1/18. Histiocytic lesion mimicking intrinsic brainstem neoplasm. Case report.This 10-year-old girl presented with a 1-month history of progressive bulbar palsy and a solitary enhancing mass originating within the floor of the fourth ventricle. Results of initial imaging studies and presentation were suggestive of neoplasia. Subtotal resection was performed and pathological examination revealed the mass to be a histiocytic lesion, with no evidence of a glioma. The patient had no other stigmata of histiocytosis and was treated with steroid medications, resulting in prolonged resolution of the lesion. This case demonstrates that for discrete brainstem lesions the differential diagnosis includes entities other than glioma for which treatment is available. biopsy sampling should be considered when technically feasible.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/18. Thermophilic multidrug-resistant campylobacter fetus infection with hypersplenism and histiocytic phagocytosis in a patient with acquired immunodeficiency syndrome.We present a case report of a patient who had acquired immunodeficiency syndrome (AIDS) and campylobacter fetus infection with a number of unusual clinical and microbiological features. The patient had prominent gastrointestinal symptoms, splenic infarction, splenomegaly with hypersplenism, and hemophagocytic histiocytosis in the spleen and lymph nodes; the organism displayed growth on Campy-selective blood agar, thermotolerance, and resistance to quinolones, piperacillin/tazobactam, ceftazidime, and erythromycin.- - - - - - - - - - ranking = 2.0762349117837keywords = spleen (Clic here for more details about this article) |
3/18. Extranodal marginal zone B-cell lymphoma of the lacrimal gland associated with crystal-storing histiocytosis.OBJECTIVE: Crystal-storing histiocytosis (CSH) is a very rare immunoglobulin (Ig) deposition disorder that can be associated with B-cell neoplasms as well as some reactive Ig-secreting lymphoproliferative disorders. This article reports the clinical, histopathologic, and molecular biologic findings of CSH in association with an extranodal marginal zone lymphoma (EMZL) of the lacrimal gland. DESIGN: Interventional case report with clinicopathologic correlation. INTERVENTION: Treatment consisted of conjunctival and orbital biopsies, as well as low-dose radiation of the left orbit. methods: Histopathologic findings of the conjunctival and orbital biopsies were compared. Further, extensive immunohistochemistry, polymerase chain reaction (PCR) for the detection of Ig heavy chain (IgH) gene rearrangement, Gene Scan analysis, and dna sequencing were performed on all tissues. RESULTS: The tumor manifestations in the specimens demonstrated a similar morphologic and immunophenotypic characteristics consistent with the diagnosis of EMZL. Immunoglobulin H PCR and Gene Scan analysis showed B cells derived from the same clone. In association with the orbital EMZL were large accumulations histiocytes filled with refractile crystals, consistent with the diagnosis of CSH. CONCLUSIONS: The current case describes an EMZL of the lacrimal gland appearing 5 years after excision and low-dose radiation of a conjunctival lymphoma. The orbital recurrence was associated with surrounding CSH, a rare Ig storage disorder more often reported in patients with multiple myeloma or lymphoplasmacytic lymphoma or immunocytoma. Ophthalmic pathologists should be aware of the diagnosis when reviewing orbital biopsies, because difficulties may arise when the crystal-storing macrophages occupy more tissue space than the Ig-secreting cells or when they resemble cell types such as rhabdomyoblasts.- - - - - - - - - - ranking = 0.25keywords = neoplasm (Clic here for more details about this article) |
4/18. Lymphoplasmacytic lymphoma with monoclonal gammopathy-related pseudo-Gaucher cell infiltration in bone marrow and spleen--diagnostic and therapeutic dilemmas.Gaucher-like cells have occasionally been described in various haematological malignancies including Hodgkin's disease, non-Hodgkin's lymphoma, multiple myeloma (MM) and chronic myelogenous leukaemia (CML). A special type of this phenomenon is crystal-storing histocytosis or the so-called pseudo-pseudo Gaucher cells (PPGC) in which crystalline protein storage in macrophages is induced by paraproteinemia. Here we describe a 54-year-old man with an initial suspicion of gaucher disease and monoclonal IgA gammopathy in whom a correct diagnosis of lymphoplasmacytic lymphoma (LPL) with massive infiltration of bone marrow and spleen by PPGC was confirmed