Granular Cell Tumor: A Clinical Study of 81 Patients. / Tumor de células granulares. Estudio clínico de 81 pacientes.

INTRODUCTION: Although a granular cell tumor (GCT) usually develops on the skin or oral mucosa, it has been described in many other organs. GCT typically presents as a solitary tumor, but multiple lesions can occur. It has also been described in association with other diseases. OBJECTIVES: To describe the clinical characteristics of cutaneous and oral mucosal GCTs and explore potential associations with other diseases. MATERIAL AND METHODS: Retrospective study of patients diagnosed with GCT at our hospital between 1995 and 2019. The following information was collected from the patients' medical records: age, sex, number of GCTs, location, diameter, time to diagnosis, tentative clinical diagnosis, surgical margin status, recurrence, follow-up time, and associated diseases. RESULTS: We detected 89 cutaneous or oral mucosal GCTs in 81 patients (43 women, 38 men) with a mean age of 40.21 years. The mean tumor diameter was 1.34cm. Five of the 81 patients (6.2%) had multiple GCTs, including noncutaneous tumors. Patients with multiple GCTs were on average younger than those with a single tumor (P=.004). There was only a single case of local recurrence and no cases of distant metastasis. None of the patients had associated diseases. CONCLUSIONS: Most GCTs are benign and local recurrence is uncommon, even in patients with positive margins. Nevertheless, the possibility of multiple tumors affecting the skin, oral mucosa, or internal organs should be borne in mind, especially in young patients.

Tumor de células granulares intratiroideo / Intra-thyroid granular cell tumor

Fundamento: los tumores de células granulares son lesiones benignas infrecuentes de crecimiento lento que pueden aparecer en cualquier sitio del organismo. La región de cabeza y cuello incluye las localizaciones más frecuentes (entre el 30 y el 50 porciento de todos los tumores de este tipo, hasta los del sistema nervioso central).Objetivo: presentar un caso de tumor de células granulares intratiroideo. Caso clínico: se presenta el caso de una paciente femenina de 3 años de edad con enfermedad nodular de la glándula tiroides de crecimiento lento con escasa sintomatología, con confirmación histológica tumor de células de la granulosa. Al examen físico se identificó aumento de volumen en la región anterolateral del cuello, en relación con el lóbulo derecho de la glándula tiroidea, que correspondía con lesión tumoral de 3 cm, mal delimitada, adherida a planos profundos, no dolorosa a la palpación. En la ecografía se observó: lóbulo derecho del tiroides de 4,3 cm x 1,2 cm x 1,4 cm, no homogéneo con lesión nodular de 2,8 x 1,4 cm mal delimitada, de bordes irregulares. Se realizó biopsia transoperatoria que se reportó como positiva de células neoplásicas malignas, sin embargo, el estudio histológico extemporáneo reveló un tumor benigno de células granulares intratiroideo. El tratamiento quirúrgico es curativo en este caso. Conclusiones: el diagnóstico de tumor de células granulares intratiroideo benigno, se presenta con las características de una lesión tumoral maligna, debe tenerse en cuenta en el diagnóstico diferencial de los tumores de la glándula tiroides(AU)

Background: the granular cells tumors are uncommon benign lesions of slow growth that can appear in any place of the organism. The head and neck region includes the most frequent localizations (between the 30 and 50 percent of all the tumors of this type, including those of the central nervous system). Objective: to present a case of intra-thyroid granular cell tumor.Clinical case: a case of a 37 year-old female patient with nodular illness of the thyroid of slow growth with scarce symptoms, with histologic confirmation of granular cells tumor is presented. To the physical exam, an increase of volume was identified in the anterolateral region of the neck, in connection with the right lobe of the thyroid gland that corresponded with a tumoral lesion of 3cm approximately, not well defined, stuck to deep planes, not painful to the palpation. In the echography, it was observed: right lobe of the thyroid of 4.3 cm x 1.2cm x 1.4 cm, not homogeneous with a nodular lesion of 2.8 x 1.4 cm not well defined, of irregular borders. During the surgery, a frozen section was examined and reported as positive of malignancy, however, the untimely histologic study revealed a benign intra-thyroid granular cell tumor. The surgical treatment is healing in this case. Conclusions: the diagnosis of intra-thyroid benign granular cell tumor is presented with the characteristics of a malign tumor, it should be kept in mind in the differential diagnosis of the tumors of the thyroid(AU)

Granular cell tumor of the infundibulum: a systematic review of MR-radiography, pathology, and clinical findings.

