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Flushing, Vomiting, and Abdominal Pain Following Abdominal Ultrasonography

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 41-year-old man presented with a 7-month history of episodes of back and abdominal pain associated with nausea, vomiting, and flushing. Immediately following workup with noncontrast abdominal ultrasonography, he experienced intense flushing and vomiting. Subsequent contrast-enhanced magnetic resonance imaging identified a 5-cm thoracic paraspinal mass (Figure, A). Computed tomography–guided biopsy of the paraspinal mass revealed a dense cellular infiltrate on histopathology composed of cells with round nuclei and abundant pale cytoplasm (Figure, B).

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B. Mastocytoma

Spine magnetic resonance imaging revealed an unresectable paraspinal mass with neural foramen encroachment and encasement of the aorta (Figure, A). Biopsy findings revealed a monomorphic infiltrate of medium-sized round cells with abundant amphophilic hypogranular cytoplasm (Figure, B), positive for CD117 (also known as KIT), tryptase (serine protease stored in mast cells), and CD2. Bone marrow biopsy demonstrated aggregates of more than 15 mast cells, negative for point variation (formerly mutation) of codon 816 on KIT (KIT D816V), with no evidence of an associated hematologic neoplasm, supporting a diagnosis of systemic mastocytosis (SM).

Mastocytomas typically present as cutaneous lesions in children and are characterized by a clonal proliferation of morphologically unremarkable mast cells that undergo spontaneous resolution at puberty. Mastocytomas in adults are unusual; extracutaneous presentations are exceedingly rare1,2 and should prompt thorough evaluation for SM.

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Article Information

Corresponding Author: Nima Nabavizadeh, MD, Department of Radiation Medicine, Oregon Health & Science University, 3181 SW Sam Jackson Park Rd, Mail Code KPV4, Portland, OR 97239-3098 (nabaviza@ohsu.edu).

Published Online: July 30, 2020. doi:10.1001/jamaoncol.2020.0787

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Janjan  NA , Conway  P , Lundberg  J , Derfus  G .  Radiation therapy in a case of systemic mastocytosis: evaluation of histamine levels and mucosal effects.   Am J Clin Oncol. 1992;15(4):337-339. doi:10.1097/00000421-199208000-00013PubMedGoogle ScholarCrossref
2.
Cohen  PR .  Solitary mastocytoma presenting in an adult: report and literature review of adult-onset solitary cutaneous mastocytoma with recommendations for evaluation and treatment.   Dermatol Pract Concept. 2016;6(3):31-38. doi:10.5826/dpc.0603a07PubMedGoogle ScholarCrossref
3.
Pardanani  A .  Systemic mastocytosis in adults: 2017 update on diagnosis, risk stratification and management.   Am J Hematol. 2016;91(11):1146-1159. doi:10.1002/ajh.24553PubMedGoogle ScholarCrossref
4.
Lim  KH , Tefferi  A , Lasho  TL ,  et al.  Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors.   Blood. 2009;113(23):5727-5736. doi:10.1182/blood-2009-02-205237PubMedGoogle ScholarCrossref
5.
Dyson  M , Luke  DA .  Induction of mast cell degranulation in skin by ultrasound.   IEEE Trans Ultrason Ferroelectr Freq Control. 1986;33(2):194-201. doi:10.1109/T-UFFC.1986.26814PubMedGoogle ScholarCrossref
6.
Fyfe  MC , Chahl  LA .  Mast cell degranulation and increased vascular permeability induced by ‘therapeutic’ ultrasound in the rat ankle joint.   Br J Exp Pathol. 1984;65(6):671-676.PubMedGoogle Scholar
7.
Lim  KH , Pardanani  A , Butterfield  JH , Li  CY , Tefferi  A .  Cytoreductive therapy in 108 adults with systemic mastocytosis: outcome analysis and response prediction during treatment with interferon-alpha, hydroxyurea, imatinib mesylate or 2-chlorodeoxyadenosine.   Am J Hematol. 2009;84(12):790-794. doi:10.1002/ajh.21561PubMedGoogle ScholarCrossref
8.
Gotlib  J , Kluin-Nelemans  HC , George  TI ,  et al.  Efficacy and safety of midostaurin in advanced systemic mastocytosis.   N Engl J Med. 2016;374(26):2530-2541. doi:10.1056/NEJMoa1513098PubMedGoogle ScholarCrossref
9.
Vega-Ruiz  A , Cortes  JE , Sever  M ,  et al.  Phase II study of imatinib mesylate as therapy for patients with systemic mastocytosis.   Leuk Res. 2009;33(11):1481-1484. doi:10.1016/j.leukres.2008.12.020PubMedGoogle ScholarCrossref
10.
Ustun  C , Reiter  A , Scott  BL ,  et al.  Hematopoietic stem-cell transplantation for advanced systemic mastocytosis.   J Clin Oncol. 2014;32(29):3264-3274. doi:10.1200/JCO.2014.55.2018PubMedGoogle ScholarCrossref
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