A Case of Superficial Acral Fibromyxoma of the Index Finger.

Superficial acral fibromyxoma (SAFM) is a rare, slow-growing benign soft tissue tumor that is typically asymptomatic in nature and usually affects the acral regions of the hands and feet. The majority of these lesions are subungual. Excisional biopsy is the primary treatment modality. Despite the distinct clinical and histopathological features, misidentification of this slow-growing tumor persists. This case report contributes to the existing literature by delineating the clinicopathologic features, radiographic and MRI findings, and treatment strategies of SAFM.

Superficial Acral Fibromyxoma on the Thumb-Nail Bed.

Superficial acral fibromyxoma, also known as digital fibromyxoma, is a benign soft tissue tumor. The acral regions, including the palms, soles, fingers, toes, and nail units, are the commonly affected locations. The subungual region of the great toe is the most common site reported in current literature. The tumor is slowly progressive and benign in nature. Histology commonly reveals a fibromyxoid neoplasm with immunoreactivity to CD34 and CD99 markers.1,2,3 We present the case of a 39-year-old female with a nine-year history of repetitive digital trauma presenting with superficial acral fibromyxoma of the thumb-nail bed. Our case is unique due to the tumor location and the patient's prior long history of trauma to the tumor site.

Superficial acral fibromyxoma: insights from case management and comprehensive literature review.

Superficial acral fibromyxoma, also known as digital fibromyxoma, is a slow-growing, benign, solitary soft tissue tumor. First described in 2001 by Fetsch et al., it is a condition that often occurs in middle-aged individuals. However, it has also been reported across a wide range of ages, ranging from 4 to 86 years, with males more commonly reported. The condition often presents as solitary soft tissue swelling over the periungual or subungual. We present the management experience of the rare presentation of this rare tumor and a detailed review of the past literature on this condition. Detailed management of the condition has been described, along with the outcome after 2 years of follow-up and treatment experience. Our detailed analysis shows that 2 years is the shortest duration of follow-up to rule out recurrence. Hence, most of the cases reported earlier had given the false sense of the recurrence rate of the tumor, which could lead to undertreatment of the condition. The purpose of this article is to allow the readers to understand better the tumor's characteristics with bone involvement and the tumor's diagnostic strategies and treatment options.

Superficial Acral Fibromyxoma: A Report of Two Cases with CD68 Expression.

Superficial Acral fibroma (SAF), also known as osteofibroma, is a rare fibromatous tumor primarily involving superficial soft tissues. Clinically, SAF typically manifests as a slow-growing, solitary, well-defined nodule or mass. Although these lesions are generally asymptomatic, some cases may present with associated pain, often linked to a history of trauma. SAF lesions commonly exhibit hemispherical, polypoid, or warty growths, with occasional occurrences of ulceration and bleeding.The majority of SAFs express CD34 and CD99, but in the two cases we report, there was diffuse expression of CD34 and focal positive expression of CD68. CD68 positivity suggests a propensity for tumor cells to metastasize to secondary sites. Notably, previously reported cases of single SAF did not display positive CD68 expression, indicating a potential association with other aggressive tumors. However, the current clinical and pathological manifestations do not clarify the diagnosis of additional malignant tumors. Consequently, regular postoperative monitoring of the patient from the aforementioned two cases is essential to detect the presence of other malignant tumors. The significance of CD68-positive expression in this case lies in its potential association with such tumors.

RB1 loss in the mesenchymal component of onychomatricoma.

BACKGROUND: Onychomatricoma is a nail neoplasm that usually presents as longitudinal nail plate thickening, involving either the partial or whole nail. Histopathologically, it is characterized by deep invaginations of the proliferating nail matrix and proliferation of CD34+ and CD10+ spindle cells with collagenous to myxoid stroma. Onychomatricoma has been considered a fibroepithelial neoplasm. Recently, RB1 loss has been verified using array comparative genomic hybridization. METHODS: This study investigated the RB1 status in onychomatricoma with morphological methods. RESULTS: Six patients with onychomatricoma were included in the study. RB1 status was assessed using immunohistochemical staining and fluorescence in situ hybridization. Immunohistochemical staining showed that all six cases experienced RB1 loss in the mesenchymal component of onychomatricoma but not in the proliferated nail matrix. Fluorescence in situ hybridization in five cases showed a monoallelic deletion of the RB1 locus in the mesenchymal component but not in the proliferated nail matrix. CONCLUSIONS: RB1 loss was observed only in the mesenchymal component of onychomatricoma. Our findings suggest that the proliferated nail matrix in onychomatricoma represents reactive hyperplasia of various degrees secondary to neoplastic mesenchymal proliferation. This indicates that onychomatricoma should be recognized as an RB1-deleted soft tissue neoplasm rather than a fibroepithelial neoplasm.

