Cases reported "Lymphatic Diseases"

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1/21. PET and PLAP in suspected testicular cancer relapse: beware sarcoidosis.

    A 31-year-old man previously treated with chemotherapy for metastatic testicular cancer presented with new mediastinal lymphadenopathy and peripheral lung opacities. serum tumour markers were not elevated and a PET (positron emission tomography) scan revealed increased FDG (fluoro-deoxyglucose) uptake in the lungs and mediastinum consistent with testis cancer relapse. A biopsy of a mediastinal lymph node was performed and the pathology was that of sarcoidosis. immunohistochemistry however was positive for PLAP (placental alkaline phosphatase) and negative for EMA (epithelial membrane antigen). This immunohistochemical profile raised concerns that the observed pathology represented a sarcoid reaction to micro-metastatic testicular cancer relapse. We performed immunohistochemical pathology analysis on four known cases of sarcoidosis and found the same immunohistochemical-staining pattern. This case highlights the problem of specificity when interpreting the significance of PET scans and immunohistochemical analysis in this situation. sarcoidosis, a condition that has been associated with testicular cancer, should always be considered in the differential diagnosis.
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2/21. T-cell receptor gammadelta T-cell leukemia with the morphology of T-cell prolymphocytic leukemia and a postthymic immunophenotype.

    T-cell prolymphocytic leukemia (T-PLL) is a postthymic T-cell neoplasm with a characteristic morphology and heterogeneous immunophenotype. Most cases of T-PLL express membrane T-cell receptors (TCRs) of the alphabeta phenotype. We experienced a 30-year-old man suffering from TCRgammadelta T-cell leukemia with morphology compatible to T-PLL with a postthymic phenotype. He was admitted with skin eruption and pancytopenia. Peripheral blood and bone marrow were occupied with medium-sized lymphocytes, which had moderately condensed chromatin with a single nucleolus and sparse, nongranular basophilic cytoplasm. The immunophenotype was CD1a-, CD2-, CD3 , CD4-, CD5 , CD7 , CD8-, and terminal deoxynucleotidyl transferase negative. Hepatosplenomegaly was absent. He was diagnosed as having T-PLL and was treated with combination chemotherapy. Six months later the leukemic cell became chemoresistant. Although the patient showed transient improvement in response to pentostatin, he died 13 months after the diagnosis. To our knowledge, this is the first case of T-PLL with a TCRgammadelta phenotype.
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3/21. An unusual tonsillolith.

    A case of a large tonsillolith of the left palatine is presented. The lesion was detected incidentally during radiographic examination of a patient presenting with right mandibular pericoronitis. The patient had no symptoms referable to the left tonsillar region. Superimposition of the tonsillolith on the left mandibular ramus on panoramic radiographic examination created the false impression of an intraosseous radiopaque lesion. Many radiopaque structures and lesions occur in the soft tissues close to the jaws and are often observed on panoramic radiographs produced by imaging units with broad focal troughs. Such radiopacities in the mandibular molar-ramus region include sialolith, phlebolith, cysticercosis and calcified lymph node, carotid artery arteriosclerosis, stylohyoid ligament mineralization, and dystrophic calcification in acne scars. Tonsilloliths may present on panoramic radiograph as radiopaque objects superimposed on the midportion of the ascending mandibular ramus, and may be initially misinterpreted as intraosseous lesions of the mandible, such as foreign body, odontoma, sclerosing osteitis, Garre osteomyelitis, fibrous dysplasia, idiopathic osteosclerosis, and osteoma.
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4/21. Hilar cholangiocarcinoma associated with sarcoid reaction in the regional lymph nodes.

    A 74-year-old woman was admitted to our hospital with a 2-week history of jaundice. Percutaneous transhepatic cholangioscopy revealed a nodular tumor originating in the upper part of the common hepatic duct, which was invading the confluence of the right and left hepatic ducts. Microscopic examination of biopsy specimens revealed adenocarcinoma. Abdominal ultrasonography and computed tomography demonstrated multiple enlarged lymph nodes around the extrahepatic bile duct and the common hepatic artery. laparotomy revealed lymph node enlargement in the hepatoduodenal ligament, behind the pancreatic head, and along the common hepatic and left gastric arteries. Extended left hepatic lobectomy, caudate lobectomy, and resection of extrahepatic bile duct with extended lymph node dissection were performed. The histology of permanent specimen revealed no tumor metastasis but a sarcoid reaction in the lymph nodes. The patient is in good health 21 months after the operation, without any evidence of recurrence. This is the first successfully resected case of hilar cholangiocarcinoma associated with sarcoid reaction in the regional lymph nodes.
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5/21. Endometrioid carcinoma of the ovary presenting with an enlarged inguinal lymph node without evidence of abdominal carcinomatosis.

    It is generally recognized that ovarian cancer tends to remain intraabdominal even in advanced cases and that dissemination is usually by invasion of adjacent viscera, diffuse intraperitoneal implantation, and metastatic involvement of aortic and pelvic lymph nodes. Primary ovarian lymphatic drainage occurs via the infundibulopelvic ligament to the paraaortic nodes. The presence of an ovarian tumor extending into adjacent pelvic viscera may allow direct lymphatic continuity with inguinal, external, and common iliac lymph nodes. In the absence of such extension it is traditionally believed that the drainage via the infundibulopelvics is so important that only with its blockage, presumably by tumor emboli, can retrograde drainage to pelvic and inguinal nodes occur. We report a case of a patient presenting with a large metastatic inguinal lymph node from a primary epithelial ovarian cancer without evidence of disseminated intraabdominal disease or gross evidence of pelvic or paraaortic lymph node involvement.
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6/21. Expanded populations of surface membrane immunoglobulin light chain-negative B cells in lymph nodes are not always indicative of B-cell lymphoma.

