Polyfibromatosis syndrome is a rare disease entity that is characterized by various clinical features such as palmar, plantar, and penile fibromatoses, keloid formations of the skin, and erosive arthropathy. Its precise pathophysiology or etiology remains unclear. In addition to distinctive diverse skin manifestations, patients with polyfibromatosis have been previously reported to show erosive arthropathy with significant limitation of movement at affected joints. However, the presence of erosive polyarthropathy in polyfibromatosis has not emphasized in previous cases. Here, we report a case of polyfibromatosis syndrome combined with painless massive structural destruction of hand and foot joints, and review the characteristics of erosive arthropathy in previous cases.
Adult ; Arthrography ; Diagnosis, Differential ; Fibroma/*diagnosis/pathology/radiography ; Foot Joints/pathology/radiography ; Hand Joints/pathology/*radiography ; Humans ; Magnetic Resonance Imaging ; Male ; Metacarpophalangeal Joint/pathology/radiography ; Osteolysis/*diagnosis/etiology

Desmoplastic fibroma of bone is a rare locally aggressive, but non-metastatic tumor. In this case report, we present a desmoplastic fibroma in an unusual location, the clavicle. Desmoplastic fibroma involving the clavicle is extremely rare, with only 2 reported cases before 1985. We report the imaging findings of a desmoplastic fibroma of the clavicle with a review of the relevant literature.
Adult ; Bone Neoplasms/*diagnosis/pathology/radiography ; *Clavicle/pathology/radiography ; Fibroma, Desmoplastic/*diagnosis/pathology/radiography ; Humans ; Magnetic Resonance Imaging ; Male ; Tomography, X-Ray Computed

Calcifying aponeurotic fibroma is a rare, benign fibroblastic tumor. The lesion has a propensity for local invasion and a high recurrent rate. Therefore, accurate preoperative diagnosis and complete excision are important to prevent the recurrence of the tumor after surgical removal. However, radiographic and magnetic resonance imaging findings of calcifying aponeurotic fibroma have been extremely rarely described in the radiology literature. Thus, we report a rare case of calcifying aponeurotic fibroma affecting the dorsal wrist in a 67-year-old man, describe radiographic and MR findings, and discuss the differential diagnosis of the tumor.
Aged ; Calcinosis/*diagnosis/pathology/radiography ; Diagnosis, Differential ; Fibroma/*diagnosis/pathology/radiography ; Humans ; Magnetic Resonance Imaging/methods ; Male ; Neoplasm Recurrence, Local/diagnosis/radiography ; Soft Tissue Neoplasms/diagnosis/pathology/radiography ; *Wrist/pathology/radiography

Bone Neoplasms ; diagnosis ; diagnostic imaging ; pathology ; Cafe-au-Lait Spots ; diagnosis ; pathology ; Child ; Diagnosis, Differential ; Female ; Fibroma ; diagnosis ; diagnostic imaging ; pathology ; Humans ; Neurofibromatosis 1 ; diagnosis ; Radiography ; Syndrome

OBJECTIVETo study the clinicopathologic characteristics, immunophenotype and differential diagnosis of superficial acral fibromyxoma (SAF).

METHODSThe clinical, pathologic and immunohistochemical features of a case of SAF occurring in left middle finger was studied, with review of literature.

RESULTSThe patient was a 62-year-old male who presented with a solitary painful nodule located in the distal aspect of his left middle finger. The nodule lied close to the nail bed and deep to the underlying periosteum. Grossly, the tumor was poorly circumscribed, measured 2 cm in greatest dimension and had a greyish-white cut surface and rubbery consistency. On low-power examination, the tumor was centred in the dermis and displayed a vague lobular pattern. The tumor cells were spindled to stellate in shape and associated with myxoid matrix. Focal fascicular or loose storiform patterns were also noted. A delicate vascular network was identified in the myxoid stroma. Mast cells were readily observed. On high-power examination, the tumor cells were relatively bland-looking and showed at most a mild degree of nuclear atypia. Mitotic figures were rare and coagulative tumor necrosis was absent. Immunohistochemical study showed that the tumor cells were positive for vimentin, CD34 and CD99. Focal staining for CD10 was also demonstrated. Other immunomarkers including actins, desmin and epithelial membrane antigen were negative.

CONCLUSIONSSAF is a distinctive soft tissue tumor occurring mainly in the digits of adults. Awareness of this entity is helpful in distinguishing SAF from other myxoid soft tissue tumors occurring there. Complete excision with clear resection margins is the mainstay of treatment.

12E7 Antigen ; Antigens, CD ; metabolism ; Antigens, CD34 ; metabolism ; Cell Adhesion Molecules ; metabolism ; Dermatofibrosarcoma ; metabolism ; pathology ; Diagnosis, Differential ; Fibroma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Fingers ; pathology ; Follow-Up Studies ; Ganglion Cysts ; metabolism ; pathology ; Humans ; Male ; Middle Aged ; Nerve Sheath Neoplasms ; metabolism ; pathology ; Radiography ; Skin Neoplasms ; metabolism ; pathology ; Soft Tissue Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Vimentin ; metabolism