Adipocytic tumors form a significant proportion of all benign and malignant musculoskeletal masses. This chapter focuses on the pathophysiology and clinical and imaging characteristics of the different benign and malignant subtypes presented according to the current WHO classification (Fletcher et al., World health organisation classification of tumors of soft tissue and bone, 4th edn. IARC Press, Lyon, 2013). MRI is emphasized throughout as this imaging technique, in particular, is most useful in this tumor group due to its ability to specifically characterize normal adipose tissue.
A 60-year-old woman affected by rheumatoid arthritis (RA) reported a 12-month history of progressive growth of multiple, painful and symmetrically distributed subcutaneous nodules affecting forearms, thighs and lower back. In the last months the size and the number of nodularities markedly increased, most recently with the appearance of a new lump every other week. These painful swellings had first appeared in the left forearm, with a single lesion progressively increasing in size, but later on similar lesions occurred in the other arm. On examination, multiple, tender and movable subcutaneous nodules with an increased consistency of sizes varying from 0.5–1.8cm were present on both forearms, thighs and lower back. The overlying skin did not show any abnormality but pain was evoked upon palpation with light pressure. The patient described the pain as burning, dull and persistent, slightly reduced during the night, ranging from 5 to 8/10 on a numeric rating scale in the last week. The histologic examination of a nodule of the left forearm showed only adipose tissue. Dercum’s disease (adiposis dolorosa) is a rare disorder of unknown etiology, first described in 1888, characterized by the development of multiple painful nodules of adipose tissue on the trunk and proximal parts of the extremities, most commonly affecting obese postmenopausal women.
Devis Benfaremo, Michele Maria Luchetti, Armando Gabrielli
Clinica Medica, Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Ancona, Italy
Prominent neurological involvement in Dercum disease
(Abstract not provided, article behind paywall)
52 year old obese postmenopausal female presented with complaints of multiple painful lumps over the legs and thighs of 2 years days duration. Clinical examination revealed multiple tender plaques and nodules over both the legs and thighs of different sizes. Biopsy suggestive of lipoma. These features were diagnostic of a rare lipomatosis, dercum’s disease. https://www.omicsgroup.org/journals/dercums-diseaseadiposis-dolorosa-2376-0427-1000252.pdf
K. Beltran and K. L. Herbst
People with lipedema or Dercum’s disease (DD) can have a similar distribution of excess painful nodular subcutaneous adipose tissue (SAT) making them difficult to differentiate.
Case series of 94 patients with DD, 160 with lipedema and 18 with both diagnoses (Lip+DD) from a single clinic in an academic medical center to improve identification and differentiation of these disorders by comparison of clinical findings, prevalence of type 2 diabetes (DM2), hypermobility by the Beighton score, and assessment of a marker of inflammation, Total complement activity (CH50).
Differences between groups were by Student’s t-test with α of 0.05. The Lipedema Group had significantly greater weight, body mass index (BMI), gynoid distributed nodular SAT, and fibrotic and heavy tissue than the DD Group. Hypermobility was significantly higher in the Lipedema (58±0.5%) than DD Group (23±0.4%; P<0.0001). DM2 was significantly greater in the DD (16±0.2%; P=0.0007) than the Lipedema Group (6±0.2%). Average pain by an analog scale was significantly higher in the DD (6±2.5%) than the Lipedema Group (4±2.1%; P<0.0001). Fatigue and swelling were common in both groups. Easy bruising was more common in the Lipedema Group whereas abdominal pain, shortness of breath, fibromyalgia, migraines and lipomas were more prevalent in the DD Group. The percentage of patients with elevated CH50 was significantly positive in both groups.
The significantly lower prevalence of DM2 in people with lipedema compared to DD may be due to the greater amount of gynoid fat known to be protective against metabolic disorders. The high percentage of hypermobility in lipedema patients indicates that it may be a co-morbid condition. The location of fat, high average daily pain, presence of lipomas and co-morbid painful disorders in DD patients may help differentiate from lipedema.
International Journal of Obesity accepted article preview 18 November 2016; doi: 10.1038/ijo.2016.205 http://www.nature.com/ijo/journal/vaop/naam/abs/ijo2016205a.html