Cases reported "Lymphatic Diseases"

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1/36. Carcinoma in villous adenoma of ascending colon associated with sarcoid reaction in the regional lymph nodes.

    A 79-year-old woman was admitted to our hospital due to continuous anal bleeding. colonoscopy showed a huge villous tumor on the middle area of the ascending colon. A typical right colectomy and lymph node dissection were performed. The resected specimen showed a villous type tumor located on the ascending colon. The histopathologic investigation demonstrated a moderately differentiated adenocarcinoma arising in a tubulovillous adenoma and extending to the submucosa. Although there was no evidence of metastatic carcinoma in the dissected lymph nodes, epithelioid cell granulomas with multinucleated giant cells lacking in the central caseous necrosis suggested sarcoid reaction.
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2/36. Reversible monoclonal lymphadenopathy in autoimmune lymphoproliferative syndrome with functional FAS (CD95/APO-1) deficiency.

    The FAS (CD95/APO-1) receptor and its ligand play an important role in the initiation of apoptosis under many physiologic conditions. Loss of function mutations of the FAS gene have been described in lpr mice and in humans with autoimmune phenomena, recurrent lymphadenopathies, and hepatosplenomegaly. This syndrome is now called autoimmune lymphoproliferative syndrome type I (ALPS I). Recently, patients with similar clinical symptoms due to a functional FAS deficiency without FAS gene mutations have been distinguished. This disease has been termed autoimmune lymphoproliferative syndrome type II (ALPS II) or autoimmune lymphoproliferative disease (ALD). This report is the first description of the lymph node pathology and immunohistochemistry in a patient with ALPS II. After recurrent bacterial infections, a 4-year-old child developed cervical giant lymphadenopathy suggesting lymphoma. Lymph node histology resembled the findings in Epstein Barr virus-associated posttransplant atypical lymphoproliferations. Confluent sheets of immunoblasts, however, showed a monoclonal expression of IgG/lambda and a monoclonal rearrangement of the JH chain. The same clone was also present in the peripheral blood. Although high-grade lymphoma could not be excluded, the patient's parents insisted on the patient's leaving the hospital with only antibiotic treatment. Surprisingly, the giant lymphadenopathy completely resolved within 7 weeks, and the clone was no longer detectable in the peripheral blood. Twelve months later the patient was still free from lymphoma and was doing well. Retrospectively, transient monoclonal B-cell populations could be identified in an archival frozen blood sample taken when the patient was 3 years old. Increased FAS-independent spontaneous apoptosis was a feature of the patient's lymphocytes and could be the molecular basis for self-elimination of B-cell clones. We conclude that the diagnosis of a FAS-FAS-L deficiency should be considered in children with an otherwise unexplained atypical lymphoproliferation and that a diagnosis of lymphoma in patients with functional FAS deficiency should be made with considerable reservation.
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3/36. Management of a giant lymphatic malformation of the tongue.

    Lymphatic malformations can occur anywhere in the head and neck, and when they do so in the oral cavity, they can present a potential hazard to the airway. We describe a 4-year-old girl with a giant lymphatic malformation of the tongue and neck. This report illustrates the particular difficulties that may be encountered in cases involving giant lymphatic malformations of the cervicofacial region in the pediatric population. An overview of the etiology, diagnosis, histology, and options for the management of such lesions is outlined.
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4/36. Kimura's disease with unusual eosinophilic epithelioid granulomatous reaction: a finding possibly related to eosinophil apoptosis.

    We report and discuss a case of Kimura's disease with an unusual eosinophilic epithelioid granulomatous reaction. A 3-year-old Japanese boy with eosinophilia and a high concentration of IgE developed lymphadenopathy and multiple cervical masses. A lymph node biopsy demonstrated the infiltration of eosinophils in the stroma, which is consistent with the findings of Kimura's disease. Interestingly, a number of apoptotic eosinophils was detected in the infiltrating eosinophils. Multiple epithelioid granulomas with central eosinophilic abscesses and necrosis were also observed. macrophages and giant cells had phagocytosed the apoptotic eosinophils at the edge of the granulomas. In situ terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end-labeling (TUNEL) assay showed that the TUNEL-positive eosinophils were both in the macrophages and in the central eosinophilic abscesses of the granulomas. These findings suggest that the eosinophils had undergone an accelerated apoptosis in this case of Kimura's disease, and that the epithelioid granulomas were produced by phagocytosis of the apoptotic eosinophils by macrophages.
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5/36. Lipogranulomatous lymphadenopathy as a potential source of error in fine needle aspiration cytology. A case report.

    BACKGROUND: Lipogranulomatosis refers to the presence, in lymph nodes and spleen, of lipid material arising from endogenous sources, such as tumors, hematomas, cholesterol deposits, fat embolism and fat necrosis. The histologic image is similar to that seen in lymph nodes after lymphography, with vacuolized histiocytes; round, empty spaces; and numerous multinucleated giant cells (MGC). CASE: A 27-year-old woman was referred to our department for fine needle aspiration of bilateral inguinal lymph nodes. One year before, a giant cell tumor of the sacrum was surgically excised at another medical center. The aspirated material showed a normal lymphoid cell background in addition to several MGC and ill-defined cellular groups of mononuclear, histiocytelike cells admixed with mature lymphocytes. After the cytologic report was issued both nodes were surgically excised. The most remarkable histologic finding was the presence of sinus histiocytosis and multifocal subcapsular and sinusoidal lipogranulomas with numerous MGC. CONCLUSION: Lipogranulomatous lymphadenopathy is a rare pathologic condition and may be responsible for smears with numerous MGC. This entity should be considered when evaluating lymph node aspirates from patients with neoplasia, particularly that with a giant cell component.
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6/36. Fine needle aspiration biopsy cytology as an adjunct in the diagnosis of childhood sarcoidosis.