INTRODUCTION: Granular cell tumors (GCTs) of the infundibulum are rare in practice and literature, resulting in a lack of evidence-based standard of care. We present two characteristic cases from our institution and perform a systematic review of the existing literature to further elucidate the presentation of this tumor and guide management. METHODS: A systematic literature search was conducted according to PRISMA guidelines, yielding 42 total individual reported GCTs suitable for evaluation. Available clinical presentation, magnetic resonance imaging (MRI) characteristics, pathology, surgical approaches, and outcomes were charted. We measured frequencies of clinical characteristics and performed an outcome comparison of open versus endoscopic surgical treatment. RESULTS: In this pooled dataset, GCT incidence was higher in females than males (3:1). Clinical presentation peaked in the fourth decade with tumor-related symptoms. MRI appearance was characterized by T1 isointensity (50%) and T2 hypointensity or isointensity (52%) with gadolinium contrast enhancement (74%). Histopathology demonstrated positive staining for PAS, PAS-D, S100, CD68, and TTF1. In a simple uncontrolled analysis, patients who underwent endoscopic surgery experienced more symptom improvement (p = 0.006) and lower incidence of new diabetes insipidus postoperatively (p = 0.047) versus patients who underwent open microsurgery. CONCLUSIONS: This first comprehensive review of GCTs of the infundibulum corroborates existing data and adds significant new MR-radiological information to the literature, notably a typical tumor appearance of T1 isointensity, T2 iso- to hypointensity, and gadolinium contrast enhancement. Future prospective studies should be conducted to validate our findings.

Granular cell tumors of the tongue: fibroma or schwannoma.

BACKGROUND: Granular cell tumors are benign lesions that typically occur in the oral cavity, but can also be found in other sites. However, the characteristics of these tumors are unclear. Thus, the present study aimed to investigate the immunohistological characteristics of these tumors of the tongue. METHODS: Seven patients were treated for granular cell tumors of the tongue at our institution during 2003-2017. Paraffin-embedded specimens were available for all cases; thus, retrospective immunohistochemical analyses were performed. RESULTS: All cases exhibited cytoplasmic acidophilic granules in the muscle layer of the tumor. Both the normal nerve cells and tumor cells also stained positive for PGP9.5, NSE, calretinin, and GFAP. A nucleus of tumor cells was typically present in the margin. The PAS-positive granules were also positive for CD68 (a lysozyme glycoprotein marker). Various sizes of nerve fibers were observed in each tumor, and granular cells were observed in the nerve fibers of a representative case. CONCLUSIONS: Based on our immunohistological findings, granular cell tumors may be derived from Schwann cells, and the presence of CD68 indicates that Wallerian degeneration after nerve injury may be a contributor to tumor formation. Thus, a safe surgical margin is needed to detect the infiltrative growth of granular cell tumors.

Epidemiology of malignant cutaneous granular cell tumors: A US population-based cohort analysis using the Surveillance, Epidemiology, and End Results (SEER) database.