by immunological, ultrastructural and molecular characterisation. The activity of leukocyte beta-glucocerebrosidase was only slightly elevated (7.3 nmol/mg protein/1 h) which ruled out the diagnosis of classic Gaucher's disease. The patient received two courses of CHOP without improvement and anti-CD20 monoclonal antibody (rituximab) with only temporary stabilisation. Subsequently, he underwent splenectomy because of prolonged severe pancytopenia and a suspicion of hypersplenism. After splenectomy significant haematological improvement was observed. Following anti-CD20 therapy, changes in immunoprofile and morphology of tumour cells were evident. Before treatment the population of LPL was more divergent, with expression of LCA, CD20, CD38 and CD138. However, after the treatment, there were more mature plasma cells which no longer expressed CD20 antigen-this picture was more consistent with the diagnosis of plasma cell myeloma. Similarly, in the spleen there were no CD-20-positive cells evident. Finally, the patient received two courses of VAD vincristine, doxorubicin, dexamethasone) with further haematological improvement but complete response was not achieved.- - - - - - - - - - ranking = 12.457409470702keywords = spleen (Clic here for more details about this article) |
5/18. Postarthroplasty histiocytic lymphadenopathy in a patient with uterine carcinoma.INTRODUCTION: Postarthroplasty histiocytic lymphadenopathy is a benign reactive sinus histiocytosis. CASE REPORT: We report on the case of a 68-year-old woman presenting with pelvic postarthroplasty histiocytic lymphadenopathy after hysterectomy because of uterine carcinoma. CONCLUSION: This unusual form of histiocytic lymphadenopathy should be included in the differential diagnosis of gynecologic oncology patients who have had joint replacements to avoid misdiagnosis of a neoplasm.- - - - - - - - - - ranking = 0.25keywords = neoplasm (Clic here for more details about this article) |
6/18. histiocytosis X with thyroid involvement: report of a case.histiocytosis X is a disease of histiocyte proliferation in response to some unknown etiology. Thyroid involvement is extremely rare in the literature. In this paper, we present an 18-year-old female with histiocytosis X with thyroid involvement. This patient had had a goiter with normal thyroid function since 12 years of age. A thyroidectomy was done under the suspicion of thyroid cancer. pathology revealed histiocytosis X. hypoparathyroidism and hypothyroidism were noted after the operation and were treated with thyroid hormone, vitamin d and calcium carbonate. This patient also had lesions on the left side of mandible, in the suprasellar region and possibly in the right mastoid. A curettage biopsy of her mandibular lesion was also compatible with histiocytosis X. She was proven to have hypothalamic and pituitary dysfunction including hypogonadism and hypoadrenalism. Her thyroid lesion did not recur after the thyroidectomy. The toothache that she had also experienced subsided after the curettage biopsy of the mandibular lesion. Hypothalamic and pituitary dysfunction were controlled by hormone replacement. Because her disease had been running a benign clinical course, no chemotherapy was given.- - - - - - - - - - ranking = 0.062045547861466keywords = cancer (Clic here for more details about this article) |
7/18. Hemophagocytic syndrome and hepatosplenic gammadelta T-cell lymphoma with isochromosome 7q and 8 trisomy.The authors describe a 15-year-old boy with hepatosplenic gammadelta T-cell lymphoma associated with hemophagocytic syndrome (HPS) along with isochromosome 7q and trisomy 8. He presented with prolonged fever, mild anemia, thrombocytopenia, and hepatosplenomegaly. physical examination, radiography, and ultrasound tomography revealed no lymphoadenopathy. He had elevated levels of serum ferritin, interferon-gamma, soluble interleukin-2 receptor, and interleukin-6. bone marrow aspirate showed hypercellularity with 50% lymphoblasts and erythrophagocytosis of macrophage. A cytogenetic study of bone marrow revealed an abnormal karyotype, 47,XY,I(7q), 8, in 5/30 cells. Clonal rearrangement of the genes for T-cell receptor gamma and delta chains was elucidated by polymerase chain reaction. He achieved a complete remission after intensive chemotherapy and underwent splenectomy 18 months after diagnosis. Although the patient was clinically in remission, minimal residual disease (MRD) was detected in the removed spleen by polymerase chain reaction. This might mean that this type of lymphoma is refractory, as reported previously, and might indicate that marrow ablative therapy is needed to achieve a cure. The present case illustrates the usefulness of MRD analysis, and MRD studies in this group of disorders may be helpful in the decision of whether to continue a more aggressive therapeutic approach.- - - - - - - - - - ranking = 2.0762349117837keywords = spleen (Clic here for more details about this article) |
