Sclerosing angiomatoid nodular transformation (SANT) of spleen is a rare inflammatory tumor-like vascular lesion composed of angiomatoid nodules in a fibrosclerotic background. We report herein on a case of SANT in the spleen with its pathologic features, and review the related literature. A 50-year-old woman presented with mild left upper quadrant discomfort and tenderness and she showed a 6 cm-sized solitary splenic mass on computed tomography. She underwent laparoscopic splenectomy. Grossly, the spleen showed a well circumscribed round-shaped solid mass with multinodular hemorrhagic surfaces. Microscopically, the mass consisted of multiple angiomatoid nodules surrounded by collagen bundles with fibroblasts and a lymphoplasma cell infiltration. Immunohistochemically, the cells of the angiomatoid nodules were positive for CD31, CD30, CD34, alpha-smooth muscle actin, and VWF-VIII, but they were negative for CD8, anaplastic lymphoma kinase protein, and D2-40. The patient has been under close follow-up without recurrence.
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Sclerosing angiomatoid nodular transformation of the spleen presenting rapid growth after adrenalectomy: Report of a case Yasuo Nagai, Daisuke Satoh, Hiroyoshi Matsukawa, Shigehiro Shiozaki International Journal of Surgery Case Reports.2017; 30: 108. CrossRef
A Case of Sclerosing Angiomatoid Nodular Transformation of the Spleen: Spoke Wheel Pattern on Computed Tomography Joo Han Park, Sun Hyuk Hwang, Ki Chan Kim, Si Yeon Kim, Ga Won Song, Jae Ho Han, Joon Seong Park Korean Journal of Medicine.2014; 86(3): 357. CrossRef
Differentiation of an Unclear Splenic Lesion in a Patient With Cholangiocarcinoma Constantin Lapa, Ulrich Steger, Christian O. Ritter, Vanessa Wild, Ken Herrmann Clinical Nuclear Medicine.2014; 39(5): 470. CrossRef
Sclerosing Angiomatoid Nodular Transformation of the Spleen: CT and MRI Features With Pathologic Correlation Rachel B. Lewis, Grant E. Lattin, Meenakshi Nandedkar, Nadine S. Aguilera American Journal of Roentgenology.2013; 200(4): W353. CrossRef
BACKGROUND Primary splenic vascular lesions include the tumor and the inflammatory condition. A primary splenic vascular tumor is rare but is the most common tumor of the benign primary splenic tumors. METHODS We describe the clinicopathological, radiological, and immunophenotypical findings of 40 cases of primary vascular lesions identified at our hospital from 1996 to 2009. RESULTS The patients included 18 men and 22 women, aged 12 to 74 years, with a mean of 43.3-years and median of 40-years. They comprised 14 hemangiomas (35%), 13 lymphangiomas (32.5%), three hamartomas (7.5%), three littoral cell angiomas (7.5%), three sclerosing angiomatoid nodular transformations (SANT, 7.5%) and four angiosarcomas (10%). The majority of the patients (65%) were asymptomatic.
Some of the patients (32.5%) complained of abdominal pain, and 2.5% of the patients presented with fever. Metastases were identified in 75% of the patients with an angiosarcoma at the initial work-up. One angiosarcoma patient died of the disease despite adjuvant chemoradiotherapy. The radiological findings for hamartoma, littoral cell angioma, and SANT were nonspecific. Microscopically, six types of vascular lesions showed classic morphological and immunophenotypical features of their type. CONCLUSIONS One should be aware of rare splenic vascular lesions when radiological findings are nonspecific.
Histomorphological and immunophenotypical features are helpful for the differential diagnosis.