BACKGROUND: Malignant cutaneous granular cell tumors (mcGCTs) are rare and associated with substantial morbidity and mortality. The literature includes single-institution studies. OBJECTIVE: To examine the incidence, secondary malignancies, treatment, overall survival, and disease-specific survival (DSS) of patients with mcGCT. METHODS: A population-based cohort analysis was conducted in the Surveillance, Epidemiology, and End Results database from 1973 to 2013 for patients with a diagnosis of mcGCT. Risk-adjusted associations between overall survival/DSS and patient characteristics and treatment modalities were assessed by Cox proportional hazard regression. Quantile regression was used to determine median survival times. RESULTS: The 5-year DSS rate was 62.8%. Patients demonstrated an increased risk for renal and pancreatic cancers. In risk-adjusted models, male sex (hazard ratio [HR], 0.21; 95% confidence interval [CI], 0.06-0.82; P = .02), advanced cancer stage (HR, 2.29; 95% CI, 1.40-3.72; P < .01), and surgical resection (HR, 0.06; 95% CI, 0.01-0.59; P = .02) predicted DSS. Median survival time in years increased for males (1.39), earlier stage (0.60), and surgical intervention (5.34). LIMITATIONS: Absent or incorrect reporting in retrospective Surveillance, Epidemiology, and End Results data is possible. The database is more likely to include academic centers. Some subanalyses may be underpowered because of the limited sample size for a rare cancer. CONCLUSIONS: Our study presents an in-depth assessment of factors that identify high-risk patients. Residency in a nonmetro area, black race, female sex, and no surgical resection were each associated with poorer DSS.

Extent of surgical resection and tumor size predicts prognosis in granular cell tumor of the sellar region.

BACKGROUND: Rarely, granular cell tumor (GCT) occurs in the sellar region. The natural history of this clinical entity is not well known. This study sought to determine the clinical features and long-term outcome of GCT of the sellar region. METHODS: Institutional databases, the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) database, and PubMed/EMBASE were searched for GCT of the sellar region. Patient-level data were extracted where available, including age, sex, symptoms, tumor size and location, presumed diagnosis, surgical approach and result, subtotal resection (STR) and gross-total resection (GTR), use of radiation, and outcome. The primary endpoints of recurrence and survival were determined. RESULTS: A total of 141 cases were analyzed. The mean age at diagnosis was 48.9 (SD 15.3) with a female predominance (sex ratio 1.49:1). Almost all patients experienced either or both neurological and endocrine symptoms. The most common pre-operative diagnosis was pituitary adenoma. Approximately 60% of patients were treated with surgery, 57.7% with a craniotomy, and 39.7% with a transsphenoidal approach. The 5-year overall survival (OS) was 84.7% (standard error, SE 4.2). Patients with tumors less than 2.5 cm experienced a greater 5-year OS (100.0%) than patients with tumors 2.5 cm or larger (74.0%, SE: 11.7), (Mantel-Cox, p = 0.024). GTR resulted in a greater 5-year OS (95.7%, SE 4.3) than STR (88.8%, SE 5.5) and no surgery or biopsy alone (75.0%, SE 15.3) (Mantel-Cox, p = 0.016). The use of radiation therapy did not improve OS. The 5-year progression-free survival rate was 80.8% (SE 9.2). CONCLUSIONS: Granular cell tumor of the sellar region is a relatively benign neoplasm with neurological and endocrine clinical manifestations. Surgical resection is appropriate for management. Tumor size and extent of resection influence overall survival.

Pituicytoma, spindle cell oncocytoma, and granular cell tumor: clarification and meta-analysis of the world literature since 1893.

BACKGROUND AND PURPOSE: Pituicytoma, SCO, and GCT are poorly understood entities with confusing nomenclature and undetermined imaging characteristics. Our purpose was to confirm published cases of pituicytoma, SCO, and GCT with the newest 2007 World Health Organization criteria and elucidate imaging findings that distinguish these tumors from common entities such as pituitary adenoma. MATERIALS AND METHODS: A literature search identified 145 published cases (81 GCTs, 48 pituicytomas, and 16 SCOs). Case diagnoses were blindly reviewed by a neuropathologist according to the latest WHO criteria, resulting in 112 pathologically documented cases (64 GCTs, 35 pituicytomas, and 13 SCOs). Imaging illustrations from proved cases were reviewed to determine location, configuration, attenuation and signal intensity, and enhancement characteristics. RESULTS: Only pituicytomas presented as purely intrasellar lesions (7/33). Most GCTs were purely suprasellar (28/45). All SCOs were both intra- and suprasellar (13/13). Twenty-five percent of pituicytomas (6/22) and GCTs (7/30) appeared separate from the pituitary gland. All SCOs were infiltrating. Seventy-nine percent of entities appeared isointense to brain on T1-weighted image (34/43). Seventy-four percent of pituicytomas enhanced homogeneously (14/19). Twelve of 23 GCTs and 5/7 SCOs enhanced heterogeneously. Most GCTs were hyperattenuated to brain on CT (18/20). Eleven of 13 cases enhanced homogeneously. Visual disturbances were common symptoms for all entities (67/112). Diabetes insipidus was rare (4/112). CONCLUSIONS: Pituicytoma may be considered for purely intrasellar masses that are clearly separate from the pituitary gland. GCT should receive consideration for purely suprasellar lesions that are hyperattenuated to brain on CT. SCO should be considered for infiltrating pituitary masses with a mixed intra- and suprasellar location. A history of diabetes insipidus helps to exclude these tumors.

