1/12. Temporary response of localized intracranial mast cell sarcoma to combination chemotherapy.Cerebral involvement of systemic mastocytosis and intracranial sarcoma of myelogenic origin are well known entities. An 8-year-old girl with an isolated cerebral mast cell tumor is presented. Specific histopathologic stains were used to confirm the diagnosis detecting immunophenotype and proliferative activity. Treatment with irradiation, intrathecal cytarabine, and interferon-alpha2b did not induce regression whereas polychemotherapy did. Systemic combination chemotherapy led to marked transient tumor regression in this proliferating mast cell sarcoma in an unusual intracranial location.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
2/12. Combined melanocytoma-mastocytoma in a case of nodular mastocytosis.A patient with long-standing nodular mastocytosis developed a slate-blue lesion on the scalp which produced symptoms of histamine release on mechanical and thermal stimulation. light and electron microscopic examination of the lesion showed zones of mast cells and zones of melanized cells. A transition zone showing cells with dual (melanosomal and mast) granulation, as well as granules representing intergrades between classical mast granules and melanosomes, was present. Features of this lesion add to the accumulated evidence that there is a histogenetic relationship between mast cells and melanocytes.- - - - - - - - - - ranking = 5keywords = mastocytosis (Clic here for more details about this article) |
3/12. Aggressive systemic mastocytosis.Systemic mastocytosis is a rare and occasionally aggressive condition that raises major diagnostic challenges. We report a case in a 72-year-old patient in whom the diagnosis of malignant mastocytosis required two bone marrow smears and three bone marrow biopsies examined using specific staining techniques. Despite interferon therapy, a mast-cell sarcoma of the sternum developed 1 year after symptom onset, followed 1 year later by acute myeloblastic leukemia, which was rapidly fatal.- - - - - - - - - - ranking = 33.552421659153keywords = mast-cell, mastocytosis (Clic here for more details about this article) |
4/12. Morphologic and immunophenotypic properties of neoplastic cells in a case of mast cell sarcoma.Mast cell sarcoma is an extremely rare and aggressive type of mast cell disease. Only a few cases have been described so far, and little is known about the biology and phenotype of afflicted cells. We describe morphologic and immunophenotypic properties of neoplastic mast cells in a case of an intracranial mast cell sarcoma. In Wright-Giemsa-stained cytospin preparations, the morphology of dispersed cells appeared to be highly atypical with a considerable percentage of metachromatic blasts and mast cells with bilobed or multilobed nuclei. Combined toluidine blue/immunofluorescence staining revealed expression of CD13, CD45, CD88, CD116, and CD117 (c-KIT) on neoplastic mast cells. As assessed by immunohistochemistry, mast cells were immunoreactive for tryptase and CD68R, In contrast, the CD2 antigen that is expressed in mast cells in patients with indolent systemic mastocytosis was not detectable. mast cells also failed to display the c-KIT mutation Asp-816-Val, which is typically found in systemic mast cell disorders. Together, neoplastic mast cells in a case of mast cell sarcoma were found to exhibit unique morphologic, phenotypical, and molecular features when compared with mast cells in indolent mastocytosis or normal tissue mast cells.- - - - - - - - - - ranking = 2keywords = mastocytosis (Clic here for more details about this article) |
5/12. Malignant systemic mastocytosis.Malignant Systemic mastocytosis is a very rare condition. Only about less than 40 well documented cases have been reported as per the available literature. The paper presents the case report of a 54 year old male patient who presented with huge hepatosplenomegaly and abdominal lymphadenopathy. splenectomy specimen was 17 x 16 x 10 cm size with cut surface studded with numerous tiny 1-2 mm nodules. Histologic sections of spleen showed extensive mast cell (typical and atypical) infiltrates. liver biopsy and abdominal lymphnode biopsy specimens and bone marrow smears also showed similar infiltration by mast cells. Special stains done for non-specific esterase and chloracetate esterase showed strong positivity for mast cells. The results of immunohistochemical and electron microscopic studies are also presented.- - - - - - - - - - ranking = 4keywords = mastocytosis (Clic here for more details about this article) |
