In this report, we present a 53-year-old woman with primary mast cell sarcoma of the thoracic spine vertebrae. Mast cell sarcoma is an aggressive and rare cancer. [ncbi.nlm.nih.gov]

• Pathologist

  Diagnosis of mastocytosis can be confirmed only by the pathologist who should use a combined immunohistochemical and molecular approach. [els.net]

  A diagnosis of ALK-negative anaplastic large cell lymphoma was favored by the submitting pathologist. On referral, additional immunostains ( Figure 3, Table 2 ) confirmed a diagnosis of mast cell sarcoma. [nature.com]

  Most pathologists support the idea of a myogenous origin for ASPS because of the presence of MyoD1, myogen, and desmin in many lesions. [emedicine.medscape.com]

  It is critical that clinicians and pathologists are aware that neoplastic mast cells in patients with SM can infiltrate the mucosa throughout the GI tract and that this infiltration can lead to symptoms and findings that can mimic inflammatory bowel disease [thedoctorsdoctor.com]

• Fishing

  Insect bites, jelly fish stings, snake bites. Infection (bacterial, fungal or viral). [patient.info]

• Cervical Lymphadenopathy

  Two years after surgery, the neoplasm recurred as left cervical lymphadenopathy and an intra-abdominal mass. Despite predonine and radiation therapy, the disease progressed, and the patient died. [ncbi.nlm.nih.gov]

• Aspiration

  Abstract Two novel stem cell factor (SCF) dependent human mast cell lines, designated LAD 1 and 2, were established from bone marrow aspirates from a patient with mast cell sarcoma/leukemia. [ncbi.nlm.nih.gov]

  Mast cells represent less than 20% of the cells in smears of bone marrow aspirate; and 2. No mast cells are identified in their circulating blood and the patient does not fit criteria for aggressive mastocytosis. [web.archive.org]

  The mast cells expressed LCA, KP1, and c-kit in immunohistochemical stains done on the core biopsy and CD15, CD33 and c-kit in flow cytometric immunoanalysis done on the marrow aspirate. [bloodjournal.org]

  Diagnosis: Biopsies Bone marrow biopsy Obtained by bone marrow biopsy and aspiration procedure Stained with Giemsa and tryptase stains Tested with antibodies for CD117, CD2, CD25 and CD34 Looking for clusters of mast cells in groups of 15 or more Looking [mastattack.org]

  Bone marrow biopsy/aspirate. Other tests include: Chromosomal analysis: 20% of patients with systemic mastocytosis have an abnormal karyotype. Molecular testing for KIT D816V mutation is always positive but JAK2 V617F is rarely positive. [patient.info]

• Sneezing

  Mast cells produce histamine, a chemical that can cause itching, sneezing, congestion, swelling, and wheezing. About mastocytosis Mastocytosis describes a group of disorders that are caused by too many mast cells in the body. [cancer.net]

• Abdominal Mass

  Two years after surgery, the neoplasm recurred as left cervical lymphadenopathy and an intra-abdominal mass. Despite predonine and radiation therapy, the disease progressed, and the patient died. [ncbi.nlm.nih.gov]

• Xanthoma

  […] slightly raised papules -Skin is normal to yellowish brown, thickened, and may exhibit discoloration with a peau d’orange appearance -Generalized erythroderma may be present -Onset usually before age 3 years -Yellow and cream-colored papules (resemble xanthomas [slideshare.net]

  The World Health Organization classification of fibrous histiocytomas is as follows: Benign Fibrous histiocytoma Dermatofibroma Deep histiocytoma Juvenile xanthogranuloma Reticulohistiocytoma Xanthoma Intermediate Atypical fibroxanthoma Dermatofibrosarcoma [emedicine.medscape.com]

• Confusion

  Mast cell sarcoma should not be confused with extracutaneous mastocytoma, a rare benign mast cell tumor without destructive growth. [en.wikipedia.org]

  People with a mast cell sarcoma have no skin lesions, and pathology examination of the tumor shows it to be very malignant with an aggressive growth pattern. [2] Mast cell sarcoma should not be confused with extracutaneous mastocytoma, a rare benign mast [ipfs.io]

  Am J Surg Pathol 2000;24:703-709 Abstract quote Mast cell disease (MCD) is a rare proliferation that may be easily confused with other hematopoietic tumors. Several paraffin section antibodies immunoreact with mast cells but most are not specific. [thedoctorsdoctor.com]

  Fibrosarcomas arise from fibroblasts and may be confused with other tumors with a prominent spindle cell component and collagen production; these include malignant fibrous histiocytoma, spindle cell carcinoma, malignant schwannoma, and synovial sarcoma [emedicine.medscape.com]

Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options. J Hematology & Oncology March 2011. [ ] 5. [mastocytosis.ca]

A bone scan, GI workup, and identification of the D816V c-kit mutation can also be helpful in cases where the diagnosis requires confirmation. [merckmanuals.com]

For example, a GI workup is dictated by symptoms of GI involvement, and lymph nodes are biopsied if lymphoma is suspected Narcotic analgesics should be used with care, particularly at high doses Cladribine, a nucleoside analog, does not appear to require [slideshare.net]

[…] as CD2 and CD25 are not unique to atypical mast cells.4,5 This becomes particularly problematic in early stages of the disease with a low disease burden or in cases of MMCAS.3 For this reason, the development of new diagnostic approaches for the SM workup [cytometry.org]

The initial immunohistochemical workup did not include stains for mast cell-specific antigens. A diagnosis of ALK-negative anaplastic large cell lymphoma was favored by the submitting pathologist. [nature.com]

Our results indicate that imatinib is of potential use in the treatment of mast cell sarcoma. [ncbi.nlm.nih.gov]

Treatment There are no standards for treatment, which is unlikely to prolong life. Common treatment options include: Surgical Excision Radiation [knowcancer.com]

[…] of advanced mastocytosis; Treatment strategies of mediator related symptoms in mastocytosis; and Neuro and psychological involvement in Mastocytosis. [books.google.de]

The prognosis of mast cell sarcoma in published literature is very poor in humans. Copyright 2014 Elsevier Inc. All rights reserved. [ncbi.nlm.nih.gov]

Prognosis is extremely poor. People with a mast cell sarcoma have no skin lesions, and pathology examination of the tumor shows it to be very malignant with an aggressive growth pattern. [en.wikipedia.org]

Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]

Etiology in many patients involves an activating mutation (D816V) in the gene coding for the stem cell factor receptor c-kit, which is present on mast cells. [merckmanuals.com]

flushing, urticaria, diarrhea, wheezing) involving / 2 organ systems without identifiable etiology KIT, the transmembrane tyrosine kinase receptor for SCF. [slideshare.net]

Plasmablastic lymphoma: CNS involvement, coexistence of other malignancies, possible viral etiology, and dismal outcome Annals of Hematology 88:351-358, 2009 Ustun C, Jillella AP, Salama ME. [siicsalud.com]

Approximately 80% of chondrosarcomas are primary lesions arising in healthy tissue, with no know etiologic factor. [emedicine.medscape.com]

Articles in this issue include Human Mast Cell Signal Transduction; Mast cell tryptase role in homeostasis and coagulation; Mastocytosis: Current Classification and Diagnostic criteria; Epidemiology, risk factors and prognosis of mastocytosis; Mast cell [books.google.de]

Epidemiology and Mortality Sources Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds): WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition) IARC: Lyon 2017 Section: Mastocytosis Pages: 69 [seer.cancer.gov]

A mutation of the oncogene C-KIT D816V is found in many (but not all) cases. [ 9 ] Epidemiology [ 10 ] This is a rare and heterogeneous group of disorders, of which urticaria pigmentosa is the most common manifestation. [patient.info]

However, the pathophysiological basis of such a coevolution has not been clarified. [thedoctorsdoctor.com]

PREVENTION No preventative measures exist. Signs & Symptoms Patients do not have skin lesion as is common in similar diseases, nor do they fit the criteria of systemic mastocytosis. [knowcancer.com]

[…] under supervision. [ 23 ] Bone pain Oral sodium cromoglicate may help. [ 21 ] Osteoporosis prevention/treatment - calcium, vitamin D, and bisphosphonates. [ 11 ] Drugs to avoid [ 24 ] Beta-blockers are contra-indicated in patients with systemic mastocytosis [patient.info]

There is no sure way to prevent UP. The inherited form is very rare, and even when the child has the abnormal gene, they may not ever develop UP. However, you can prevent the disorder from getting worse. [healthline.com]

NIH: National Cancer Institute Cancer (Medical Encyclopedia) Cancer and lymph nodes (Medical Encyclopedia) Cancer treatment -- early menopause (Medical Encyclopedia) Cancer treatment: preventing infection (Medical Encyclopedia) Cancer treatments (Medical [icdlist.com]

Cromolyn 200 mg orally 4 times a day (100 mg 4 times a day for children 2 to 12 years; not to exceed 40 mg/kg/day) may help by preventing mast cell degranulation. Ketotifen 2 to 4 mg orally twice a day is inconsistently effective. [merckmanuals.com]