[Granular cell tumor of the esophagus: description of an infrequent benign tumor]. / Tumor esofágico de células granulares: descripción de un tumor benigno poco frecuente.

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.

Peripheral nerve sheath tumors of the gastrointestinal tract: a multicenter study of 58 patients including NF1-associated gastric schwannoma and unusual morphologic variants.

The frequency and morphological spectrum of gastrointestinal peripheral nerve sheath tumors (PNSTs) from consecutive case material has not been studied in the c-KIT era. We reviewed all mesenchymal gastrointestinal (GI) lesions at our departments according to current diagnostic criteria. PNSTs formed the third commonest group of mesenchymal GI tumors with a lower frequency (< or =5%) compared to gastrointestinal stromal tumors (GISTs; approximately 50%) and smooth muscle neoplasms ( approximately 30%). Granular cell tumors (GCTs; n = 31) and schwannomas (n = 22) were the most common types of PNSTs encountered. Rare tumors included neurofibromatosis 1 (NF1)-associated PNSTs (n = 5) and gastric perineurioma (n = 1). Thirteen schwannomas (including also some recent cases) were initially diagnosed as GIST, leiomyoma, or neurofibroma. Unusual histological variants included sigmoid GCT with prominent lipomatous component (n = 1), reticular-microcystic schwannoma of small (n = 1) and large (n = 1) bowel, NF1-associated gastric schwannoma (the first case to date), and psammomatous melanotic colonic schwannoma unrelated to Carney complex (n = 1). PNSTs coexisted with GIST in four patients (three had definite NF1). In conclusion, PNSTs of the GI tract are rare uniformly benign neoplasms that may show schwannian, perineurial, fibroblastic, or mixed differentiation. Most of them (92%) occurred sporadically unassociated with NF1 or NF2. Gastrointestinal PNSTs are still underrecognized by general pathologists. Awareness of their diverse morphology will help to avoid confusing them with smooth muscle neoplasms and GIST that they may closely mimic.

[Cutaneous granular cell tumor: a clinical and pathologic analysis of 34 cases]. / Tumor de células granulares cutáneo: análisis clínico-patológico de treinta y cuatro casos.

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.

A review of Granular cell tumours at the University Hospital of the West Indies: 1965-2006 / Un estudio de los tumores de células Granulares en el Hospital Universitario de West Indies: 1965&2006

OBJETIVE: Granular cell tumours are uncommon lesions that occur in a wide variety of sites. They are usually benign, but as they are infrequently diagnosed preoperatively, they may be confused clinically with malignant lesions. The objective of this study was to assess the relative frequency and the clinico-pathologic characteristics of granular cell tumours identified at the University Hospital of the West Indies (UHWI) over a 41-year period. METHODS: The archives of surgical pathology reports in the Department of Pathology at the UHWI from 1965 to 2006 were searched for all cases of granular cell tumour. From these records, a number of demographic and other data were recorded and analyzed. RESULTS: One hundred and thirty cases of granular cell tumours were found in 122 patients. Of these, 99 patients were female and 23 male, providing a male: female ratio of 1 to 4.3. The ages ranged from 5 days to 82 years with a mean age (excluding the 2 youngest cases) of 34.4 years. Lesions ranged in size from 0.2 cm to 10 cm in greatest dimension, the average size being 1.85 cm and were found in a diverse array of anatomic locations, the most common being the vulva, breast and tongue. The correct clinical diagnosis was proffered preoperatively in only one case. In contrast, a malignant diagnosis was suggested in 19 cases. CONCLUSIONS: Compared with other studies, there was a notable difference in the distribution of granular cell tumours in this series. In particular, lesions of the tongue accounted for fewer than expected, while lesions of the breast and vulva were considerably increased. The well-recognized female predominance was also substantially higher than in other studies.