    Immunophenotypic analysis is useful in distinguishing reactive from neoplastic lymphoproliferations, particularly when tissue is limited or histologic findings are equivocal. Surface membrane immunoglobulin (SmIg) light chain restriction in B cells is especially helpful in documenting clonality, and the loss of SmIg by B cells in extramedullary sites also has been used as a criterion to support the presence of lymphoma. However, we identified 3 cases of benign follicular hyperplasia (in 101 cases analyzed) with profound expansions (56%-88% of the B cells) of SmIg light chain-negative B cells without clonality by immunoglobulin heavy chain gene polymerase chain reaction. Thus, although uncommonly encountered, lack of SmIg light chain expression by B cells should not necessarily be interpreted as indicative of lymphoma. Interestingly, 2 of the 3 patients with these "aberrant" expansions were hiv , and such patients are at heightened risk for the development of lymphoma. Therefore, there is the potential for misdiagnosing lymphoma if flow cytometric data are interpreted inappropriately in isolation.
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7/21. Sinus histiocytosis with massive lymphadenopathy--a review of seven cases.

    Sinus histiocytosis with massive lymphadenopathy (SHML) also called as Rosai Dorfman disease is a rare histiocytic proliferative disorder of unknown etiology. Histological features currently define it. Persistent painless lymphadenopathy due to expansion of sinuses infiltrated with benign histiocytes and plasma cells and emperipolesis are the characteristic features of SHML. Our study includes seven cases (5 nodal and 2 extranodal) of SHML over a 5-year period whose slides and blocks were reviewed. IHC was performed on the main lesion, from a panel of S100, CD68, LCA, CD20, CD3, CD30, CD43, bcl2, cytokeratin and epithelial membrane antigen. In our series we have work up available in 7 cases out of which a detailed follow-up is available in 5 patients. Out of these 5 patients, 4 have a stable disease, while one developed histiocytic sarcoma after a gap of four years.
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8/21. Mesothelial cell inclusions in mediastinal lymph nodes mimicking metastatic carcinoma.

    The authors report two extremely unusual cases in which metastatic cancer was mimicked by mesothelial cell inclusions in mediastinal lymph nodes. The cells appeared only in the nodal sinuses and occurred predominantly as single individual cells and small clusters. The nuclei were bland, the N/C ratio was low, and the cell borders were well defined. So-called mesothelial windows were noted when cells formed groups; mitoses were not observed. Immunohistochemical analysis demonstrated the inclusions to be positive for cytokeratin (both AE1/3 and CAM5.2) but negative for epithelial membrane antigen, Leu-M1, and carcinoembryonic antigen. Nearly all cells were negative for B72.3; rare cells in one case contained unusual minute granular dot-like positivity in the region of the Golgi for this marker. The pattern of cytokeratin immunoreactivity was consistent with a mesothelial cell: namely, stronger immunoreactivity in a perinuclear location with some fading at the cell periphery. Ultrastructural analysis of both cases documented long microvilli processes consistent with a mesothelial origin. An extensive clinical workup in each case has failed to identify a primary carcinoma. It is interesting that both patients had a pleuritis with pleural effusion and both had mediastinal widening. In the first case, the exact cause of the benign pleural process was unknown but thought to be infectious. The second patient had follicular lymphoma in the same lymph node together with pleural involvement clinically and evidence of congestive heart failure. The patients are alive three years and ten months from diagnosis, respectively. Recognition of this new and previously unrecognized entity is important to prevent a diagnosis of carcinoma in such rare instances.
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9/21. Lymphadenopathy due to fatal histiocytic proliferative disorder containing Michaelis-Gutmann bodies.

    A 59-year-old white man had generalized lymphadenopathy, fever, weight loss, and hypercalcemia. Histologic examination of seven lymph nodes from three different anatomic sites revealed a diffuse histiocytic infiltrate containing numerous typical Michaelis-Gutmann (MG) bodies. Histochemical and immunohistochemical results confirmed the histiocytic nature of the predominant cell within the infiltrate. Approximately equal numbers of residual normal B-cells, T-helper, and T-suppressor cells were present. Electron microscopy revealed extracellular and intracellular MG bodies, many of which were membrane-bound. Special stains, electron microscopy, and lymph node culture did not demonstrate microorganisms. The histologic features of the infiltrate were those of malacoplakia. However, the disease behaved like a lymphoma, with the occurrence of lymphadenopathy and a fatal outcome.
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10/21. Hansen's disease (leprosy). diagnosis by aspiration biopsy of lymph nodes.

    A 61-year-old male native of mexico presented with generalized enlargement of lymph nodes. Fine needle aspiration (FNA) biopsy established lepromatous leprosy as the cause of the lymphadenopathy. The cytologic findings included abundant, frequently multinucleated histiocytes (globus cells), the cytoplasm of which showed multiple vacuoles; cytoplasmic membrane-bound vacuoles were seen free in the background. The vacuoles contained large numbers of acid-fast bacilli. Globus cells, while characteristic, are not specific for mycobacterium leprae infection and are seen in certain atypical mycobacterioses in immunodeficient patients. This appears to be the first report of lymphadenopathy due to lepromatous leprosy in which the diagnosis was made by FNA biopsy. The immunologic spectrum of leprosy is correlated with clinical and pathologic findings, and the need to remember infectious processes in evaluating lymphadenopathy and the value of reserving air-dried and alcohol-fixed smears for special stains are emphasized.
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