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A Case of Splenic Hamartoma Diagnosed by Contrast-enhanced Ultrasonography and Magnetic Resonance Imaging Hyeon Sik Kim, Tae Hyo Kim, Jae Min Lee, Hyun Jin Kim, Woon Tae Jung, Ok Jae Lee, Ji Eun Kim, Kyung Soo Bae The Korean Journal of Gastroenterology.2014; 64(6): 380. CrossRef
To investigate the effect of tumor removal and administration of OK432 on the splenic natural killer (NK) cell activity in the subcutaneous tumor bearing rats, NK cell activity assay using a 4-hour 51Cr release assay and flow cytometric analysis for NK cell population were performed. The results were as follows: 1. Splenic NK cell activity and population in the subcutaneous tumor bearing rats decreased along with the growth of the tumor. 2. The rats with subcutaneous tumor removal showed decrease of splenic NK cell activity, but splenic NK cell population was not decreased. 3. In the rats with subcutaneous tumor removal and OK432 administration, splenic NK cell activity was significantly increased 1 week after administration of OK432 and then gradually returned to normal, whereas increase of NK cell population was not significant. In the present study, splenic NK cell activity was significantly decreased despite removal of subcutaneous tumor. But with the administration of OK432, splenic NK cell activity returned to normal. Considering the role of NK cells on the first line of defense against the metastatic implantation of circulating tumor emboli, we suggest that perioperative administration of immunopotentiator such as OK432 may improve the patient's outcome after surgery of human neoplasm.
Gaucher's disease is an autosomal recessive disorder resulting fror i mutation at the glucocerebrosidase locus on chromosome 1q21. As a result, glucocerebroside accumulates principally in the phagocytic cells known as Gaucher ce Is.
In our case, a five-year old girl was admitted with seven days history of fever and abdominal distension. At physical examination the patient had hepatosplenomegaly. Laboratory tests revealed a hemoglobin concentration of 2.8g/L: platelet counts of 23,0001?l: normal range of white cell and differential counts, and negative Coombs' test. Liver enzymes were normal. For the evaluation of hepatosplenomegaly, fine needle aspiration was aerformed blindly against the palpable spleen. Wet-fixed hematoxylin and eosin-stained smears are made. The smears from the spleen showed predominantly macrophages with abundant cytoplasm and rather small, uniform, often eccentric nuclei with small nucleoli. The multinucleated cells were often found. The cytoplasm was pale, with more or less distinct fibrillarity. The cells had the characteristic appearance of Gaucher cells. Gaucher cells were also found it the tissue section from the liver, spleen and lymph node and the bone marrow aspirate. The diagnosis was later confirmed by determination of beta-glucosidase activity in peripheral blood leucocytes. Fine needle aspiration of the spleen is considered as a convenient procedure with a low complication rate for the diagnosis of lysosomal storage disease.
A 20-year-old young man who had undergone treatment for a suprasellar/perichiasmal tumor 2 years before, was presented with a huge palpable splenic mass. A fine needle aspiration cytology from the splenic mass showed dissociated large pleomorphic tumor cells having irregular nuclear outline, coarse chromatin, and one or two macronucleoli, and scattered small lymphocytes in fine granular background.
Above cytologic findings were regarded as the characteristics of germinoma. Differential diagnosis from the large cell lymphoma of spleen was emphasized.
There have been a few reported cases of pancreatic pseudocyst with involvement of adjacent structures including the stomach, duodenum, and even the mediastinum. An intrasplenic pancreatic pseudocyst is an uncommon complication of acute or chronic pancreatitis. We report a case of pancreatic pseudocyst with splenic involvement in a 69-year-old man with a review of literatures. Abdominal CT revealed a 4 cm sized cyst at the pancreatic tail which was extending into the splenic hilum and splenic parenchyma.
Distal pancreatectomy and splenectomy was performed.
Pathologic findings demonstrated a pseudocyst of pancreatic tail which communicated with the infacted splenic parenchyma. Also vasculitis, endothelial hyperplasia and medial calcification of the splenic artery were observed.
We report a case of an Epstein-Barr virus (EBV)-associated inflammatory pseudotumor-like follicular dendritic cell tumor (IPT-like FDC tumor). The tumor occurred in the spleen of a 64-year-old woman with a history of a diffuse large B-cell lymphoma (DLBCL) of neck nodes that presented four years ago. The splenectomy specimen revealed a 5 cm-sized, tan-colored and well-circumscribed mass. Histologically, spindle or ovoid cells with large vesicular nuclei were admixed with abundant inflammatory cells.
Immunohistochemically, spindle cells were positive for FDC marker CD35, but negative for CD20, CD30 and ALK. EBV was detected almost exclusively in spindle cells by EBER in situ hybridization. IPT-like FDC tumors are rare, and are recognized as a distinctive clinicopathologic variant of FDC tumors. Among only 18 similar cases reported in the English language literature, the present case is the first case of a patient with a history of DLBCL.
