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Highlights • Familial multiple lipomatosis (FML) is a rare hereditary benign disease. • The origin of extra-gastrointestinal stromal tumors (EGISTs) remains controversial. • EGISTs and cells of Cajal have similar features—the expression of CD117 and CD34. • Surgical resection is the standard treatment of EGISTs. • Targeted medical therapy of EGISTs by tyrosine kinase inhibitors can be useful., Introduction Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Rarely, GISTs can be located in mesentery, retroperitoneal space, omentum or pancreas. In these cases, the neoplasm is defined as “extra-gastrointestinal stromal tumors” (EGISTs). Presentation of case We reported a case of a 63-year-old male patient diagnosed by computer tomography with large intraabdominal tumor with vague origin, postoperatively determined as an EGIST. The diagnosis was confirmed by immunohistochemical study. The patient had multiple, subcutaneous, painless lipomas localized in the arms, forearms, thighs, abdomen and thorax. Because of the family history and the clinical presentation the disease was determined as familial multiple lipomatosis (FML). We performed radical tumor resection with distal pancreatectomy and splenectomy, and abdominoplasty, removing redundant skin and underlying subcutaneous fat tissue with multiple lipomas. Discussion FML is a rare hereditary benign disease. On the other hand, only few cases with familial GIST have been reported. In cases with extensive abdominal involvement, the primary origin of EGIST may be impossible to determine so the differential diagnosis is very difficult. Conclusion Although we could not prove correlation between the observed diseases, they are extremely rare and their combination is unusual which makes the presented case valuable and interesting.

Highlights • Rosai–Dorfman diseasе (RDD) is a rare histiocytic disorder оf unclear etiology. • RDD is characterized by emperipolesis and presence of S-100 positive histiocytes. • RDD usually presents as a massive, bilateral enlargement of the neck lymph nodes. • Localization of RDD in the liver and gallbladder is extremely rare. • Treatment is recommended in cases with symptomatic localized or extended RDD., Introduction Rosai–Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic disorder of unclear etiology. Lymphadenopathy commonly affects the lymph nodes of the neck and the head, but extranodal involvement has been observed too. Presentation of case We describe an unusual case of RDD with involvement of the gallbladder, liver and cystic lymph node, clinically manifested as an exacerbated cholecystitis. The patient underwent a cholecystectomy with atypical liver resection because the finding was macroscopically suspected of gallbladder cancer. The diagnosis of RDD was confirmed by immunohistochemical study. Discussion Histological features of RDD include histiocytic proliferation, emperipolesis and positive immunostaining for S-100 protein and typically negative for CD1a. Gastrointestinal localization of RDD, especially in the liver and pancreas, is extremely rare. Typically, the liver is affected as a part of systematic spread of RDD. We have not found reports for involvement of the gallbladder. Conclusion The presented case is valuable due to its rarity and difficulty in differential diagnosis.

Background IgG4-related disease (IgG4-RD) is a newly recognized disorder, characterized by massive IgG4+ lymphocyte and plasma cell infiltration, storiform fibrosis, causing enlargement, nodules or thickening of the various organs, simultaneously or metachronously. Involvement of the gastrointestinal tract is very rare and can be presented as a diffuse wall thickening or polyp or mass-like lesion. Up to now, there have been reported only a few cases of isolated gastric IgG4-RD. Case presentation We present an unusual case of IgG4-RD of the stomach with involvement of the regional lymph nodes, clinically manifested as a gastric cancer with related pyloric stenosis. The patient underwent distal gastrectomy, omentectomy and lymph node dissection. The postoperative serum IgG4 level was increased. The diagnosis was confirmed by immunohistochemical study. Conclusions In the most of the reported cases there was not sufficient data about the regional lymph nodal status, although the majority of the patients had been operated with presumptive diagnosis of gastric neoplasm. Our case is rare and valuable because it presents a gastric IgG4-related lesion larger than all previously reported in literature, and IgG4-related lymphadenopathy, confirmed histologically, which contributes to better knowledge of the disease.

Introduction: Metastatic lesions account for approximately 8% of all ovarian neoplasms. About 12% of clinically apparent metastases from non-gynecological primary site originate from breast carcinomas with a variable period of manifestation and detection. Report of Case: We present a case of 58-year-old woman who underwent bilateral adnexectomy with small bowel resection during urgent operation for an incarcerated umbilical hernia. There was a slight enlargement in both ovaries and presence of cystic formation with some papillary structures in the right one. Histologically, bilateral infiltration of tumor cells with moderate cytological and nuclear atypia and tubular, nested and cystic growth pattern was observed. The patient had a long list of co-morbidities: liver cirrhosis, ascites, isolated cytology with presence of tumor cells, elevated level of CA125 and quadrantectomy of the right breast, performed 15 years ago. Diagnostic examination continued in differing primary ovarian carcinoma from metastatic process using immunohistochemical markers – Gross cystic disease fluid protein-15 (GCDFP-15), Mammaglobin, Wilms tumor protein (WT1), p53, Ki67 and hormone receptors for estrogen (ER), progesterone (PR) and human epidermal growth factor (HER2). Considering the clinical data and the results from the pathological examination we concluded that the patient had bilateral ovarian metastases from breast carcinoma. We share our stepwise approach to the diagnosis discussing some of the difficulties emerged in the process and some of the pitfalls reaching the final conclusion.