Primary extragastrointestinal stromal tumor arising in the vaginal wall: Significant clinicopathological characteristics of a rare aggressive soft tissue neoplasm.

Gastrointestinal (GI) stromal tumor is the most common mesenchymal neoplasm of the GI tract but also occurs with a lower frequency in extragastrointestinal regions and is called extragastrointestinal stromal tumor (EGIST). We report an unusual case of EGIST presenting as a vaginal mass. A 41-year-old woman presented with a gradually enlarging vaginal mass for the last 2 years. Physical examination revealed an elliptical, non-tender mass about 7.5 cm × 7 cm in size in the posterior vaginal wall and was resected completely. Under histological examination, the tumor showed a spindle cell type with coagulation necrosis, hemorrhage and high mitotic count. Immunohistochemical analysis revealed tumor cells were positive for DOG1, CD117, CD34 and p53 protein. Ki-67 labeling was 8%. Genetic analysis showed a deletion of exon 11 of the c-kit gene at codons 557-558. EGISTs should be kept in mind in the differential diagnosis in patients presenting with solid mass of the vaginal wall.

IgG4-related prostatitis progressed from localized IgG4-related lymphadenopathy.

Immunoglobulin G4-related disease (IgG4-RD) is a recently described inflammatory disease involving multiple organs. Prostate involvement with IgG4-RD is very rare. In this report, we describe a case of IgG4-related prostatitis progressed from localized IgG4-related lymphadenopathy. This patient was present with urine retention symptoms. MRI and CT examination revealed the prostatic enlargement and the multiple lymphadenopathy. Serum IgG4 levels were elevated. Prostatic tissue samples resected both this time and less than 1 year earlier showed the same histological type of prostatitis with histopathologic and immunohistochemical findings characteristic of IgG4-RD. The right submandibular lymph nodes excised 2 years earlier were eventually proven to be follicular hyperplasia-type IgG4-related lymphadenopathy. This is the first case of IgG4-RD that began as localized IgG4-related lymphadenopathy and progressed into a systemic disease involving prostate and multiple lymph nodes. This patient showed a good response to steroid therapy. This leads us to advocate a novel pathogenesis of prostatitis, and a novel therapeutic approach against prostatitis. Pathologists and urologists should consider this disease entity in the patients with elevated serum IgG4 levels and the symptoms of prostatic hyperplasia to avoid ineffective medical or unnecessary surgical treatment.

Clinicopathological features of Kaposiform hemangioendothelioma.

Kaposiform hemangioendothelioma (KHE), an intermediate tumor of endothelial origin in childhood, is often associated with Kasabach-Merritt phenomenon (KMP). In this study, 22 cases of KHE were immunochemically studied for CD31, CD34, ERG, smooth muscle actin (SMA), D240, GLUT1 and Ki67. The patients (15 males and 7 females) ranged in age from 13 days to 7 years (median, 2 mo). Lesion developed on the extremities/joint (12 cases), chest/abdominal wall (6 cases), head/neck (4 cases), and presented both superficial and deep soft tissue. The superficial change was commonly enlarging cutaneous lesion with ill-defined red to purple indurated plaque. 15 of the 22 cases (68%) developed KMP, with consumptive thrombocytopenia or bleeding complications. Tumors consisted of infiltrating nodules of fascicles of spindleshaped endothelial cells and slitlike vascular channels with irregular tumor margins. On immunohistochemistry (IHC), endothelial cells were diffusely positive for CD34, CD31 and ERG but negative for GLUT1, and the peripheral area of proliferative capillaries were markedly positive for D240. Adjuvant medical therapy and sclerotherapy were prepared for the tumor and the associated KMP, and then all patients were treated by complete surgical excision. Follow-up information was available in 22 patients (8 to 26 months, mean 15 mo), and indicated that 1 died of multiple organ failure and 21 were alive without residual disease. In conclusion, our results suggest that KHE can occur in the embryonic period, and patients with KMP often have earlier onset time and larger lesional size. KHE patients given with adjuvant corticosteroids and urea injection and complete resection rarely relapse.

Study on Clinicopathological Features of Gastrointestinal Stromal Tumor and Relevant Prognostic Factors.

The objective of the study is to investigate and analyze clinicopathological features of gastrointestinal stromal tumor (GIST) and relevant prognostic factors. Between September 2008 and April 2014, 168 patients who presented with pathologically confirmed GIST and underwent surgical resection of the tumors in our institution were included in this study. A retrospective study on clinicopathological features of the disease and follow-up study on 1-year, 3-year, and 5-year survival rates were performed. Of 168 patients, 113 were males and 55 females of age ranging from 18 to 78 years (mean age 52.6 ± 10.1 years). Seventy patients (41.7 %) presented abdominal distension, 74 (44.0 %) abdominal pain, and 82 (48.8 %) bloody stools. Of all patients, 115 (68.4 %) developed tumors in GI tract, 51 (30.4 %) presented multiple tumors, 88 (52.4 %) tumors displayed a maximal diameter >5 cm, mitotic count >5/50HPF were observed in 80 patients. Positive rate of CD117 was 92.9 % (156/12), DOG1 97.0 % (163/5), CD34 53.0 % (89/79) and S-100 25.6 % (43/125). Follow-up study achieved in 149 patients. Survival analysis and Cox regression analysis demonstrated that no significant prognostic effects were observed for gender, clinical presentations, tumor location, number of tumors, CD34 and S-100 expression (p > 0.05). However, tumor diameter and mitotic count were factors with significant effect on prognosis of GIST (p < 0.05). Tumors with diameter >5 cm and mitotic count ≥5/50HPF resulted in poor prognosis. Common symptoms of GIST include abdominal pain and blood stools. Tumor diameter and mitotic count are helpful for the evaluation of prognosis with favorable clinical value.

Epithelioid inflammatory myofibroblastic sarcoma treated with ALK inhibitor: a case report and review of literature.

Epithelioid inflammatory myofibroblastic sarcoma is extremely rare and belongs to a variant of inflammatory myofibrobalstic tumor with aggressive clinical course. We describe a case of a 22 years old man presented with an abdominal huge tumor. Microscopically, the neoplasm cells were rounded and epithelioid in shape. Abundant interstitial edema and less myxoid stroma were also present together with an inflammatory infiltrate. Fluorescence in situ hybridization revealed that ALK gene presented mutation. After surgery the patient received chemotherapy with an anaplastic lymphoma kinase (ALK) inhibitor, crizotinib. The patient continues to be alive with disease for 16 months and has no recurrence. Although EIMS has a poor prognosis, this is the few successful case with sustained response of targeted therapy.