Malignant granular cell tumor: Clinical features and long-term survival.

BACKGROUND: Malignant granular cell tumor GCT (mGCT) has not been well described. We sought to investigate associations between tumor characteristics, treatments and survival. METHODS: Patients diagnosed with mGCT years 1995-2014 were identified using the Surveillance, Epidemiology and End Results database. Descriptive statistics regarding tumor and treatment characteristics were calculated. Chi-square tests determined associations between tumor location and features. Survival analyses included Kaplan-Meier functions and Cox proportional hazard ratios (HR). RESULTS: Of 113 patients included, median age was 54 years and 77.0% were female. Frequent tumor sites included soft tissues (36.3%), ovary/testis (16.8%), and skin (11.5%). Median tumor size was 4.0 cm. Metastases to regional lymph nodes (12.5%) and distant sites (11.4%) occurred. Treatments included surgery (85.0%), radiotherapy (12.4%) and chemotherapy (8.9%). Overall five and 10-year cause-specific survival was 74.3% and 65.2%, respectively. Survival was worse for patients with tumors >5 cm compared to those with tumors ≤5 cm (HR = 34.03; 95% confidence interval [CI]: 2.57-450.17), and patients with metastasis (HR = 15.25; 95% CI: 1.19-195.72) compared with those without metastasis. Patients who underwent surgery had superior survival than those who did not (HR = 0.13; 95% CI: 0.05-0.34). CONCLUSIONS: Particular tumor features and treatments are associated with superior survival. This information may be used to more accurately estimate prognosis.

Granular cell tumor experience at a comprehensive cancer center.

BACKGROUND: Granular cell tumors (GCTs) are rare lesions occurring almost anywhere in the body. Multiple case reports have been published. However, there are very few large-scale studies regarding GCT. The aim of this study was to define characteristics, treatment patterns and outcomes of patients with GCT. METHODS: An institutional review board-approved retrospective chart review was performed. Descriptive statistics, chi-square analyses, and Kaplan-Meier survival estimates were produced. RESULTS: Fifty patients were treated for GCT at our institution between 1992 and 2015. The median age was 47 y; 62% of patients were female and 64% were whites. Median tumor size was 0.8 cm. Four percent of patients had malignant tumors, 10.0% had atypical tumors, and 86.0% had benign tumors. The most frequent location of tumors was the gastrointestinal tract (n = 30; 60%), followed by skin/subcutaneous tissues (n = 19; 38%), then respiratory tract (n = 1; 2%). Most patients underwent surgical excision or endoscopic removal of their tumors without prior biopsy. Three patients (6%) had multifocal tumors; they were more likely to experience recurrence than patients with unifocal tumors (33.3% versus 10.6%, respectively; P = 0.05). Six patients (12.0%) experienced recurrence, with a median time to recurrence of 13.5 mo. Overall cancer-specific 5-y survival was 98.0%. Overall recurrence-free 5-y survival was 86.4%. Patients with atypical tumors had a lower recurrence-free 5-y survival rate than those with benign tumors (75.0% versus 89.7%, respectively; P = 0.04). CONCLUSIONS: Patients with GCT fair well, particularly when tumors are benign. Patients with multifocal tumors are more likely to experience recurrence and should be closely monitored.

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.

Atypical and malignant granular cell tumors in Japan: a Japanese Musculoskeletal Oncology Group (JMOG) study.