BACKGROUND Recent studies have proposed the use of peroxisome proliferator activated receptor-gamma (PPARgamma) ligands as new chemotherapeutic agents for human malignant tumors. However the in vivo mechanism of PPARgamma ligands on cellular toxicity is not clear. Therefore we examined the anti-tumor effects of the PPARgamma ligand, rosiglitazone (ROS), in animal models. METHODS To evaluate the effect of RSO on splenocytes, an in vitro and in vivo study was performed. Cytolytic activity was measured by use of a 51Cr release assay. The splenic natural killer (NK) cell population and effector-target conjugation were measured by flow cytometric analysis. RESULTS In 9L glioma bearing rats, 30 mg/kg/d of ROS treatment induced a significant decrease of subcutaneous tumor growth accompanied by an increased cytolytic activity of splenocytes and of the splenic NKR-P1bright/CD3- NK cell population. In normal rats, systemic administration of ROS also increased the cytolytic activity of splenocytes, the splenic NK cell population, and effector-target conjugation.
Moreover, we found that a concentration of 20micrometer ROS caused an increase in the cytolytic activity of splenocytes, and a concentration of 50micrometer ROS increased effector-target conjugation in vitro. CONCLUSIONS These results suggest that increased splenic cytolytic activity and NK cell population may contribute to the anti-tumor effects of PPARgamma ligands in vivo.
However, the roles of NK cells in the PPARgamma ligand-induced anti-tumor activity should be further investigated.
Cystic lesions in the accessory spleen are extremely rare and they present a challenging clinical differential diagnosis. We report here on two cases of epithelial cyst of intrapancreatic accessory spleen that mimicked pancreatic cystic tumor. In both cases, the patients underwent distal pancreatectomy under the impression of a benign cystic tumor of the pancreas. Unilocular or multilocular cysts in the pancreas tail were observed, and these were later shown to be epithelial cysts in the accessory spleen located within the pancreatic tail. The cysts were lined by columnar, cuboidal or stratified squamous epithelium.
True histiocytic sarcoma is an extremely rare tumor. Its clinicopathological features are not clearly understood.
Here, we report the first Korean case of primary splenic histiocytic sarcoma. A 64-year-old female having refractory thrombocytopenia, anemia and splenic mass was admitted to the hospital, and received splenectomy. Grossly, spleen was enlarged up to 18 x 13 x 8 cm and occupied with multinodular masses. Microscopically, the masses were composed of atyical large cells with abudant cytoplasm and vesicular nuclei with prominent hemophagocytosis. The tumor cells were CD68 (+), S-100 protein (-), CD21 (-), CD1a (-). After splenectomy, thrombocytopenia and anemia were corrected. However two months later the symptoms recurred, and the patient died 15 months after splenectomy. This case shared the common clinicopathologic features with the several previously reported cases in other countries, represented by splenic mass formation and prominent hemophagocytosis associated with thrombocytopenia and anemia, often leading to poor outcome.
BACKGROUND The purpose of this study was to elucidate whether or not splenectomy alters the acute toxic effects on rat tissue induced by barium sulfate. METHODS A total of 80 Sprague-Dawely male rats were divided into three groups; the control group and the barium sulfate treated groups with and without splenectomy. The pathologic changes in the solid organs were examined at the time after intraperitoneal treatment with barium sulfate. The changes of white blood cell count were also examined. RESULTS The barium induced toxicity was most prominent in the spleen. The changes in the other organs were proportional to the experimental duration, and the changes in the barium only treatment group were more severe than the observed changes in the splenectomy and barium treated group. CONCLUSIONS These results suggest that the degree of barium toxicity of barium only treated group was more severe than that of the splenectomy group.
Hairy cell leukemia is an uncommon lymphoreticular disorder which primarily involves bone marrow, spleen, and peripheral blood. Patients, mostly men, present with splenomegaly and pancytopenia usually. A 62-year-old man was admitted with an abdominnal mass which had grown slowly for 20 years. On physical examination, an enlarged spleen was palpated without tenderness. An abdominal CT scan showed a diffusely enlarged spleen, which measured 20 cm in greatest dimension.
