[A patient with a hemorrhagic intestinal cyst].
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Primary angiosarcoma of the spleen is a very rare neoplasm with a poor prognosis. The definitive diagnosis is usually based on the histologic evaluation of the splenectomy specimen. We describe a case of angiosarcoma diagnosed by fine-needle aspiration cytology prior to splenectomy. A 69-year-old white woman presented with heterogeneous lesions in the spleen during a follow-up computed tomographic scan for a history of liposarcoma of the right buttock. A malignant endothelial neoplasm was diagnosed by fine-needle aspiration cytology using immunocytochemistry, and a splenectomy confirmed the presence of angiosarcoma. To our knowledge, this is the first well-documented and confirmed case of primary angiosarcoma of the spleen diagnosed by fine-needle aspiration cytology. This report emphasizes the value of fine-needle aspiration cytology as an important diagnostic tool in splenic neoplasms.
Splenic fine needle aspiration biopsy (FNAB) is rarely performed in the United States for several reasons: there is a perception that there is an increased risk of bleeding, and rendering a diagnosis of lymphoma (the most common primary splenic neoplasm) by FNAB is controversial. During a three-year period, we saw 11 cases of splenic FNAB. We present four cases of lymphoreticular neoplasms occurring between 1988 and 1991 in which the spleen was the only or most accessible organ in which to establish a diagnosis by FNAB: one case of small cleaved cell lymphoma, two cases of immunoblastic lymphoma (one T cell, one B cell by immunophenotyping studies) and one case of hairy cell leukemia in which the disease was confined to the spleen. All FNABs were radiologically guided, none of the patients suffered significant complications, and all had specimens sufficiently cellular to establish the diagnosis. Splenic puncture with FNAB may be an important and underutilized procedure for establishing a diagnosis in patients who have undetectable disease at other sites.
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BACKGROUND: Primary malignant splenic neoplasms are rare tumors. Apart from involvement of the spleen by malignant lymphoma, hemangiosarcoma is the most common form of primary malignant neoplasm in the spleen. The diagnosis often is made at autopsy or after spontaneous splenic rupture. The prognosis is usually poor, and widespread metastases soon occur. METHOD: Currently, no case of metastasis to the brain has been reported to the authors' knowledge. RESULTS: This case report describes a patient who, as the first sign of metastatic disease, had symptoms from brain metastases 3 months after splenectomy. CONCLUSION: Magnetic resonance imaging was diagnostic.
Although vascular neoplasms of the spleen are rare, they are the most common nonhemopoietic proliferation of the organ, and include hemangiomas, lymphangiomas, hamartomas, littoral cell angiomas, hemangioendotheliomas, and angiosarcomas, as well as the recently described myoid angioendothelioma (MA). MA is an uncommon, benign tumor of the spleen, which is morphologically characterized by a composite of vascular spaces and stromal cells with myoid features. In 1999, in the only report of this unusual neoplasm, Kraus and Dehner described the features of 3 cases. We present another case of MA of the spleen occurring in a 51-year-old man that demonstrated the characteristic morphologic and immunohistochemical features of this neoplasm. In addition to the features described by Kraus and Dehner, our case also displayed the previously unreported findings of focal spindling of the stromal cells and scattered S100-positive cells in the stroma. The case was further unique in having a central stellate scar. Careful attention to histology, possibly with the aid of immunohistochemistry, should distinguish other splenic neoplasms from MA. Although MA is a morphologically distinct lesion, its histologic spectrum, biological behavior, and relationship to other vascular tumors are yet to be fully discovered. It is hoped that the recognition of further cases, and the use of newer molecular technologies, will help better define the nosological position and implications of the diagnosis of this unusual tumor.