OBJETIVO: Los tumores de células granulares son lesiones poco comunes que ocurren en una amplia variedad de lugares. Por lo general son benignos, pero como son raramente diagnosticados preoperativamente, puede confundírseles clínicamente con lesiones malignas. El objetivo de este estudio fue evaluar la frecuencia relativa y las características clínico-patológicas de los tumores de células granulares identificados en el Hospital Universitario de West Indies (HUWI) en un período de 41 años. MÉTODOS: Se investigaron los archivos de los reportes de patologías quirúrgicas en el Departamento de Patología de la UHWI, de 1965 a 2006, en busca de todos los casos de tumores de células granulares. A partir de estos archivos, se registraron y analizaron un número de datos demográficos y de otra índole. RESULTADOS: Se hallaron un total de ciento treinta casos de tumores de células granulares en 122 pacientes. De estos, 99 pacientes era mujeres y 23 hombres, para una proporción varón: hembra de l a 4.3 Las edades estuvieron en un rango de 5 días a 82 años con una edad promedio (excluyendo los dos casos de menos edad) de 34.4 años. El tamaño de las lesiones fluctuó de 0.2 cm a 10 cm como la mayor dimensión, siendo el tamaño promedio 1.85 cm. Estas lesiones se encontraban distribuidas en una variedad de lugares anatómicos, siendo los más comunes la vulva, el pecho y la lengua. El diagnóstico clínico correcto fue presentado preoperativamente sólo en un caso. En contraste con ello, se sugirió un diagnóstico maligno sólo en 19 casos. CONCLUSIONES: En comparación con otros estudios, hubo una notable diferencia en la distribución de los tumores de células granulares en este serie. En particular, las lesiones de la lengua dan cuenta de menos casos de los que se esperaba, mientras que las lesiones del pecho y la vulva presentaban un aumento considerable. El bien reconocido predominio femenino fue sustancialmente más alto que en otros estudios.

Tumor de células granulares cutáneo: análisis clínico-patológico de treinta y cuatro casos / Cutaneous Granular Cell Tumor: A Clinical and Pathologic Analysis of 34 Cases

Introducción: El tumor de células granulares cutáneo (TCG) o de Abrikossoff es una neoplasia benigna e infrecuente, probablemente de origen neural a partir de las células de Schwann. Clínicamente se manifiesta como un nódulo solitario y asintomático, y su etiopatogenia ha estado ampliamente debatida en la literatura. Objetivos: Analizar las características clínicas, histológicas e inmunohistoquímicas asociadas a este tumor y determinar si estos hallazgos se corresponden con lo descrito hasta el momento en la literatura. Métodos: Se realiza un estudio retrospectivo de treinta y cuatro casos con diagnóstico histológico de TCG donde se analizan las características clínicas (localización, edad, sexo, tiempo de evolución y sospecha diagnóstica), histológicas (delimitación, atipia, mitosis, afectación de anejos cutáneos, hiperplasia seudoepiteliomatosa y presencia de los recientemente descritos cuerpos pústulo-ovoides [CPO]) e inmunohistoquímicas (tinción S-100 en 16 casos seleccionados aleatoriamente). Resultados: El 58,82 % fueron hombres y el 41,18 % mujeres. La edad media fue de 31,74 años. La localización más frecuente fue la cavidad oral (61,76 %). El diagnóstico clínico más comúnmente sospechado fue el de fibroma (17,65 %). En un 85,29 % se presentaba como una lesión mal delimitada y difusa. La hiperplasia seudoepiteliomatosa estuvo presente en el 58,82 %. Se encontraron atipias nucleares en el 29,41 % y mitosis en un 20,59 %. Hubo un caso maligno (2,94 %) y dos se consideraron atípicos (5,88 %). Los CPO estuvieron presentes en el 47,06 %. La tinción con S-100 fue positiva en el 100 % de los casos analizados. Conclusiones: Nuestra serie confirmaría las características descritas hasta el momento para el TCG salvo con algunas peculiaridades y apoyaría la existencia de los CPO como un nuevo hallazgo histológico adicional para el diagnóstico de este tumor (AU)

