Superficial acral fibromyxoma saf is a myxoid tumor that was recently. Superficial acral fibromyxoma archives of pathology. Superficial acral fibromyxoma saf is a rare tumor of soft tissues, with slow growth and located in the subungual or periungual region of the hands and feet 17. Forces institute of pathologys nonmelanocytic tumors of the skin atlas of tumor pathology. Conjunctival myxomas are thought to originate in tenons capsule and can masquerade as conjunctival lymphoma, lymphangioma, ocular surface squamous neoplasia ossn, or amelanotic melanoma. Superficial acral fibromyxoma is a benign and rare tumor of the soft tissues. Histopathologically, safm characteristically presents as a wellcircumscribed. Included in the acral fibrokeratoma group 112 are lesions reported as acquired digital fibrokeratoma, 1116 acquired periungual fibrokeratoma, garlic clove fibroma, 117 and the subungual and periungual fibromas of tuberous sclerosis. It is a benign slowgrowing soft tissue lesion occurring in males. Histopathologically, safm is composed of stellate cells in a myxocollagenous matrix with a poorly circumscribed margin. We report a case of saf diagnosed by fine needle aspiration cytology and confirmed by histopathology.
We present the case of a 35yearold male with a safm located in the toe, with involvement of the nail and erosion of the distal phalanx. Histopathology images of superficial acral fibromyxoma by. We have studied 14 safm nine male and five female, mean age 52 years derived from consultation practice. They are small isolated lesions with a predilection for the fingers, toes, and nails histology of superficial acral fibromyxoma. Superficial angiomyxomas have a lobular appearance and are purely myxoid, lacking the collagenous component of the stroma of acral fibromyxoma. The presence of a very slow growing solitary tender mass in the subungual area is the typical clinical feature at presentation. Superficial acral fibromyxoma pathology dermnet nz. Superficial acral fibromyxoma saf is an uncommon tumor of the superficial soft tissues of acral extremities in middleaged adults, firstly described in a series of 37 cases by fetsch et al. Superficial acral fibromyxoma saf is a rare, distinctive benign soft tissue lesion that often involves the fingers and toes, with the great toe being the most frequently affected site. The vast majority occur on the fingers or toes, usually adjacent to the nail, hence the other name. Associate clinical professor david geffen school of medicine at ucla.
Superficial acral digital fibromyxoma stanford university. In superficial acral fibromyxoma, sections show a paucicellular fibromyxoid lesion arising in the dermis of acral skin figure 1. Superficial acral fibromyxoma is a relatively new entity, most likely representing a fibrohistiocytic neoplasm. Superficial acral fibromyxoma is a benign, slowgrowing softtissue neoplasm that has a predilection for the peripheries. Superficial acral fibromyxoma saf is a rare, slow growing soft tissue tumor. The differential diagnosis of acral fibromyxoma includes superficial angiomyxoma, perineurioma, and myxoid neurofibroma. An 18yearold female was referred to our clinic with the complaint of painless subungual nodule of great toe for a. Superficial acral fibromyxoma on the second toe europe. Superficial acral fibromyxoma saf is a rare lesion initially described by fetsch as a tumour with histological and immunohistochemical features located at acral sites. Superficial acral fibromyxoma and other slowgrowing. The mean age at diagnosis of safm is 43 years, with a range of 14 to 72 years. Patients generally look for late medical help as it is generally painless. Superficial angiomyxoma shows a predilection for head and neck and trunk rather than acral sites and shows a lobular growth pattern.
Pedal presentation of superficial acral fibromyxoma. Skin nonmelanocytic tumors acquired digital fibrokeratoma. Superficial acral fibromyxoma is a single softtissue tumor that should enter the differential diagnosis of periungual and subungual acral lesions. All patients presented with a solitary and slowly enlarging mass.
Superficial acral fibromyxoma saf is a myxoid tumor described in 2001 by fetsch et al, 1 which affects preferentially the subungual or periungual regions. Superficial acral fibromyxoma saf was first reported by fetsch et al. Primary localized cutaneous nodular amyloidosis on a toe. These lesions are painless, slow growing, present 3 months to 30 years before resection, and involve the subungual or periungual region of the fingers and toes. There have been quite a few reports of cases with this myxoid tumor. Superficial examples of lgfms very rarely arise in the digits,17,18 have fig. The american journal of surgical pathology, 366, 789798. Superficial acral fibromyxoma saf is a slowly growing soft tissue tumor that tends to appear in the acral sites.
The study group included 70 male and 54 female patients 1. Pdf superficial acral fibromyxoma involving the nails apparatus. Myxoid tumors often have overlapping features and may show significant heterogeneity from area to area within a given specimen and from individual case to case. Pedal presentation of superficial acral fibromyxoma a case report robin lenz, dpm rene kafka, dpmf kevin jules, dpalj bradley w.
