Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hamartomas of the spleen are rare benign tumours, which are usually asymptomatic, incidental findings at laparotomy or autopsy (Komakl and Gombas, 1976; Brinkley and Lee, 1981; Norowitz and Morehouse, 1989). There are a few well documented reports of symptomatic splenic hamartoma associated with haematological disturbances, marked splenomegaly or even spontaneous rupture that required an emergency operation (Iozzo et al., 1980; Morgenstern et al., 1984). We report a patient with splenic hamartoma who presented with splenomegaly and iron deficiency anaemia. Computed tomographic (CT) and ultrasound (US) evaluation demonstrated an inhomogeneous mass within the enlarged spleen. Malignant melanoma was mistakenly diagnosed by US-guided fine needle aspiration of the mass, and necessitated splenectomy. The final diagnosis was hamartoma of the spleen.
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PMID:Case report: unusual presentation of splenic hamartoma; computed tomography and ultrasonic findings. 160 2

A study of 12 cases of splenic hamartomas, rare benign lesions of the spleen, included one case of spontaneous rupture of a hamartoma that required emergency operation and two cases of hamartomatosis with splenomegaly. An increased incidence of accessory spleens was also noted. Therefore, although rarely symptomatic, hamartomas may occasionally be of clinical importance. Surgeons should be aware of these lesions in the differential diagnosis of splenic tumors that appear as filling defects on scintiscan or as splenic masses on abdominal exploration. Since it may also be a source of spontaneous splenic rupture, it should be considered as one of the rare causes of such an occurrence.
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PMID:Hamartomas of the spleen. 649 34

Primitive bening spleen tumors are very rare in pediatrics. They can be solid or cystic. Authors report two cases of spleen tumors: one hamartoma of red pulp in a 4 1/2 year old male, with symptoms of an enlarged spleen and immunological deficit; and the other case a simple cyst, that developed as an enlarged spleen, lined by mesotelium. Based on this experience, they comment on frequency, clinical and radiologic diagnosis, pathology and treatment of these tumors.
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PMID:[Benign tumors of the spleen in pediatrics]. 670 34

In a 41-year-old woman who had suffered from hypofibrinogenemia, thrombocytopenia, leukopenia and splenomegaly for several years a causative hamartoma of the spleen (splenoma) was removed surgically. The results of a preoperative therapeutic trial with heparin, the marked accumulation of 125I-activity in the splenoma following intravenous injection of radioiodinated fibrinogen, the immunohistochemical demonstration of extensive fibrinogen deposits in the splenoma, and normalization of fibrinogen levels and blood cell counts after surgery point to the pathogenetic role of this rare splenic tumor. The salient clinical and pathological features of 41 splenoma cases in the literature are briefly reviewed. Hematologic signs were observed in only 9 splenoma patients.
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PMID:[Chronic defibrination syndrome and thrombocytopenia in splenic hamartoma (splenoma)]. 711 62

Symptomatic splenic hamartomas are rare in the pediatric age group, with only four previous reports in the literature. Splenic hamartoma has been reported as a solid homogeneous mass without calcification on CT and ultrasound (US), and only one previous report of the findings on MRI has been published. We report a case of a large symptomatic splenic hamartoma in a 14-year-old girl who presented with splenomegaly, pancytopenia and growth retardation. A solid mass with multiple punctate foci resembling calcifications was seen on US. The mass was heterogeneous and better demarcated on enhanced CT. Radiocolloid scintigraphy demonstrated uptake within the lesion, but less than that of normal spleen. The mass was isointense relative to normal splenic tissue on T1-weighted MRI (0.5 T) and of increased intensity with T2 weighting. At splenectomy, a red pulp hamartoma was identified, which contained nodules of hyalinization and necrosis thought to account for the punctate foci seen on US.
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PMID:Radiological features of a symptomatic splenic hamartoma. 878 Nov 6

An 11-year-old girl with low-grade fever, night sweats, thrombocytopenia, and an 8-year history of progressive splenomegaly underwent an elective splenectomy. Pathologic diagnosis was multiple splenic hamartoma. The patient's symptoms resolved after the splenectomy. Since first described by Rokitansky in 1861, approximately 140 cases of splenic hamartoma have been described in the literature. Most of the splenic hamartomas were discovered incidentally. A minority of these lesions were associated with hematologic symptoms such as pancytopenia, anemia, and thrombocytopenia. Only 20 of the reported cases of splenic hamartoma occurred in pediatric patients. However, compared with the adult patients, nearly half of these cases in pediatric patients was associated with symptoms. Splenectomy and partial splenectomy have relieved these symptoms. With advances in imaging, splenic hamartomas are being discovered with increasing frequency. A multimodal radiologic work-up has enabled some cases of splenic hamartoma to be diagnosed preoperatively. Inclusion of this benign entity in the differential diagnoses of symptomatic splenomegaly in a pediatric patient is important in the preoperative management and counseling of the patient and family. In patients who have discrete lesions, consideration of this entity preoperatively may avoid total splenectomy.
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PMID:Symptomatic splenic hamartoma: case report and literature review. 956 43

