Temporary Response of Localized Intracranial Mast Cell Sarcoma to Combination Chemotherapy

Guenther, P.; Huebner, Angela; Sobottka, Stephan B.; Neumeister, Volker; Weissbach, G; Todt, Horst; Parwaresch, Reza

doi:10.1097/00043426-200102000-00014

Cited by 38 publications

(29 citation statements)

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“…However, the characteristic longitudinal nuclear grooves of Langerhans cell histiocytosis were not seen in our mast cell sarcoma cases, and essentially all cases of Langerhans cell histiocytosis should show immunohistochemical expression of S100, CD1a, and langerin. The well- Auquit-Auckbur et al 9 Bugalia et al 7 Ma et al 8 Brcic et al 6 Guenther et al, 22 Chott et al 5 Kojima et al 4 Auquit-Auckbur et al 9 Bugalia et al 7 Ma et al 8 Brcic et al 6 Guenther et al, 22 Chott et al 5 Kojima et al 4 14 and should prompt consideration of other diagnoses. Variable KIT expression may occasionally be seen in T cell lymphomas, but the uniformly strong expression seen in two of our three mast cell sarcoma cases would be highly unusual, and expression of CD68 should not be seen.…”

Section: Discussionmentioning

confidence: 99%

Mast cell sarcoma: a rare and potentially under-recognized diagnostic entity with specific therapeutic implications

et al. 2013

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Mast cell sarcoma is a rare, aggressive neoplasm composed of cytologically malignant mast cells presenting as a solitary mass. Previous descriptions of mast cell sarcoma have been limited to single case reports, and the pathologic features of this entity are not well known. Here, we report three new cases of mast cell sarcoma and review previously reported cases. Mast cell sarcoma has a characteristic morphology of medium-sized to large epithelioid cells, including bizarre multinucleated cells, and does not closely resemble either normal mast cells or the spindle cells of systemic mastocytosis. One of our three cases arose in a patient with a remote history of infantile cutaneous mastocytosis, an association also noted in one previous case report. None of our three cases were correctly diagnosed as mast cell neoplasms on initial pathological evaluation, suggesting that this entity may be under-recognized. Molecular testing of mast cell sarcoma has not thus far detected the imatinibresistant KIT D816V mutation, suggesting that recognition of these cases may facilitate specific targeted therapy.

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Section: Discussionmentioning

confidence: 99%

Mast cell sarcoma: a rare and potentially under-recognized diagnostic entity with specific therapeutic implications

et al. 2013

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“…Localized MC proliferations are also extremely rare and include both the extracutaneous mastocytoma (of the lung) and the 'true' MC sarcoma of which less than five published cases exist [43][44][45] . Since cytomorphological atypia of MC sarcoma is high (according to a grade 3 sarcoma), it is impossible to achieve the correct diagnosis without appropriate immunohistochemical stainings.…”

Section: Who Classification For Mastocytosismentioning

confidence: 99%

Mastocytosis: State of the Art

Horny¹,

2007

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Mastocytosis is a neoplastic disease involving mast cells (MC) and their CD34+ progenitors. Symptoms in mastocytosis are caused by biological mediators released from MC and/or the infiltration of neoplastic MC in various organs, the skin and the bone marrow being predominantly involved. A WHO consensus classification for mastocytosis exists, which is widely accepted and includes three major categories: (1) Cutaneous mastocytosis (CM), a benign disease in which MC infiltration is confined to the skin, is preferentially seen in young children and exhibits a marked tendency to regress spontaneously. (2) Systemic mastocytosis (SM) which is commonly diagnosed in adults and includes four major subtypes: (i) indolent SM (ISM, the most common form involving mainly skin and bone marrow); (ii) a unique subcategory termed SM with an associated non-mast cell clonal hematological disease (SM-AHNMD); (iii) aggressive SM usually presenting without skin lesions, and (iv) MC leukemia, probably representing the rarest variant of human leukemias. (3) The extremely rare localized extracutaneous MC neoplasms, either presenting as malignancy (MC sarcoma) or as benign tumor termed extracutaneous mastocytoma. Diagnostic criteria for mastocytosis are available and are widely accepted. SM criteria include one major criterion (multifocal compact tissue infiltration by MC) and four minor criteria: (1) prominent spindling of MC; (2) atypical immunophenotype of MC with coexpression of CD2 and/or CD25 (antigens which have not been found to be expressed on normal/reactive MC); (3) activating (somatic) point mutations of the c-kit proto-oncogene usually involving exon 17, with the imatinib-resistant type D816V being most frequent, and (4) persistently elevated serum tryptase level (>20 ng/ml). To establish the diagnosis of SM, at least one major and one minor criterion, or at least three minor criteria, have to be fulfilled. The natural clinical course of mastocytosis is variable. Most patients, in particular those with CM and ISM, remain in an indolent stage over many years or even decades, while others, in particular those with aggressive SM, SM-AHNMD, or mast cell leukemia, show a progressive course, usually with a fatal outcome.

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“…Localized mast cell tumours comprise the extremely rare mast cell sarcoma with only five published cases so far (Horny et al, 1986;Gu¨nther et al, 2001) and an entity designated as extracutaneous benign mastocytoma which obviously is even more rare (and has not been ever seen in the routine work-up of the author).…”

Section: Classification Of Mastocytosismentioning

confidence: 99%

Mastocytosis and Mast Cells

Horny¹

2010

Encyclopedia of Life Sciences

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Mastocytosis is defined by abnormal accumulation of mast cells in various sites in the body. The clinical features of this chronic, multifaceted disorder originate from mast cell infiltration leading to systemic and local effects induced by numerous pharmacological mediators. Since mastocytosis is an unusual disorder and presents a multiplicity of symptoms, it is an often overlooked consideration in a differential diagnosis that includes not only asthma, chronic diarrhoea, urticaria, syncope, peptic ulceration but also various haematological neoplasms. Diagnosis of mastocytosis can be confirmed only by the pathologist using a combined immunohistochemical and molecular approach. Recognition and subtyping of its rare, life‐threatening high‐grade disease variant (i.e. mast cell leukaemia) includes not only the investigation of tissue samples (usually bone marrow) but also the blood and bone marrow smears. Key concepts: Mastocytosis is an unusual haematopoietic disorder derived from transformed bone marrow progenitor cells. Clinically and histologically, mastocytosis presents an extremely broad spectrum of subvariants ranging from a benign, sometimes even regressive, cutaneous disease to the progressively fatal mast cell leukaemia. Mastocytosis is a histological diagnosis established by the pathologist and cannot be confirmed by clinical findings alone. Most patients with systemic mastocytosis carry the activating point mutation KITD816V in the c‐kit proto‐oncogene. The presence of KITD816V not only explains pathophysiological disease characteristics but also enables targeting therapy using the recently developed tyrosine kinase inhibitors. Although stated in the recent WHO classification book on haematopoietic neoplasms, mastocytosis should not be grouped among myeloproliferative neoplasms. Mastocytosis should be considered as a separate group of disorders among haematological neoplasms. Mastocytosis must be separated from a variety of reactive (i.e. mast cell hyperplasia) and neoplastic (i.e. basophilic leukaemia) states.

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Temporary Response of Localized Intracranial Mast Cell Sarcoma to Combination Chemotherapy

Cited by 38 publications

References 28 publications

Mast cell sarcoma: a rare and potentially under-recognized diagnostic entity with specific therapeutic implications

Mast cell sarcoma: a rare and potentially under-recognized diagnostic entity with specific therapeutic implications

Mastocytosis: State of the Art

Mastocytosis and Mast Cells

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