Cases reported "Splenic Neoplasms"

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1/38. Unusual vascular changes in the red pulp of the spleen accompanying breast carcinoma metastasis.

    The prevalence of splenic metastasis from carcinomas varies between 2% and 13% in autopsy studies. Most of them are clinically inapparent. We report herein the case of a splenic metastasis revealing breast carcinoma in a 73-year old woman. splenectomy was performed to correct hypersplenism. Macroscopically, the cut surface of the spleen was uniform and pale. On microscopical examination, the metastatic infiltration involved both red and white pulp as single cells, cords and micro-nodules. Tumor cells were positive for cytokeratin and epithelial membrane antigen (EMA). The breast origin of this splenic metastasis was supported by the increase of CA 15-3 level, and by the appearance of axillary lymphadenopathy. In addition, the red pulp sinuses were obliterated by multiple thrombi at different stages of development and the splenic cords were collagenized. These changes could result from an unusual stromal reaction.
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2/38. Splenic lymphoma presenting as warm autoimmune hemolytic anemia associated with pure red cell aplasia.

    BACKGROUND AND OBJECTIVE: Warm autoimmune hemolytic anemia (AIHA) is a condition in which peripheral red blood cell (RBC) destruction is induced by the presence of an autoantibody. Pure red cell aplasia (PRCA) represents an isolated process of decreased erythropoiesis. The combination of both is quite rare, with a very poor prognosis. We describe a patient with isolated splenic lymphoma whose presentation was a combination of AIHA and PRCA. The patient was resistant to all treatment. MATERIALS AND methods: Erythroid colony assays were performed, in order to compare the effect of the patient's serum on colonies with that of a normal control. RESULTS: The patient's serum significantly suppressed normal erythroid colony growth. A red cell eluate revealed the presence of a warm autoantibody. CONCLUSIONS: The patient's serum contained warm autoantibody responsible for peripheral RBC destruction and a humoral factor, perhaps the warm autoantibody, which suppressed bone marrow erythropoiesis. Establishing an early diagnosis, and treatment of the underlying disease might result in a better prognosis.
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3/38. A case of epidermoid cyst of the intrapancreatic accessory spleen.

    A 54-year-old woman presented with a huge palpable mass on left upper quadrant of the abdomen. After preoperative work-up, a cystic disease of pancreatic tail or accessory spleen was initially suspected. We performed exploratory laparotomy and resected both the spleen and a 15 x 11 cm-sized huge cystic mass containing a part of solid component which extended continuously to the pancreatic tail. The solid component, comprising the upper portion of the resected cyst, was reddish brown and granular like as normal splenic tissue. The inner surface of the cyst was smooth and was filled with yellowish white material. Histologic examination showed an epidermoid cyst originating in the accessory spleen of the pancreatic tail lacking hair or skin appendages.
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4/38. A case of splenic lymphoma with marked diffuse nodular fibrosis and calcification, complicated with severe autoimmune hemolytic anemia.

    A splenic lymphoma, possibly of a splenic marginal zone lymphoma, marked by small nodular splenic calcified fibrosis and complicated by intractable autoimmune hemolytic anemia, was studied by immunohistochemical, molecular genetic, and ultrastructural analyses. The patient was a 57-year-old Japanese man who had moderate splenomegaly, and who had undergone splenectomy for improvement of severe autoimmune hemolytic anemia and to rule out malignancy in the spleen. In the resected spleen, proliferative atypical lymphoid cells were observed both in the red and white pulp with diminished germinal centers and irregularly widened marginal zones with peculiar dimorphic pattern. Ultrastructural study revealed no hairy cells or villous lymphocytes. Diffuse nodular hyalinous fibrosis surrounding the small arterioles in the white pulp overlapped with frequent calcification was a unique histologic feature in this case. Degenerative connective tissue, extracellular matrix, or collagen fibers surrounding the arterial sheath in the white pulp caused by some immunological abnormalities associated with this splenic lymphoma could be assumed to be the predisposing factor for this excessive fibrosis and dystrophic calcification in the spleen.
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5/38. Malignant lymphoma of the stomach in association with inflammatory myofibroblastic tumor of the spleen. A case report.

    We report on a case of a 40-year-old male patient who underwent a gastrectomy because of a biopsy-proven large B-cell lymphoma of the stomach. On surgery, a nodule in the spleen also was noted. Grossly and microscopically, the two lesions were different: the tumor of the stomach appeared white-gray on the cut surface and was a centroblastic variant of diffuse large B-cell lymphoma. Histologically, one perigastric lymph node was involved. Grossly, the splenic nodule was gray-yellow and had a histological appearance of an inflammatory myofibroblastic tumor (IMT). The association between malignant tumor and IMT is rare. In such an association, the latter lesion most often has been reported in the spleen. As EBV may be involved in the genesis of both lymphoma and IMT, we tested both lesions for its presence using in situ hybridization, but the tests were negative. It remains to be verified whether the association between lymphoma and IMT is more than fortuitous.
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6/38. Splenic lymphoma with villous lymphocytes in two sisters.

    Splenic lymphoma with villous lymphocytes is a new entity characterised by the presence of atypical lymphocytes in the peripheral blood and bone marrow, and splenic infiltration in the white and red pulp. cell membrane markers are those of a B mature cell, and no particular chromosomal abnormalities have been associated with this disease. A case of this rare lymphoma occurred in two sisters. Histological examination of splenic tissue was identical in both cases, with the same immunological surface markers, although the clinical and laboratory features were different. karyotype analysis showed an abnormal pattern in one case; no environmental causative factor could be detected. Familial cases of other lymphoproliferative disorders have been reported, but no consistent common link has been found. It is suggested that further reports of this lymphoma, including cytogenetic and molecular studies, may provide a better understanding of the aetiology.
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7/38. Gamma/delta T cell lymphoma.