BACKGROUND: Malignant granular cell tumors (MGCTs) are extremely rare neoplasms with only a limited number of studies published to date. The aim of this study is to elucidate the clinicopathological characteristics and prognostic factors of MGCTs. METHODS: This is a multi-institutional retrospective study of MGCTs with a central pathological review. A total of 18 cases were retrieved. Specimens were blindly reviewed by two pathologists based on the diagnostic criteria by Fanburg-Smith et al. Kaplan-Meier survival probabilities were calculated, and risk factors for poor prognosis were evaluated. RESULTS: Three and fifteen cases were diagnosed as atypical GCTs (AGCTs) and mGCTs according to the Fanburg-Smith et al. classification, respectively. Four (one atypical and three malignant) cases had metastasis at the first presentation, including lymph node metastasis. Three out of ten cases treated with wide resection developed local recurrence. Although prolonged static disease periods of ≥1 year were observed in four cases receiving chemotherapy, all cases with local recurrence or metastasis, including two atypical cases, eventually died of disease. The 5- and 10-year overall survival rates for localized MGCTs were 69.2 and 34.6 %, respectively. The presence of necrosis was revealed as a risk factor associated with adverse clinical outcomes. CONCLUSIONS: MGCTs have high rates of recurrence and metastasis including lymph node metastasis. As histologically atypical cases also have metastatic potential, close attention should be paid to AGCTs. The combination of histological evaluation and tumor size may lead to more accurate diagnosis of this rare neoplasm.

Novel diagnosis and treatment of esophageal granular cell tumor: report of 14 cases and review of the literature.

BACKGROUND: Granular cell tumors (GCT), especially in the esophagus, are rare neoplasms originating from the nervous system. There is still some controversy regarding the diagnosis and treatment of esophageal GCT. METHODS: We report 14 cases of esophageal GCT diagnosed and treated from January 2004 to March 2013. Their clinical manifestations, endoscopic image, endoscopic ultrasonography (EUS) appearance, pathology, immunohistochemistry, treatment, and prognosis were reviewed. RESULTS: The typical images of EUS were hypoechoic, homogenous, and smooth-edged tumors restricted to deep mucosal and submucosal layers. However, there were 2 cases with tumors invading muscular layer. Endoscopic ultrasonography was valuable to assess the tumor size, location, depth of invasion, and nature. According to EUS manifestation, 11 cases with lesions 3 cm or less in diameter without muscular layer invasion underwent endoscopic resection without complication and the other 3 cases underwent surgical resection. A new technique of submucosal tunnel endoscopic resection was performed in 3 submucosal cases with lesions ranging from 2 cm to 3 cm in diameter. All of these cases were benign and histology was necessary for differential diagnosis. CONCLUSIONS: Endoscopic ultrasonography plays an important guiding role in the diagnosis and treatment of esophageal GCT. Submucosal tunnel endoscopic resection is safe and effective. Further study is needed to determine whether this technique can be expanded into other applications.

[Prognostic factors in granulosa cell tumor of the ovary].

OBJECTIVE: To study the effect of clinical and pathologic factors on prognosis for granulosa cell tumor of the ovary. METHODS: The clinical records and tumor sections of 100 patients with granulosa cell tumors of the ovary between 1958 and 1995 were reviewed. The relationship between age, stage, mitosis and the adjuvant therapy for early stage (stage I) were analyzed retrospectively. Patients with recurrent tumors were compared with patients who remained without disease, patients with early recurring tumors were compared with late recurring tumors. RESULTS: The overall 5 and 10 year survival rates were 80% and 72%. There were no significant difference between ages and survival (P > 0.05). The survival rates in stage I were 98% and 96% after 5 and 10 years, respectively, and in stages II were 70% and 60%, after 5 and 10 years, all of 4 patients with stage III-IV were dead of recurrent disease in 1 year. The frequency of observed mitosis influenced the survival rate: with less 5/10 high-power fields (HPF) the survival were 96% both at 5 and 10 years, with more or equal 5/10 HPF the 5 and 10 year survival rates were 58% and 36%, respectively (P < 0.01). No significant correlation could be established between the adjuvant therapy and 5 and 10 years survival in 56 stage I patients. There were 44 patients with recurrent disease in this group, median time to recurrence was 53 months. Late recurrence appeared in 8 cases. The significant differences in stage and abdominal mass were noted between the recurrent tumors and the group without disease (P < 0.05). When early and late recurring tumors were compared, statistically significant differences were again noted: early recurring tumors had higher mitotic rates and late stage, and late recurring tumors had lower mitotic rages and early stage. Patients without recurrent tumors were similar to the patients with early recurring tumors. CONCLUSIONS: Tumor stage and mitotic rate are the clinical and pathologic prognosticators in granulosa cell tumor. It is difficult to predict late recurrences using these clinical and pathologic parameters.