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 (AU)

Tumours of peripheral nerves in the upper extremity: a 22-year epidemiological study.

Peripheral nerve tumours are uncommon. Our aims were to calculate the incidence and relative frequencies, to define sites of nerve tumours and to judge preoperative symptoms and outcomes of intervention. The results of 53 patients, with 68 tumours and histopathological diagnoses of true neoplasms, who had been operated on at the Department of Hand Surgery, Malmo, Sweden, between 1986 and 2007, were analysed. Schwannomas were the most common tumour (n=42). The incidence of schwannomas was 0.62/100 000 inhabitants/year in Malmo during that time period. The median nerve was most affected, closely followed by the ulnar and digital nerves. The preferred sites were the forearm, the thumb, and the digits. The most common preoperative symptom was pain. Loss of sensation was the most common postoperative complication. However, 33/53 patients (62%) were completely free of symptoms after excision. Patients should be provided with meticulous information preoperatively.

A review of granular cell tumours at the University Hospital of the West Indies: 1965-2006.

OBJECTIVE: Granular cell tumours are uncommon lesions that occur in a wide variety of sites. They are usually benign, but as they are infrequently diagnosed preoperatively, they may be confused clinically with malignant lesions. The objective of this study was to assess the relative frequency and the clinicopathologic characteristics of granular cell tumours identified at the University Hospital of the West Indies (UHWI) over a 41-year period. METHODS: The archives of surgical pathology reports in the Department of Pathology at the UHWI from 1965 to 2006 were searched for all cases of granular cell tumour. From these records, a number of demographic and other data were recorded and analyzed. RESULTS: One hundred and thirty cases of granular cell tumours were found in 122 patients. Of these, 99patients were female and 23 male, providing a male:female ratio of l to 4.3. The ages ranged from 5 days to 82 years with a mean age (excluding the 2 youngest cases) of 34.4 years. Lesions ranged in size from 0.2 cm to 10 cm in greatest dimension, the average size being 1.85 cm and were found in a diverse array of anatomic locations, the most common being the vulva, breast and tongue. The correct clinical diagnosis was proffered preoperatively in only one case. In contrast, a malignant diagnosis was suggested in 19 cases. CONCLUSIONS: Compared with other studies, there was a notable difference in the distribution of granular cell tumours in this series. In particular lesions of the tongue accounted for fewer than expected, while lesions of the breast and vulva were considerably increased. The well-recognized female predominance was also substantially higher than in other studies.

Granular cell tumor of the breast: a series of 17 cases and review of the literature.

Seventeen cases of granular cell tumor (GCT) of the breast are reviewed. The demographics and clinical features are reviewed and the radiologic and pathologic features as well as the immunohistochemistry are discussed. To our knowledge, our series of 17 breast GCT cases represent the largest series published to date.

Granular cell tumor of the trachea in pregnancy: a case report and review of literature.

OBJECTIVES/HYPOTHESIS: The objectives were to present a case report of a woman with recurrent disease during consecutive pregnancies and to discuss the diagnosis and management of granular cell tracheal tumors, with particular attention to pregnancy and hyperestrogenic states. STUDY DESIGN: Case report and literature review. METHODS: A Medline search and comprehensive review of literature to assess all cases of granular cell tumors of the trachea were compared and collected, with particular attention to individual cases of pregnancy or cases related to the hyperestrogenic state. RESULTS: Thirty-two cases of granular cell tumors of the trachea were found. Only a few cases related to the hyperestrogenic state were obtained. The case report describes a woman with recurrent disease in subsequent pregnancies during the later stages of pregnancy. CONCLUSION: Few cases of granular cell tumors of the trachea have been reported, with even fewer being pregnancy related. The diagnosis and management of these tumors include careful attention to patient symptoms and airway management.

