The characteristic imaging findings in computerized tomography (CT) and contrast-enhanced positron emission tomography (PET) in a patient with aggressive systemic mastocytosis (SM) have been described in a case report recently published in Cureus.
The case involved a 54-year-old woman who presented with a history of persisting diarrhea over the last three months and approximately 10 kg weight loss in four months. Laboratory examination was remarkable for anemia, low proteins and elevated tryptase, and an endoscopy revealed multiple ulcers.
SM is a rare condition in which an excessive proliferation of mast cells leads to organ infiltration and a great variety of symptoms varying from fatigue, diarrhea, malabsorption, and flushing, among others.
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SM is classified into several subtypes depending on disease severity. The term B findings refers to findings associated with mast cell proliferation, while C findings refer to findings related to organ infiltration.
“The establishment of the subtype is essential for appropriate risk stratification and management, and radiological examination such as computed tomography (CT) is a valuable non-invasive tool for comprehensively assessing the overall picture of a disease,” the authors wrote.
Aggressive SM is defined by the presence of C findings combined with 20% infiltration of the bone marrow by mast cells; the median survival of patients with aggressive SM is approximately 40 months.
According to the report, CT examination of the patient revealed bone damage, engrossed liver (hepatomegaly), and liquid infiltration (edema) in the large and small intestines. There were engrossed lymph nodes in the thorax as well.
The PET examination showed that the lymph nodes in the thorax, intestines, and spleen did not absorb contrast, which further supported the SM diagnosis. The SM diagnosis was finally confirmed through a bone marrow biopsy.
The patient started treatment with corticosteroids, showing moderate improvement in her symptoms, and is doing well months after diagnosis.
“Given the recent advent of novel therapeutics, a comprehensive understanding of these imaging manifestations is crucial for facilitating timely diagnosis, precise risk stratification of SM, and optimal patient management,” the authors concluded.
