcoma, dermatofibroma, dermatofibrosarcoma protuberans, and angiosarcoma.

The occurrence of granular cell tumor (GCT) in penile tissue is very rare, with only 9 examples reported to date in the English-language literature. Herein, we describe the clinicopathologic and immunohistochemical findings in 9 additional cases. The patients ranged in age from 20 to 60 years (mean, 42 years; median, 40 years) at time of diagnosis. All penile tumors were solitary and arose in the dermis of the penile shaft (n=4), prepuce (n=3), and corona (n=2). A patient had a history of multiple cutaneous GCTs. Duration of symptoms before surgery ranged from 5 days to 2 years with the presence of an asymptomatic nodule representing the most common tumor-related complaint (n=8). The lesions ranged in size from 0.6 to 2.5 cm (mean, 1.5 cm; median, 1.5 cm). Microscopically, the tumors were moderate to highly cellular and were composed of oval to polygonal-shaped cells with abundant coarsely granular eosinophilic cytoplasm. Tumor cells grew in infiltrating nests, cords, and trabeculae and showed neural (n=2) and vessel wall (n=1) invasion or formed a relatively well-marginated solid nodule. Bland cytological features with only rare cells showing nucleomegaly (n=7) or spindling (n=3) were exhibited by 8 tumors. A tumor demonstrated diffuse nuclear atypia and was classified as "atypical." Mitotic activity ranged from 0 to 8 mitoses (mean, 1.4 mitoses) per 50 high-powered fields with no atypical division figures identified. All tumors tested showed moderate to strong immunohistochemical expression of S100 protein (n=6) and low-affinity nerve growth factor receptor (n=5), which was useful for detecting small deposits of tumor and helpful in evaluating surgical margins. Focal tumor cell immunoreactivity was observed for calretinin (4/6 cases) and glial fibrillary acidic protein (1/6 cases). All patients underwent simple (local) excision of their tumor. Complete follow-up data (mean, 21 years; interval range, 0.5-28 years) were available for 6 patients. No patient experienced recurrence or metastatic spread of tumor although surgical margins were microscopically involved by tumor in 5 cases. Benign GCT involving superficial soft tissue of the penis can be adequately managed by a simple excision. Patients with microscopically involved surgical margins can be clinically followed without immediate additional surgery.

Context: Granular cell tumor (GCT) is a rare tumor of nerve sheath origin with a predilection for upper aerodigestive tract, skin, and soft tissue. The neoplastic cells typically express S100 and CD68 (KP-1), the latter due to cytoplasmic lysosome content. However, the histogenesis of this tumor is unknown. Additionally, distinction between benign and malignant GCT is difficult because of histologic similarity and lack of reliable criteria that can predict clinical behavior., Objective: To perform a comparative, side-by-side immunohistochemical assessment of the traditional immunohistochemical markers for GCTs (S100, CD68), along with the newer markers (inhibin-alpha, protein gene product 9.5) for these tumors., Design: To address diagnostic and prognostic issues, we studied 30 specimens of GCT (27 primary and 3 recurrent tumors, 2 of which occurred consecutively in the same patient) for (1) nuclear pleomorphism, prominent nucleoli, necrosis, spindling, high nuclear-cytoplasmic ratio, and mitoses; (2) immunohistochemical expression of inhibin-alpha, protein gene product 9.5, S100, CD68 (KP-1), and Ki-67 using the avidin-biotin complex method on formalin-fixed, paraffin-embedded sections; and (3) correlation between tumor grade, proliferative fraction, and clinical data., Results: Twenty-seven of 27 primary GCTs and 1 of 3 recurrent GCTs had typical histologic features, while the 2 consecutive recurrent GCT specimens from the same patient were atypical (moderate nuclear atypia and prominent nucleoli alone). The mean age for primary GCT was 37.3 years (range, 5-67 years), and mean size was 1.89 cm. None of the cases metastasized. All 30 specimens showed diffuse (2+ to 3+) staining for S100, CD68, and inhibin-alpha, and 3+ staining for protein gene product 9.5; pseudoepitheliomatous hyperplasia was nonreactive. The Ki-67 proliferative index was less than 1% to 20% in typical nonrecurrent cases, 1% in the typical recurrent case, and 1% and 10% in 2 sequential recurrences of the atypical case., Conclusion: Our study expands the immunophenotype of GCT (S100, CD68, protein gene product 9.5, and inhibin-alpha) regardless of location and supports a neural origin. Intensity of immunohistochemical staining had no prognostic significance. Although 1 of the 2 recurrent GCTs had atypical features, the Ki-67 proliferative index did not distinguish reliably between typical (nonrecurrent) and atypical or recurrent GCTs. The significance of inhibin expression with regard to cell differentiation and pathogenesis is unclear and warrants further investigation.