Granular cell tumors of the tracheobronchial tree.

OBJECTIVE: To describe the population-based incidence and clinical characteristics of granular cell tumors of the tracheobronchial tree. METHODS: All newly registered tracheobronchial granular cell tumors in the Dutch Network and National Database for Pathology for 10 consecutive years (1990-1999) were identified. The histologic diagnosis was confirmed and patient demographics, management, and follow-up data were analyzed. RESULTS: Thirty-one tumors were registered in 30 patients (12 male patients and 18 female patients; mean age 51 years; range 11-84) in a population of approximately 15 million. Tracheal tumors were identified in 11 patients and bronchial tumors in 19 patients (1 patient had 2 tumors). About half of the patients were asymptomatic. In the majority of the patients (61%) the granular cell tumor was an incidental finding during workup for lung carcinoma. Tracheal granular cell tumors were more frequent in women, whereas bronchial granular cell tumors showed no sex predilection. In the lung these tumors occurred more often in the upper (10 tumors) than in the lower lobes (3 tumors). There was no preference for either side. Four patients with tracheal and 4 with bronchial granular cell tumors were treated with surgery and remain in complete remission. Four bronchial granular cell tumor patients were treated locally with neodymium/yttrium-aluminum-garnet laser or electrocautery and are in complete remission or have stable residual disease. In 3 patients no residual disease was found after biopsy. Of all granular cell tumor patients 17 received no treatment for a variety of reasons, but none of these patients died in the follow-up period because of the granular cell tumors. CONCLUSION: Tracheobronchial granular cell tumor is a benign tumor with a good prognosis. In symptomatic patients surgical intervention is the first choice of treatment, but local treatment is a reasonable option and gives successful results.

Multiple cutaneous granular cell tumors: a case report and review of the literature.

Granular cell tumors are benign neoplasms derived from Schwann cells. Their clinical presentation ranges from large verrucose nodules, as in our patient, to small, nonspecific, subcutaneous papules. Approximately half of granular cell tumors develop in the head-and-neck region, most commonly on the tongue. Multiple granular cell tumors are not a sign of malignancy and may be more common than generally reported.

Occurrence of esophageal granular cell tumors in The Netherlands between 1988 and 1994.

Granular cell tumors (GCT) of the esophagus are rare. The tumor is generally beleived to be of neurogenic origin and shows a malignant course in 2-4% of cases. No unanimity has been reached regarding the management of this tumor. A national survey was conducted on the incidence of GCT of the esophagus, related symptoms, management, and follow-up. A national survey was performed on all newly registered esophageal GCTs in the PALGA system (Dutch register of all pathology diagnoses) for seven consecutive years (1988-1994). Fifty-two new cases (17 men, 35 women; median age 46 years, range 22-77 years) were registered. In 44 cases clinical data could be obtained (survey response 85%). The majority of the GCTs were solitary (42/44) and localized in the distal esophagus (33/44). At endoscopy the size of the tumor was estimated at <5 mm in 50%, 5-10 mm in 25%, and 10-30 mm in 18%. Most patients (40/44) presented with nonspecific gastrointestinal symptoms, only four had dysphagia (tumor size >1 cm). No malignancies were reported. Management of the tumor included excisional biopsy (1/44), endoscopic polypectomy (3/44), and surgical excision (1/44). Endoscopic follow up (1-60 months) in 16 out of 17 patients left untreated showed either a stable tumor size or regression of the tumor. In one case with multiple GCT's a slight tumor growth was seen after a follow-up period of 48 months. Esophageal GCTs in the Netherlands are rare, and mostly diagnosed incidentally. Most patients suffer from nonspecific symptoms; dysphagia occurs only with tumors >1 cm. The usual clinical course of esophageal GCTs is benign. Patients without dysphagia probably do not require routine endoscopic follow-up, provided they are instructed to contact their physician, once dysphagia develops.