    Fine needle aspiration biopsy cytology performed in three children with sarcoidosis expedited clinical investigation and diagnosis of their disease. Each patient had a different clinical presentation; in two of them lymphoma was part of the initial differential diagnosis. Aspiration cytology in all cases revealed collections of epithelioid histiocytes, and multinucleate foreign body-type giant cells, without accompanying necrosis or acute inflammation. A diagnosis of non-caseating granulomas consistent with sarcoidosis was made in all aspirates. Special stains for identification of organisms performed on the smears of one case, and culture of aspirate material from one case were negative. Subsequent serum angiotensin converting enzyme levels in all patients were elevated. Chest x-ray films in all patients showed mediastinal and hilar lymphadenopathy. One patient had an interstitial pulmonary infiltrate. All patients responded to steroid therapy. Fine needle aspiration biopsy can be a useful diagnostic tool in the evaluation of children with suspected sarcoidosis.
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keywords = giant
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7/36. Systemic elastolytic granulomatosis with cutaneous, ocular, lymph nodal, and intestinal involvement. Spectrum of annular elastolytic giant cell granuloma and sarcoidosis.

    A 15-year-old Japanese girl had widespread annular serpiginous erythematous plaques, bilateral granulomatous uveitis, bloody diarrhea, and seronegative arthralgia. She also had anemia and leukopenia. The histopathologic findings were compatible with those of annular elastolytic giant cell granuloma. Elastolytic granulomas were also found in the cervical lymph nodes, terminal ileum, parietal peritoneum, and mesentery. Bilateral hilar lymphadenopathy, hypercalcemia, and an increased level of angiotensin converting enzyme were not observed throughout the clinical course. To the best of our knowledge, systemic elastolytic granulomatosis has not been previously described in annular elastolytic giant cell granuloma or sarcoidosis. This case may represent a type of granulomatosis in the broad spectrum of annular elastolytic giant cell granuloma and sarcoidosis.
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ranking = 7
keywords = giant
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8/36. Reactive hyperplasia with giant follicles in lymph node lesions from systemic lupus erythematosus patients. Report of three cases.

    Lymph node enlargement is common in active systemic lupus erythematosus (SLE), a disease that is characterized by well-defined clinical criteria. Histologically, although lymphadenopathy associated with SLE exhibits marked histological diversity and occasionally shows atypical lymphoproliferative disorders, there has not been any description of the histopathological features of reactive lymph node hyperplasia with giant follicles (RHGF). We here report three such cases. The subjects were a 23-year-old Japanese female, a 44-year-old Japanese female and a 49-year-old Japanese male. All three patients initially presented with systemic lymphadenopathy. They also had systemic symptoms and abnormal laboratory findings indicating active disease, although two patients did not fulfill the diagnostic criteria for SLE at lymph node biopsy. Histologically, three lesions were characterized by numerous enlarged, coalescing lymphoid follicles with distortion rather than effacement of the lymph node architecture. By in situ hybridization, Epstein-Barr virus (EBV) genomes were demonstrated in two cases. The present three cases indicate that lymphadenopathy associated with SLE representing RFGH should be differentiated from the early stage of hiv-related lymphadenopathy as well as follicular lymphoma, particularly the floral variant. The authors would like to stress that the RHGF which is described in the present study should be listed in the pathohistology of SLE lymphadenopathy.
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ranking = 5
keywords = giant
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9/36. Silicone lymphadenopathy associated with failed prosthesis of the hallux: a case report and literature review.

    Three years after replacement of a first metatarsophalangeal joint with a silicone prosthesis, a tennis player developed an enlarged ipsilateral femoral lymph node. The implant was also noted to have failed. Fine needle aspiration of the node revealed a foreign body giant cell reaction to particulates morphologically compatible with silicone elastomer. This finding suggests the potential utility of fine needle aspiration in the evaluation of patient response to foreign materials used in prosthetic devices. The lymphadenopathy also suggests that prosthetic metatarsophalangeal replacement in patients with high demand activities run the risk not only of failure of the prosthesis, but also of central migration of the particulate debris resulting from silicone elastomers.
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ranking = 1
keywords = giant
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10/36. Fine needle aspiration cytology of silicone lymphadenopathy in a patient with an artificial joint. A case report.

    Three years after replacement of the left first metatarsophalangeal joint with a silicone prosthesis, a patient noted enlargement of a left femoral lymph node. Fine needle aspiration of the node revealed a foreign body giant cell reaction to particulates morphologically compatible with silicone elastomer. This finding suggests the potential utility of fine needle aspiration in the evaluation of patients' responses to a variety of foreign materials used in prosthetic devices and as pharmaceuticals.
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keywords = giant
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