    A 54-year-old woman complained of fever and hepato-splenomegaly. The pathological findings of a liver biopsy specimen revealed the infiltration of lymphocytes in the sinusoids and that of the laparoscopically resected spleen revealed the infiltration of lymphocytes in the red pulp, which was positive for CD3, CD43, CD45RO and T-cell intracellular antigen-1 (TIA-1) and was negative for betaF1, while the white pulp was spared. Genetic analysis of the spleen cells revealed the rearrangement of T-cell receptor (TCR) Cbeta1, Jdelta1 and Jgamma. Epstein-Barr virus (EBV) genomic dna was detected in the spleen cells. Atypical lymphocytes appeared in the peripheral blood and bone marrow, chromosomal analysis revealed del (13) (q12 q14), trisomy 8 and breakage of RB gene. Elevated level of serum vascular endothelial growth factor (VEGF) was observed. Hepatosplenic gammadelta T cell lymphoma (GDTL) was diagnosed. The patient was treated with chemotherapy by cyclophosphamide, hydroxydoxorubicin, vincristine and prednisolone (CHOP), however, it was ineffective, and the patient died of hemorrhage from the lymphoma involvement of the intestine 5 months after the onset of disease.
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8/38. breast carcinoma diffusely metastatic to the spleen. A report of two cases presenting as idiopathic thrombocytopenic purpura.

    carcinoma metastatic to the spleen is found at autopsy in 6% to 13% of patients who die of cancer, yet clinical symptoms referable to splenic metastases are unusual. Two cases of breast carcinoma metastatic to the spleen discovered incidentally at therapeutic splenectomy for idiopathic thrombocytopenic purpura are described. On gross examination, the spleens were mildly enlarged with a homogeneous congested cut surface; rare 0.2-cm white nodules were present in one case. Microscopic examination revealed large, poorly cohesive cells that diffusely involved both the red and white pulp. Histochemical, immunohistochemical, and ultrastructural analyses confirmed the epithelial nature of the cellular infiltrate. These cases show that idiopathic thrombocytopenic purpura may herald the presence of diffuse splenic metastases when metastatic disease is not otherwise clinically suspected. The lack of a discrete tumor mass in the spleen in such cases may make the diagnosis of metastatic carcinoma a challenge both clinically and pathologically. Immunohistochemical and electron microscopic examinations are useful to establish the appropriate diagnosis in such cases.
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9/38. Blastic transformation after splenectomy in a patient with nonvillous splenic marginal zone lymphoma with p53 overexpression: a case report.

    A 61-year-old man with no subjective symptom was admitted to our hospital for further examination of the causes of anemia (hemoglobin, 9.5 g/dL) and thrombocytopenia (platelets, 9.2 x 10(4)/microL), which had been pointed out in a medical checkup half a year previously. A bone marrow examination showed 73% lymphoid cells. immunophenotyping of these cells were CD19 CD20 CD3-CD5-CD10-CD23-, and light chain restriction (kappa) was positive by fluorescence-activated cell sorting analysis. A computed tomography scan showed mild splenomegaly. To confirm the diagnosis histologically, we performed a splenectomy. Finally, we diagnosed the patient's disease as nonvillous splenic marginal zone lymphoma (SMZL). A month after the splenectomy, the white blood cell count was remarkably increased to 7 x 10(4)/microL with the blastic transformation of lymphoid cells. We first treated the patient with fludarabine and then with the CHOP regimen (cyclophosphamide, hydroxydaunomycin, vincristine [Oncovin], and prednisone), but the disease was so refractory that the patient died of the disease 13 months after the splenectomy. Immunohistochemical staining and a molecular examination for p53 were carried out with specimens from the splenectomy. We found overexpression of the p53 protein in lymphoid cells and a point missense mutation in codon 280 at exon 8 that changed AGA (Arg) to AGT (Ser). This case may indicate the existence of a more aggressive subset of SMZL, suggesting a reconsideration of the roles of splenectomy and p53 overexpression in the diagnostic and therapeutic approaches to patients with SMZL.
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ranking = 12.316702532808
keywords = blood cell, white
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10/38. A rare case of diffuse neonatal hemangimatosis.

    Diffuse neonatal hemangiomatosis (DNH) is a rare neonatal condition in which cutaneous and visceral hemangiomas coexist. If left untreated, DNH is usually fatal at an early age. We report a case of a 6-month-old male infant who was brought to our institution with hepatosplenomegaly and a history of anemia and thrombocytopenia since 1 month of age. cytogenetic analysis and liver biopsy were normal and bone marrow aspirate was nondiagnostic. Congenital red blood cell abnormality was ruled out. Ultrasound confirmed an increase in size of the spleen from 5 to 15 cm, and magnetic resonance imaging demonstrated intense splenic enhancement consistent with a hemangioma or vascular malformation. Despite severe thrombocytopenia, an exploratory laparotomy was done and the patient underwent a splenectomy and omentectomy. The final pathology confirmed hemangiomatosis of the spleen and omentum. In the neonate with unexplained anemia and thrombocytopenia, DNH should be considered as part of the differential diagnosis. In our case, the patient not only exhibited no obvious cutaneous involvement, but also had rare splenic involvement. Although there are risks involved when operating on a thrombocytopenic patient, the benefits of operating on a patient with DNH far outweigh the risks, and operative intervention should proceed without delay.
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