Figure 1
Plain radiographic study
Radiographic study revealed punctiform pattern of sclerosis in the spine, ribs, humeral and femoral heads and pelvic bones.
Systemic mastocytosis first suggested in a radiographic study
SectionMusculoskeletal system
Case TypeClinical Cases
AuthorsFilipa Vieira, Diogo Rocha
Connected authors
58 years, male
Clinical History
A 58-year-old man presented asymptomatic and with no relevant background. Laboratory tests only showed a slight increase in trypsin levels (19 ng/mL).
Imaging Findings
The patient presented generalized infiltrative osseous lesions in a radiographic study (Fig. 1).
A CT was performed and revealed the presence of diffuse bone sclerosis interspersed with focal areas of osteopenia and lytic lesions (Fig. 2 and 3).
He underwent a bone marrow biopsy.
A CT was performed and revealed the presence of diffuse bone sclerosis interspersed with focal areas of osteopenia and lytic lesions (Fig. 2 and 3).
He underwent a bone marrow biopsy.
Discussion
Mastocytosis is a heterogeneous group of disorders characterized by the accumulation of MCs in organ systems [1, 2]. The clinical manifestations are caused by the release of biological mediators from MC and/or the destructive infiltration of MC in tissues.
The diagnosis is based on the World Health Organization criteria, which subdivides mastocytosis into seven major categories [3]. Systemic mastocytosis is characterized by involvement of at least one extracutaneous organ, with or without evidence of skin infiltration. Bone marrow is the most commonly involved extracutaneous site and bone marrow aspiration and biopsy are commonly employed to establish the diagnosis [4, 5]. Prognosis is good in patients with indolent forms of the disease.
The diagnosis is based on the World Health Organization criteria, which subdivides mastocytosis into seven major categories [3]. Systemic mastocytosis is characterized by involvement of at least one extracutaneous organ, with or without evidence of skin infiltration. Bone marrow is the most commonly involved extracutaneous site and bone marrow aspiration and biopsy are commonly employed to establish the diagnosis [4, 5]. Prognosis is good in patients with indolent forms of the disease.
Differential Diagnosis List
Mastocytosis
Metastasis
Myeloma
Myelofibrosis
Final Diagnosis
Mastocytosis
References
[1] Robyn J, Metcalfe DD. (2006) Systemic mastocytosis. Adv Immunol 89:169-243 (PMID: 16682275)
[2] Horny HP, Sotlar K, Valent P (2007) Mastocytosis: state of the art. Pathobiology 74(2):121-32 (PMID: 17587883)
[3] Valent P, Sperr WR, Schwartz LB, Horny HP (2004) Diagnosis and classification of mast cell proliferative disorders: delineation from immunologic diseases and non-mast cell hematopoietic neoplasms. J Allergy Clin Immunol 114(1):3-11 (PMID: 15241337)
[4] Avila NA, Ling A, Worobec AS, Mican JM, Metcalfe DD. (1997) Systemic mastocytosis: CT and US features of abdominal manifestations. Radiology 202(2):367-72 (PMID: 9015059)
[5] Valent P, et al. (2007) Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Invest 37(6):435-53 (PMID: 17537151)
Case information
| URL: | https://www.eurorad.org/case/13897 |
| DOI: | 10.1594/EURORAD/CASE.13897 |
| ISSN: | 1563-4086 |
License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Figure 2
CT axial images
CT scan, axial images, showing diffuse bone sclerosis with focal areas of osteopenia (arrows) and lytic lesions (arrowheads).
Figure 3
CT sagital e coronal images
CT scan, sagital and coronal images, showing widespread osteosclerosis in axial skeleton.
Plain radiographic study
Radiographic study revealed punctiform pattern of sclerosis in the spine, ribs, humeral and femoral heads and pelvic bones.
H. Braga.
H. Braga.
CT axial images
CT scan, axial images, showing diffuse bone sclerosis with focal areas of osteopenia (arrows) and lytic lesions (arrowheads).
H. Braga.
H. Braga.
CT sagital e coronal images
CT scan, sagital and coronal images, showing widespread osteosclerosis in axial skeleton.
H.Braga.
H.Braga.