Systemic Mastocytosis and Mastocytosis-Like Syndrome: Radiologic Features of Gastrointestinal Manifestations

Ac, Johnson; Johnson, S. Harris; Pd, Lester; Halter, Susan A.; James, Ebor Jacob

doi:10.1097/00007611-198806000-00011

Cited by 10 publications

(5 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Such nodularity has been documented by others, both radiologically and endoscopically. 2,[6][7][8]18,19,23,[34][35][36][37][38] Other nonspecific findings seen in this series and reported in the literature include erosions, loss of mucosal folds and friability. A substantial proportion of cases shows no obvious endoscopic or radiological abnormality.…”

Section: Discussionmentioning

confidence: 95%

Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

et al. 2008

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 95%

Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

et al. 2008

View full text Add to dashboard Cite

“…Nearly half of the SM had skin lesions and skin symptoms were more common in indolent SM than ASM [1]. Although 61% of those SM had gastrointestinal manifestations, few had mast cells aggregates under endoscopic biopsies [5]. This patient was diagnosed by close pathological examinations before skin symptoms were noticed.…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Aggressive Systemic Mastocytosis Involving the Gastrointestinal System and Review of Literature

Wang

Zheng

Niu

et al. 2021

Preprint

View full text Add to dashboard Cite

Background Systemic mastocytosis is a rare disease and most patients have pigmented urticarial skin lesions. It can be easily missed and misdiagnosed in small biopsies, especially in those patients with nonspecific clinical complaints or untypical skin lesions. Case presentation We report a case of 69-year-old man who have presented with 2-year of diarrhea, progressive weight loss of 20kg and abdominal distention for 3 months. Ultrasound and abdominal CT scan showed massive effusions in abdominal and pelvic cavity. Colonoscopy was performed and showed intensive mucosal proliferations forming polypoid appearances. Microscopically, monotonously uniform, small, round tumor cells with slightly rich cytoplasm were concentrated between the residual glands in the colonoscopic biopsy. The tumor cells showed positive expression of CD117 and S-100, and low Ki-67 proliferation index of 2%, while Trypsin, CK, CD68, CD1a, Langerin, Syn, CgA, CD56, SATB2, CD20, CD3, α-inhibin and SMA were all negative. KIT D816V mutation was detected as well. Liver biopsy showed that CD117 positive cells were more than 15 cells in aggregates around the hepatic portal area and less than 15% mast cells were found in bone marrow smears. No multiple or diffuse pattern of mast cells infiltration was seen by repeated skin biopsies of skin lesions. With all these considered, the diagnosis of aggressive systemic mastocytosis was made. Conclusions The diagnosis of aggressive systemic mastocytosis is challenging for untypical clinical manifestations and subtle or inconspicuous lesions, especially in endoscopic biopsies, which requires awareness and a close teamwork of pathologists and clinicians.

show abstract

“…In addition, the involvement of colonic damage in the pathogenesis of diarrhea in SM is not clear. The observed colonic lesions include small nodules of the mucosa, 41 mucosal edema, and urticarian lesions, 34,42 and polypoid lesions. 15,43,44 Histologically, these lesions often consist of an inflammatory infiltrate with polycyclic eosinophils and mast cells, or mast cells alone.…”

Section: Colon and Rectummentioning

confidence: 99%

Gastrointestinal involvement and manifestations in systemic mastocytosis

Sokol¹,

Georgin‐Lavialle²,

Grandpeix-Guyodo³

et al. 2010

Inflammatory Bowel Diseases

View full text Add to dashboard Cite

Mastocytosis is a rare and heterogeneous disease characterized by various biological and clinical features with different prognosis and treatments. The disease is usually divided into 2 categories: a pure cutaneous and a systemic disease. Clinical features can be related to mast cells' mediators release or to pathological mast cells infiltration. The diagnosis of mastocytosis is based on clinical, biological, histological, and molecular international criteria. Among all manifestations of the disease, gastrointestinal (GI) symptoms are common and can significantly impair the quality of life. The aim of this article is to review the data regarding GI involvement in mastocytosis. Articles dealing with clinical, pathophysiological, and therapeutic aspects of mastocytosis GI tract involvement were searched for using PubMed. GI manifestations in mastocytosis are reviewed. Pathogenesis of GI symptoms in systemic mastocytosis and their treatment are critically discussed. The most frequent GI symptoms are abdominal pain, diarrhea, nausea, and vomiting. GI lesions may involve all the digestive tract, from the esophagus to the rectum. The histological diagnosis of GI involvement is difficult. The treatment of GI symptoms aims to prevent and limit mast cells degranulation and/or its consequences and more rarely to control tumoral mast cells infiltration. The high prevalence of GI symptoms in mastocytosis and their important functional impact deserves better characterization and treatment in order to improve patients' quality of life. Diagnosis of mastocytosis GI manifestations should be evoked in the case of unexplained severe GI disorders.

show abstract

Systemic Mastocytosis and Mastocytosis-Like Syndrome: Radiologic Features of Gastrointestinal Manifestations

Cited by 10 publications

References 0 publications

Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

A Rare Case of Aggressive Systemic Mastocytosis Involving the Gastrointestinal System and Review of Literature

Gastrointestinal involvement and manifestations in systemic mastocytosis

Contact Info

Product

Resources

About