Acral fibrokeratoma af is a solitary, benign, round, firm lesion of the soft tissue. The initial pathology report described a myxoid tumour, most probably a neurofibroma. The cause of tumor formation is unknown and no particular risk factors have been identified. Myxoid or mucinous background change is a common finding in a wide variety of cutaneous tumors and other diseases. Superficial acral fibromyxoma journal of plastic, reconstructive. Superficial acral fibromyxoma dermatofibrosarcoma protuberans. Superficial acral fibromyxoma safm is a rare soft tissue tumor most often located in the ungual region of the fingers and toes. David weedon ao md frcpa fcaphon, in weedons skin pathology third edition, 2010. Superficial acral fibromyxoma is a rare soft tissue tumour that was first described by fetsch et al.
Superficial acral fibromyxoma safm is a rare, slowgrowing, solitary, soft tissue tumor, which was initially described by fetsch et al1 in 2001. However, the heel, palm and ankle can also be affected. Differential diagnostic possibilities as acral manifestations of soft tissue tumors including superficial acral fibromyxoma, inclusion body fibromatosis, and acral fibrokeratoma were considered. This tumor was first described in 2001, and since then very few cases have been reported. More studies are needed to better understand this pathology, which was first described in 2001. As prognosis and treatment vary considerably between many of these entities, accurate diagnosis is essential. Usually has some classic nonmyxoid areas with tight storiform. A local ultrasound examination revealed a hypoechoic tumor measuring 2.
Superficial acral fibromyxoma is a rare, slowgrowing soft tissue tumor, which is commonly located in the periungual and subungual regions of the fingers and toes in adults. Myxoma is a rare, benign stromal tumor of mesenchymal origin often confused with other conjunctival stromal tumors. Superficial acral fibromyxoma is a benign and slowgrowing solitary softtissue neoplasm. It usually manifests itself through a painless mass of slow growth that affects mainly males in the fifth decade of life. Here, we report a case of a saf with distinctive immunophenotype charachteristics. Cytological diagnosis of superficial acral fibromyxoma.
Superficial angiomyxomas occur mostly on the trunk and head and neck region. Superficial acral fibromyxoma saf is a myxoid tumor that was recently described by fetsch et al. It is generally manifested in the form of a slowgrowing painless mass that mainly affects male adults. Superficial acral fibromyxoma are benign fibromyxoid lesions. Superficial acral fibromyxoma safm is a rare fibromyxoid mesenchymal tumor with a predilection for the distal extremities and frequent nail bed involvement. Superficial acral fibromyxoma involving the nails apparatus. Acral fibrokeratoma journal of the american podiatric. Superficial acral fibromyxoma saf is a slow growing soft tissue tumor that mainly appears in the acral areas. The lesion was surgically removed for further histopathological evaluation. References this dermal and subcutaneous growths article is a stub.
The lesion was surgically excised and histopathology revealed features suggestive of superficial fibromyxoma with positive. It consisted of myxoid stroma with spindle and stellate cells and scattered. This report details the histologic, immunophenotypic, and clinical findings in 124 cases of digital fibromyxoma. Acral fibrokeratoma an overview sciencedirect topics. Pdf superficial acral fibromyxoma is a rare tumor of soft tissues. This tumor is thought to be benign, but its natural course is not fully understood. Differential diagnosis of myxoid dfsp is diverse and the lesion may be easily confused with a few myxoid neoplasms, such as myxoid neurofibroma, superficial angiomyxoma, myxoid liposarcoma, and lowgrade myxofibrosarcoma. Pdf on jan 1, 2015, funda tamer and others published superficial acral. Superficial acral fibromyxoma typically arises as a solitary, slowgrowing nodule on a toe or finger, with the great toe being the most commonly affected site.
Superficial acral digital fibromyxoma is composed of bland spindled or stellate cells arranged in a loose fascicular growth pattern in a myxoid background with variable amounts of collagen. Pathology of superficial acral fibromyxoma dr sampurna. Acral fibromyxoma afm is an uncommon, benign tumor of the soft tissues, mostly present around or below the fingernail or toenail. To date, however, few cases of saf have been described in the literature. A superficial acral fibromyxoma is a type of myxoma and is a rare cutaneous condition characterized by a mesenchymal neoplasm that typically occurs on the digits of middleaged adults 1817. Superficial acral fibromyxoma safm is a distinctive benign soft tissue tumour with a predilection for the hands and feet, described by fetsch et al.
It is not listed in the current world health organizations pathology and. Superficial acral fibromyxoma of the toe, with erosion of. The cells express cd34, cd99, and, frequently, epithelial membrane antigen. This tumor was first described in 2001, and since then very few.