We performed a specimen fine-needle aspiration biopsy (FNAB) of a littoral cell angioma (LCA) from a 33-yr-old male who underwent elective splenectomy due to thrombocytopenia secondary to Wiscott-Aldrich syndrome. Gross examination revealed a 420-g, diffusely enlarged spleen which contained two moderately well-circumscribed, soft brown lesions measuring 0.3 and 1.0 cm, respectively. Benchtop aspiration of the lesions following splenectomy yielded a cellular sample composed predominantly of dispersed single cells, which ranged from columnar to spindle to circariform in shape. Nuclei were round to oval with even chromatin, and many contained single longitudinal grooves. A majority of the cells contained abundant, granular hemosiderin pigment, a key cytologic feature. Immunohistochemical staining revealed reactivity for antibodies to CD68 and factor VIII-related antigen with no reactivity for S-100 protein and CD8. Littoral cell angioma must be differentiated from splenic hamartoma, hemangioma, angiosarcoma, littoral cell angiosarcoma, and epithelioid and spindle cell hemangioendothelioma. A combination of cytologic features and immunohistochemical results should enable an accurate diagnosis.
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PMID:Specimen fine-needle aspiration cytology of littoral cell angioma with histologic and immunohistochemical confirmation. 1061 72

Hamartoma is a rare benign lesion of the spleen. Between 140 and 150 cases seem to have been described so far. Hamartoma of the spleen may appear as a single or multiple lesions which may tend to converge. It appears in all ages, mainly in elderly persons. About 20% of patients were described in paediatric subjects. Half of the patients have no symptoms, so that hamartomas were discovered by chance at autopsy. Other 50% of patients had pain, splenomegaly, haematologic abnormalities (most frequently thrombocytopenia or pancytopenia) and spontaneous rupture with intra-abdominal bleeding. In children, hamartoma of the spleen with haematologic abnormalities may be followed by growth retardation, frequent infections, fever and night sweating. The bigger the hamartoma the greater probability to cause symptoms. The exact preoperative diagnosis is rarely established. Hamartoma has to be taken into account always when tumour of the spleen is diagnosed, particularly in children. Splenectomy is the most frequent treatment of symptomatic hamartoma of the spleen. Partial splenic resection is the preferred surgery whenever it may be carried out, particularly in children. We report a 58 year old woman with a five-year history of left subcostal and lumbar pain in whom in the lower pole of moderately enlarged spleen a tumorous mass, 107 x 75 mm in diameter, was discovered on ultrasonography. She was submitted to splenectomy as well as to cholecystectomy due to gall bladder stones. Histological findings of the spleen showed hamartoma. She had an uneventful recovery. The pain disappeared after surgery. She stayed symptom free so far.
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PMID:[Hamartoma of the spleen]. 1125 88

Hamartomas of the spleen or splenomas, are uncommon benign tumorous growths in this organ which have not been well characterized in children. We report four patients, 4 to 11 years old, who had splenomegaly and splenic "hamartomas" associated with different hematologic conditions (refractory microcytic anemia, sickle cell anemia, hereditary spherocytosis, and dyserythropoietic hemolytic anemia). All patients had total splenectomy as a primary therapeutic approach or to lessen their transfusion requirements. In only one patient was a focal splenic mass identified preoperatively with contrasted computed tomography (CT) scans and magnetic resonance imaging (MRI). None of the patients showed a mass by ultrasonography. Gross examination showed enlarged spleens (315-724 g) which on cut surface revealed a single nodule in one and multiple bulging nodules in three specimens. The nodules varied from 1.3 to 7 cm and were indistinct from the surrounding nonlymphoid splenic (i.e., red pulp) parenchyma. Histology of the nodules showed red splenic pulp with variable histiocytic proliferation, focal extramedullary hematopoiesis, lympho-plasmacytosis, fibrosis, and siderotic-calcific deposits. Intranodular small T- and B-cell lymphoid aggregates but no organized secondary follicles or periarteriolar sheaths were seen. Proliferation antigen Ki-67 (Mib-1) immunostains showed a low (< 5%) proliferation index in the nodules and surrounding tissue. Reticulin stains did not show a capsule or border between the normal spleen and the nodules. The critical histologic differential diagnosis for these lesions is with benign vascular tumors. These can be identified by their more disorderly pattern, by immunohistochemistry and by their higher proliferation index. It is our contention that these splenic nodules are not true hamartomas, as they seem to result from remote ischemic or infectious/inflammatory insults, leading to the fibro-inflammatory reaction and deposition of calcium and hemosiderin that is better designated with the descriptive term of splenoma. Review of the literature and our own experience indicates that most children with splenic hamartomas or splenomas as we prefer to call them, have an underlying hematologic disorder likely made worse by a state of hypersplenism that explains the consistent improvement in the blood values after splenectomy.
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PMID:"Hamartoma" of the spleen (splenoma) in children. 1503 46

Small localized hemangiomas are common neoplasms of the spleen. Isolated diffuse splenic hemangiomatosis, however, is very rare. This lesion can be accompanied by severe hypersplenism and other complications. We report on a case with significant splenomegaly caused by diffuse hemangiomatosis, which was an incidental finding without any clinical disorders. After splenectomy, the normal parenchyma was found to be widely replaced by multiple spongy nodules. Histologically, cavernous vessels were distributed throughout the whole organ, with endothelial cells expressing vimentin, factor VIII and CD 31, but not CD8. Splenic sinus lining cells exhibited a strongly positive reaction with CD8, which became faint and disrupted in highly dilated sinuses in the vicinity of cavernous vessels. In some areas, there seemed to be a gradual transition from cystically dilated splenic sinuses to cavernous vessels. The differential diagnosis must consider other splenic vascular tumors, such as littoral cell angioma, lymphangioma, peliosis of the spleen, and hamartoma. The pathogenesis of diffuse splenic hemangiomatosis is controversial, and a malformative or neoplastic origin is under debate. A derivation from splenic sinusoidal cells was suggested by some authors, but was rejected by others. Our findings cannot exclude a neoplastic origin from splenic sinuses but, finally, the etiology and pathogenesis of this vascular lesion remain uncertain.
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PMID:Isolated diffuse hemangiomatosis of the spleen: case report and review of literature. 1531 Jan 52


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