6/12. Fibrous mastocytoma in a patient with generalized cutaneous mastocytosis.A 57-year-old woman with cutaneous mastocytosis of 23 years duration developed a hyperpigmented abdominal plaque composed of confluent indurated papules that enlarged for a period of 1 year to 12 x 8 cm. biopsy showed dermal infiltration by closely packed spindle-shaped mast cells, fibroblasts, collagen, and scattered lymphocytes, predominantly T-suppressor cells. Electron microscopy showed close contact between mast cells, fibroblasts, and lymphocytes. Piecemeal mast cell degranulation and extrusion of mast cell granules was seen, with rare mast cell granules in fibroblasts, and collagen fibers in peripheral and perinuclear endoplasmic reticulum of mast cells. the term Fibrous mastocytoma is suggested for this tumor-like dermal fibrosis, possibly induced by lymphokines.- - - - - - - - - - ranking = 5keywords = mastocytosis (Clic here for more details about this article) |
7/12. Malignant mastocytosis with circulating mast cells.A case of malignant mastocytosis with peripheral blood involvement is presented. The course of the patient's illness was complicated by recurrent hypotensive episodes, presumed to have been caused by mast cell degranulation. Treatment with hydroxyurea was associated with persistent hypotension which resulted in death. It has been proposed that the diagnosis of mast cell leukemia be given to patients presenting with greater than 10% atypical mast cells in the blood. However, review of 16 published cases of malignant mastocytosis with circulating mast cells reveals that the clinical manifestations, complications, and survival do not vary significantly with the percentage of peripheral blood mast cells. patients with malignant mastocytosis with significant involvement by atypical mast cells in the bone marrow and peripheral blood should be considered as having an aggressive disease, regardless of the percentage of circulating mast cells.- - - - - - - - - - ranking = 7keywords = mastocytosis (Clic here for more details about this article) |
8/12. Mast cell sarcoma of the larynx.A 74 year old woman presented with a primary subglottic tumour. Neither cutaneous mastocytosis (urticaria pigmentosa) nor spread to the bone marrow, liver, or spleen were detected. About two years after initial manifestation of the tumour nodular skin metastases appeared, as well as local recurrence in the larynx. Despite chemotherapy and radiation the disease progressed and was fatal. The terminal phase was characterised by generalisation of the mast cell tumour with diffuse infiltration of bone marrow and, shortly before death, leukaemic transformation. The patient died four years after onset of disease with symptoms of a hemorrhagic diathesis. As far as we know this is the first case of mast cell sarcoma to be reported in man.- - - - - - - - - - ranking = 1keywords = mastocytosis (Clic here for more details about this article) |
9/12. Systemic mastocytosis with extensive large cutaneous mastocytomas: surgical management.A case of systemic mastocytosis is reported with an observation period of 20 years. During these two decades multiple manifestations of the disease appeared including urticaria pigmentosis, episodic histamine release, gastro-intestinal involvement and hepatosplenomegaly. The most extraordinary, and possibly unique phenomenon, has been the development of a massive proliferation of large mastocytomas mainly, but not exclusively, limited to the lower extremities. For different reasons (mechanical disability, bleeding, cosmesis), these tumors have required repeated admissions for surgical removal. The most successful technique has involved use of the ultrasonic scalpel.- - - - - - - - - - ranking = 5keywords = mastocytosis (Clic here for more details about this article) |
10/12. Diffuse cutaneous mastocytosis with multiple cutaneous mastocytomas. Report of a case with clinical, histopathological and ultrastructural aspects.A patient with diffuse cutaneous mastocytosis, present since birth, developed several cutaneous mastocytomas without any symptoms after puberty. Although the histological picture of the tumours, showing nuclear atypia, mitotic figures and varying metachromasia of the granules, raises suspicion of systemic involvement, this has not appeared so far. The absence of symptoms could be due to the synthesis of an abnormal secretory product by the mast cells, which is supported by the absence of oedema and dilated blood vessels in the presence of extensive degranulation and by the unusual ultrastructural picture of the mast cell granules, i.e. little electron density with the almost complete absence of lamellar and crystalline structures.- - - - - - - - - - ranking = 5keywords = mastocytosis (Clic here for more details about this article) |
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