PURPOSE: To evaluate the clinical, pathologic, and computed tomographic (CT) features of littoral cell angioma of the spleen in eight patients. MATERIALS AND METHODS: Two abdominal radiologists retrospectively reviewed the contrast material-enhanced CT images obtained in six, the contrast-enhanced and nonenhanced CT images obtained in two, and the photographs of gross pathologic specimens resected from seven patients. They also retrospectively reviewed clinical data (ie, demographic data, presenting signs and symptoms, physical findings, and medical histories). Histopathologic specimens from the eight patients were reviewed by a hematopathologist. The CT images were reviewed for the presence of splenomegaly. The number, size, and enhancement characteristics of the splenic masses at CT were compared with the histopathologic and gross pathologic specimen findings. RESULTS: All patients had laboratory evidence of hypersplenism. Seven patients (88%) had splenomegaly and innumerable splenic masses ranging from 0.2 to 6.0 cm in diameter at CT. The single patient with a normal spleen size had four splenic masses. The splenic masses were hypoattenuating relative to the normal spleen at CT in all patients and correlated with blood-filled nodules at gross pathologic examination and with blood-filled vascular channels of littoral cell angioma at histopathologic examination. The early and late portal venous phase CT images that were available in one case demonstrated progressive homogeneous contrast enhancement of the masses such that they were indistinguishable from the normal splenic parenchyma. CONCLUSION: Littoral cell angioma is a primary splenic neoplasm that most commonly manifests at CT as multiple hypoattenuating masses in an enlarged spleen. Histopathologically, these masses represent blood-filled vascular channels.
In six carcinogenicity bioassays, male and female F344 rats were fed diets containing aniline hydrochloride (CAS: 142-04-1; hydrochloride benzenamide), p-chloroaniline (CAS: 106-47-8), azobenzene (CAS: 103-33-3), o-toluidine hydrochloride (CAS: 636-21-5), dapsone (CAS: 80-08-0; 4,4'-sulfonyldianiline), or D & C red No. 9 [CAS: D85500000; 5-chloro-2-[2-hydroxy-1-naphthalenyl)azo)-4-methylbenzenesulfon ic acid, barium salt]. The rats, from 6 weeks to 2 years old, were given the compounds at two dose levels, the estimated maximum tolerated dose and one-half that dose. In all six bioassays, dose-dependent incidences of splenic sarcomas and fibrosis were seen, with the highest incidences in male rats. Fibrosis occurred in the splenic parenchyma and/or the capsule. Fatty infiltration also was seen in the spleen. Sarcomas appeared to arise in the splenic red pulp or splenic capsule, usually in association with areas of parenchymal and capsular fibrosis and pigmentation. Larger tumors metastasized to the peritoneal cavity and abdominal organs. In some rats there was marked osseous metaplasia when the primary tumor metastasized to peritoneal surfaces. Other, less common, splenic neoplasms included hemangiosarcoma and hemangiopericytoma. Some rats had such extensive peritoneal involvement that the site of origin of their sarcoma was difficult to determine.
Hepatocellular carcinoma (HCC) in an ectopic liver is very rare and its CT findings have never been reported. We present an ectopic HCC between the spleen and left diaphragm, mimicking a splenic neoplasm on the CT scan. A follow up CT at seven months postoperatively revealed multiple recurrent HCCs in the mother liver.
Superparamagnetic iron oxide (SPIO) particles are a potent new class of MR contrast agents affording improved detection of hepatic and splenic neoplasms. In this report we review the development of this agent through preclinical studies and early clinical results at Massachusetts General Hospital during a 5-year investigation. SPIO particles are sequestered by normal phagocytic Kupffer cells of the reticuloendothelial system (RES) but are not retained in tumor tissue. Consequently, there is a fivefold increase in T2 relaxation between normal RES tissue and tumor, with a comparable advantage in quantitative signal-to-noise ratio, contrast-to-noise ratio, and lesion detectability in the liver and spleen on MR imaging. Increased lesion conspicuity can be exploited to decrease threshold size for lesion detection to less than 3 mm. Clinically beneficial effects occur with a variety of mildly T2-weighted spin-echo pulse sequences; gradient-echo techniques show even greater benefit after administration of SPIO. Metabolically, pharmaceutical-grade preparations are biodegradable and bioavailable, being rapidly turned over into body iron stores and incorporated into erythrocyte hemoglobin. Early dose-escalation clinical trials have identified a probable clinical dose range of 10-20 mumols Fe/kg body weight. In the United States, SPIO compounds evaluated to date are still approved for use in investigational studies only. Newer commercial formulations currently being evaluated may extend clinical safety margins.