In the peripheral blood, many atypical lymphocytes with abundant, delicate, surface projections were noted. They had tartrate-resistant acid phosphatase(TRAP) activity.
Thrombocytopenia (60,000/mm3) was observed in the complete blood counts, Other laboratory data were within normal limits. He underwent splenectomy. The submitted spleen measured 26x15x5 cm and weighed 2150 gm. It was well encapsulated and the outer surface was smooth. Cut surfaces were diffusely dark-red. White and red pulps were indistinct. There was no mass-like lesion. Microscopically, the spleen consisted of monotonous mononulcear cells which involved red pulp. The white pulp was diminished, and could be barely recognized. Cells had small round nuclei and abundant cytoplasm. Ultrastructurally, cells with numerous slender surface projections were noted. In Korea, hairy cell leukemia is exceedingly rare. We report a case of hairy cell leukemia with characteristic pathologic features of spleen as well as those of peripheral blood.
Lymphangioma of the spleen is a rare neoplasm and approximately 100 cases have been reported in the world literature. This lesion may involve the spleen alone or may be a part of a syndrome of multivisceral involvement. The pathogenesis is unclear whether this is a true neoplasm of a developmental defect. We report a case of a 87-year-old woman with solitary splenic lymphangioma. The cut surface mass is not encapsulated and located at the subcapsular area in the mid portion of spleen. It is composed of variable sized cystic spaces containing myxoid tissue. Microscopic finding reveals dilated lymphatic spaces lined by endothelial cells and the content is proteinaceous eosinophilic fluid.
Richer's syndrome is a development of a high grade malignant lymphoma in a patient with preexisting chronic 1ymphocytic leukemia, small lymphocytic lymphoma or Waldenstrom's macroglobulinemia. A rare case of Richer's syndrome arising in the spleen of a 35-year-old-man was studied by morphology, immunohistochemistry and gene rearrangement study. He has had weight loss and night sweat for last 6 months. Hepatosplenomegaly and abdominal lymphadenopathy were noted on CT scanning. Especially an ovoid radiolucent mass was found within the image of splenomegaly. Lymph nodes and liver biopsy, bone marrow aspiration and splenectomy were done. In the lymph nodes, liver and bone marrow, well differentiated small lymphocytic infiltrations were found but, in the spleen, pleomorphic, large cells with occasional multinucleated giant cells formed a nodular mass surrounded by diffuse, extensive infiltration of small well differentiated lymphocytes. The two distinctive areas in the spleen had positive staining for B-cell marker (HLA-DR and L26), negative staining for T-cell marker (UCLH1), and positive staining for IgM heavy chain and kappa light chain by immuohistochemical study. so this case was diagnosed as a diffuse large cell 1ymphoma transformed from small lymphocytic lymphoma. We made an another effort to clarify their clonality. Gene rearrangement method usingcomplementarity.determining region 3(CDR3) of immunoglobulin heavy chain (IgH) gene and T-cell receptor gamma (TCRgamma) gene by polymerase chain reaction (PCR) technique was done. The two lymphomas in the spleen demonstrated the same rearrangement pattern in both IgH and TCRgamma gene. We think these findings strongly suggest that the large cell lymphoma has the same clonality with that of the small lymphocytic lymphoma.
A 46-year-old woman presented with multiple purpuric skin rashes together with splenomegaly and thrombocytopenia. The resected spleen weighed 720 gm, and contained multiple, discrete, dark red, bulging nodules throughout the entire splenic parenchyma. Microscopically, the nodules revealed various range of endothelial cell proliferation which included cavernous vasculature, anastomosing vascular channels, papillary intravascular growth and solid pattern.
Intrasinusoidal sequestration of platelets along with extramedullary hematopoiesis was present in the neoplastic vascular spacws. Neither exposure to thorotrast nor vinyl chloride was nonfirmed. Ultrastructurally, the tumor cells demonstrated a row of pinocytotic vesicles, occasional intercellular junctional structures, and the paucity/absence of filamentous structures or Weibel-Palade bodies supports origin of sinus lining type cells. After splenectomy, the thrombocyte count returned to normal. We conclude this is the first case of primary splenic angiosarcoma with Kasabach-Merritt syndrome in Korean literature.