Two cases of compound melanocytic nevi with granular cell changes were studied. Histological examination revealed compound melanocytic nevi with a portion of the dermal component exhibiting a sheet like proliferation of nevoid cells with granular cytoplasm. Immunohistochemically, both populations of cells stained positive for S-100 and Melan-A. However, HMB-45 was positive only for the nevus cells with granular cytoplasm. We believe this represents granular cytoplasmic changes of nevus cells and does not denote malignant transformation.

A granular cell tumor (GCT) in a 39-year-old white man is reported. It was localized in the intrapancreatic part of the common bile duct and caused obstruction of the bile downflow. The patient underwent radical surgical procedures because a malignant tumor was clinically suspected. Macroscopically, the tumor appeared as a duct stricture caused by diffuse infiltration of neoplastic cells in the walls. In the cytoplasm smaller and larger PAS-positive granules were present and constantly reactive to S-100 and NSE antibodies. Ultrastructurally, cytoplasmic granules appeared as membrane-bound vacuoles of variable size and shape containing debris, disrupted mitochondria, and myelin figures. No basal lamina around cell cytoplasm was observed. GCTs are relatively uncommon soft tissue tumors usually presenting in the skin and subcutaneous tissues or tongue. The prognosis in any location is quite good, but very rare malignant GCTs (1-2%) are documented. Complete excision reduces the risk of recurrence. Accurate operative diagnosis seems to be critical when the tumors are located in the intrapancreatic common bile duct as in this reported case. Gastro-pancreatico-duodenectomy is too radical a procedure for such a benign lesion and additional assessments and investigations are recommanded before such an extensive radical surgery.

Granular cell tumors (GCT) are infrequently found in the gastrointestinal tract (GIT), and only four previous reports have described lesions occurring simultaneously in different sites. The present case of 11 GCT, located in the esophagus, stomach, colon and pericolic adipose tissue, occurred in a 50-year-old Japanese woman. All GCT appeared histologically benign and there was no sign of recurrence at 3 years after surgery. Immunohistochemical analysis and comparison between this case of multifocal GCT and six cases of solitary benign GCT of the GIT, which were taken from the files of the Department of Pathology at Kitasato University (1986-2000), demonstrated the follow-ing: (1) all diffusely expressed S-100, DCC and bcl-2, and (2) median labeling indices for Ki-67, cyclin D1, p53 (Pab1801), and p21WAF1/CIP1 of 4%, 24%, 1% and 28%, respectively, for the multifocal tumors, and 3.5%, 23%, 1% and 29%, respectively, for the solitary lesions, with no significant difference between the two groups. Thus, the expression of cyclin D1 and p21WAF1/CIP1 may be involved in the tumorigenesis of both types of GCT. The present case emphasizes the need to evaluate the entire GIT when a single GCT is identified. Multifocal lesions should be treated conservatively by local excision because, as with the solitary tumors, they exhibit a benign biological behavior, consistent with their low Ki-67 immunoreactivity.

This is a report of a case of an S-100-negative polypoid granular cell tumor arising in the lower lip of a 4-year-old patient that may represent the entity primitive polypoid granular cell tumor. Unlike the usual granular cell tumors arising in the tongue or buccal mucosa, such lesions are not S-100 positive and can exhibit mitoses and cytologic atypia, features that may suggest a low-grade mesenchymal malignancy necessitating aggressive therapy. However, this is in fact a nonaggressive neoplasm that is not associated with significant morbidity and therefore should be treated with simple excision.

A 57-year-old woman presented with a 2-year history of a palpable mass in the upper inner quadrant of the right breast. A 1.1-cm, poorly circumscribed, firm tumor nodule was noted, consisting of 2 histologically distinct lesions in the same location, with some areas showing purely well-differentiated invasive ductal carcinoma and others composed of granular cell tumor. In 1 area, the 2 tumors collided and infiltrated each other. The invasive ductal carcinoma was admixed with ductal carcinoma in situ of solid and cribriform types. To our knowledge, this is the first case report demonstrating colocalization of these 2 neoplasms, which raises questions regarding causal relationship. We also review the literature on granular cell tumor of the breast.

Background: Granular cell tumor (GCT) is a relatively uncommon soft tissue tumor of putative Schwann cell origin. This tumor can occur in multiple sites as a small, nontender nodule, but the parotid gland is unusual, and only several cases have been reported., Case: A 46-year-old woman presented with a slowly growing mass in the left preauricular region for three years. Imaging studies confirmed a nodular lesion in the superficial lobe of the left parotid gland. Fine needle aspiration (FNA) cytology revealed scattered cellular clusters and single cells with abundant granular cytoplasm and indistinct cell borders. Background exhibited eosinophilic, granular, cytoplasmic material, and some scattered naked nuclei were also noted. Histologic examination with supportive immunohistochemical and ultrastructural studies confirmed GCT., Conclusion: GCT of the parotid gland is very unusual. Recognition of this tumor is important to make a definitive diagnosis before an operation. FNA is useful procedure in GCT of parotid gland for a preoperative diagnosis and proper treatment.