Atypical decubital fibroplasia. A distinctive fibroblastic pseudotumor occurring in debilitated patients.
We report 28 cases of atypical decubital fibroplasia, a distinctive pseudosarcomatous fibroblastic proliferation occurring primarily but not exclusively in physically debilitated or immobilized patients. The subjects included 16 women and 12 men ranging in age from 15 to 95 years. Peak incidence was in the 8th and 9th decades of life. Anatomic locations included the soft tissues overlying the shoulder (eight cases), posterior chest wall (five cases), sacrum (five cases), greater trochanter (four cases), buttock (two cases), thigh (two cases), and arm (two cases). Symptoms were due to a painless mass of 3 weeks' to 6 months' duration. Most lesions were ill-defined, focally myxoid masses that ranged from 1 to 8 cm. Histologically, they were situated in the deep subcutis and secondarily involved adjacent skeletal muscle (11 cases) and tendon (three cases). Extensive epidermal ulceration was typically absent. Microscopically, the lesions had a lobular configuration. They were characterized by zones of fibrinoid necrosis and a prominent myxoid stroma rimmed by ingrowing, ectatic, thin-walled vascular channels. All cases contained atypical, enlarged, degenerated fibroblasts with abundant basophilic cytoplasm, large hyperchromatic, smudged nuclei, and prominent nucleoli; these features resulted in a superficial resemblance to proliferative fasciitis. The enlarged, atypical fibroblasts stained diffusely and strongly for vimentin (15 of 15 cases) and focally for muscle-specific actin (10 of 15 cases), keratin (one of 15 cases), CD68 (10 of 15 cases), and CD34 (five of nine cases) antigens; none of the cases stained for desmin. A malignant diagnosis was considered in 43% of cases. Follow-up in 21 patients ranged from 2 to 78 months (median, 12 months). Two lesions recurred once, one recurred twice, and none metastasized; no deaths were attributable to the lesions. The clinical, histologic, and immunohistochemical features of atypical decubital fibroplasia indicate it is a unique type of pressure sore displaying degenerative and regenerative features distinct from decubitus ulcer. Its recognition by pathologists and clinicians in elderly and debilitated patients is important to avoid misdiagnosis as a sarcoma and to prevent or minimize the occurrence of decubital fibroplasia in progressively aging patient populations.
Montgomery
E A
EA
Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, D.C. 20306-6000.
Meis
J M
JM
Mitchell
M S
MS
Enzinger
F M
FM
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D003937:Diagnosis, Differential; D005208:Fasciitis / Q000175:diagnosis* / Q000473:pathology; D005260:Female; D005350:Fibroma / Q000175:diagnosis* / Q000473:pathology; D016330:Frail Elderly; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D003668:Pressure Ulcer / Q000175:diagnosis*