8/18. Nodular histiocytic/mesothelial hyperplasia: a potential pitfall.We present five cases of nodular histiocytic/mesothelial hyperplasia (two peritoneal, two pulmonary, and one pericardial) with identical microscopic features. All the lesions were biphasic and composed of cohesive monotonous epithelioid clusters of polygonal or oval cells with round or deeply grooved nuclei in association with darker cuboidal cells. Because of the increased cellularity and monotonous histologic pattern with some degree of cytologic atypia, neoplastic processes were seriously considered in the differential diagnoses. The majority of the cells marked as histiocytes by immunostain. A few scattered individual cells or small epithelial cell clusters were confirmed by calretinin stain to be mesothelial cells. The histologic patterns of the current lesions, irrespective of the location, were identical to nodular histiocytic/mesothelial hyperplasia. Histiocytic proliferations can be erroneously confused with primary mesothelial lesions or neoplasms such as granulosa cell tumor, eosinophilic granuloma, chronic myelogenous leukemia, and carcinoma. The purpose of this article is to describe the clinicopathologic features of nodular histiocytic/mesothelial hyperplasia and help familiarize pathologists with this lesion to prevent an erroneous diagnosis, particularly when it occurs in locations where mesothelial cells are not normally present.- - - - - - - - - - ranking = 0.25keywords = neoplasm (Clic here for more details about this article) |
9/18. Intra-abdominal, crystal-storing histiocytosis due to clofazimine in a patient with lepromatous leprosy and concurrent carcinoma of the colon.We report a case with abdominal complications of clofazimine treatment which included blackish discolouration of the lymph nodes, omentum and peritoneum. A 44-year-old female with lepromatous leprosy and a history of adverse reaction to clofazimine 2 years previously, presented with rectosigmoid junction adenocarcinoma. laparotomy revealed an inoperable tumour with pigmentation of the bowel, serosa and peritoneum. A second operation had o be performed for transverse loop colostomy and a mesenteric lymph node biopsy sent for frozen section showed typical clofazimine crystals. Despite widespread use for many years in the treatment of leprosy, this drug is not known to be carcinogenic and this case provides no evidence for an association or link between its use and the patient's cancer. Apart from its use in leprosy, clofazimine may be used in the treatment of disseminated mycobacterium avium-intracellulare infection, buruli ulcer due to M. ulcerans and occasionally in other mycobacterial infections. An awareness of the rare side-effect described above may help in the clinical assessment and management of such cases, including the avoidance of unnecessary laparotomy.- - - - - - - - - - ranking = 0.062045547861466keywords = cancer (Clic here for more details about this article) |
10/18. Massive bone marrow crystal-storing histiocytosis in a patient with IgA-lambda multiple myeloma and extensive extramedullary disease. A case report.We report a case of multiple myeloma (MM) displaying an unusual course, with metastatic spread in uncommon sites (gastroduodenal and upper respiratory tract, breast, skin and liver) and with a fatal outcome. In our patient this plasma cell neoplasm was associated with a rare condition named crystal-storing histiocytosis (CSH), resulting from the storage in reactive histiocytes of crystalline immunoglobulin inclusions. These crystal-forming paraprotein components are secreted by the neoplastic plasma cells and give rise to the crystalline material of the histiocytes only after their ingestion and degradation by the same histiocytes. In this disorder crystal-storing cells may be present in various tissues (in our case mainly in bone marrow), often with functional alterations of the involved organs. In our opinion the association of this "atypical" MM with CSH is to be considered an uncommon event.- - - - - - - - - - ranking = 0.25keywords = neoplasm (Clic here for more details about this article) |
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