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The splenic marginal zone is a morphologically and perhaps immunologically distinct B-cell compartment. Lymphomas arising from cells of the splenic marginal zone are rare. Here we describe the morphologic, immunologic, and clinical features of 14 cases. Patient age ranged from 35 to 79 years (median, 68 years) with a male-to-female ratio of 1:1.8. The spleen was uniformly enlarged (median, 1,540 g; range, 388-3,845 g) in all patients, the neoplastic infiltrate had a nodular pattern in three cases, nodular and diffuse in seven cases, and diffuse in four cases. The neoplastic cells had small to medium-sized nuclei with round, oval, or slightly indented contours, small eosinophilic nucleoli, and a moderate amount of pale cytoplasm. Extrasplenic involvement was present in 12 patients. Lymph nodes often had a vaguely nodular pattern and preservation of sinuses; bone marrow was infiltrated focally (seven cases) or diffusely (one case). Five patients had hepatic involvement. Ultrastructurally, neoplastic cells differed from other small B cells and resembled normal marginal zone cells by having long, serpentine rough endoplasmic reticulum profiles. All lymphomas marked as B cells and light chain restriction was demonstrated in 12 cases. Bcl-2 protein expression was present in all cases. Most cases (70%) were negative for DBA.44 (CD72). Plasmacytic differentiation was present in three cases. In conclusion, splenic marginal zone lymphoma is a B-cell neoplasm with distinctive clinical, morphologic, immunologic, and ultrastructural characteristics.
The splenic index was determined in 155 persons, without evidence of splenic disease, in order to obtain a simple measure of splenic size. Subsequently 36 patients with malignant lymphomas and who then had their spleens removed were examined by CT. A comparison of the CT and pathological-anatomical findings showed that it is a valuable non-invasive method for diagnosing splenic involvement, having a specificity of 86%, sensitivity of 77% and accuracy of 83%.
Splenic inflammatory pseudotumor is extremely rare and may mimic splenic neoplasms, such as lymphomas or hamartomas, clinically and radiologically. A case of a surgically proved splenic inflammatory pseudotumor is presented in which Tc-99m colloid SPECT and Ga-67 scintigraphy characterized the changes in the spleen, but the findings of ultrasound and unenhanced CT and MRI were nonspecific. This report indicates the utility of radionuclide imaging for diagnosing splenic inflammatory pseudotumor.
Splenic arterial interventions are increasingly performed to treat various clinical conditions, including abdominal trauma, hypersplenism, splenic arterial aneurysm, portal hypertension, and splenic neoplasm. When clinically appropriate, these procedures may provide an alternative to open surgery. They may help to salvage splenic function in patients with posttraumatic injuries or hypersplenism and to improve hematologic parameters in those who otherwise would be unable to undergo high-dose chemotherapy or immunosuppressive therapy. Splenic arterial interventions also may be performed to exclude splenic artery aneurysms from the parent vessel lumen and prevent aneurysm rupture; to reduce portal pressure and prevent sequelae in patients with portal hypertension; to treat splenic artery steal syndrome and improve liver perfusion in liver transplant recipients; and to administer targeted treatment to areas of neoplastic disease in the splenic parenchyma. As the use of splenic arterial interventions increases in interventional radiology practice, clinicians must be familiar with the splenic vascular anatomy, the indications and contraindications for performing interventional procedures, the technical considerations involved, and the potential use of other interventional procedures, such as radiofrequency ablation, in combination with splenic arterial interventions. Familiarity with the complications that can result from these interventional procedures, including abscess formation and pancreatitis, also is important.
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Spontaneous rupture of the spleen secondary to metastatic carcinoma is rare. We report the second such case. Rupture is usually diagnosed at laparotomy. However ultrasound and computerized tomography can play a role in diagnosis.