Onychomatricoma: a clinicopathological, immunohistochemical, and molecular study of 10 cases highlighting recurrent RB1 deletion and the potential diagnostic value of LEF-1.

AIMS: Onychomatricoma (OM), an uncommon benign fibroepithelial neoplasm of the nail unit, is sometimes diagnostically challenging for clinicians and pathologists. OM consistently expresses CD34, but no specific immunohistohemical markers or recurrent genetic alterations have been identified to date. Recent studies have suggested that Wnt signalling is a key molecular characteristic of OM. METHODS AND RESULTS: Ten cases were analysed: four classical OM including two with pleomorphic cells; two superficial acral fibromyxoma-like variants of OM; three micropapilliferum variants of OM including one with pleomorphic cells; and one proliferating variant of OM. Immunohistochemically, the spindle cells were positive with CD34 (n = 10) and CD99 (n = 1), with focal reactivity for CD10 (n = 5). The epithelial component of the tumours expressed immunopositivity for LEF-1. Using array comparative genomic hybridization (aCGH), we demonstrated that all OM, including its variants that were tested (n = 8), harboured a few copy number alterations with losses of whole or part of chromosome 13 including the RB1 gene (n = 8) and chromosome 16 (n = 6). CONCLUSION: We report a recurrent loss of RB1 (13q) as a possible driver molecular event in OM indicating a relationship between OM and other lesions of the spectrum of the so-called '13q/RB1' family of tumours. We did not identify a role for the Wnt/beta-catenin signalling pathway, as has been proposed in a recent study. LEF-1 could be a potential sensitive and specific marker of OM and should be used in the differential diagnosis between OM, superficial acral fibromyxoma, and the CD34-positive fibrosing family of tumours.

Superficial Acral Fibromyxoma: Clinicopathologic Analysis of Five Cases.

Superficial acral fibromyxoma is a relatively rare benign slow-growing soft-tissue tumor, first described by Fetsch's group [Hum Pathol. 2001;32:704-14]. Since then, around three hundred publications have concerned this relatively new entity. The tumor involves peri- and subungual regions of fingers and toes in middle-aged adults with slight male predominance. This acral fibrous tumor is poorly known, and in certain cases, histology results may suggest myxoid dermatofibrosarcoma, which carries a completely different prognosis. In this article, we discuss the clinicopathologic features of this acral fibrous tumor through the report of 5 cases including 1 particular clinical presentation that revealed as a retronychia in a young woman.

Nail Tumors.

This article describes nail tumors and their clinical features, biologic behavior, and treatment. Tumors included are onychopapilloma, onychomatricoma, periungual fibromas/fibrokeratomas, glomus tumors, subungual exostosis, myxoid cysts, and squamous cell carcinoma.

Superficial Acral Fibromyxoma, Appearing as Retronychia: A Rare Clinical Entity.

We report a case of a subungual superficial acral fibromyxoma (SAFM) in a 37-year-old male patient who presented with a persistent chronic proximal paronychia of the big toenail of the right leg. Our clinical diagnosis was retronychia, an often misinterpreted condition, which must be suspected in cases of persistent paronychia, especially in the setting of trauma. The nail plate avulsion revealed a subungual tumor, which was surgically excised. Histopathology in combination with immunohistochemistry revealed features suggestive of SAFM. The follow-up examination of the patient showed no recurrence 9 months after the surgery.

A Case of Superficial Acral Fibromyxoma Showing Erythronychia / 대한피부과학회지

No abstract available.

Aggressive superficial acral fibromyxoma of the great toe: A case report and mini-review of the literature.

Superficial acral fibromyxoma is a very rare soft tissue tumor. Fetsch et al first described the condition in 2001. It often occurs in the fingers and toes and has slow-progressive features. Since being described, a few cases occurring in the great toe have been reported. The present study indicated a case of a 37-year-old male kickboxer with a history of a rapid-growing lump under the nail of his left great toe with bone erosion. The patient was suspected to have a soft tissue tumor under the nail, such as a glomus tumor, as a differential diagnosis. However, a malignant soft tissue tumor as a differential diagnosis could not be ruled out because of the observed bone erosion. The patient underwent surgical excision, and pathologic analysis revealed superficial acral fibromyxoma. Twenty-four months after the treatment, he had no complaints or functional disorder and no recurrence was noted. Although an unusual diagnosis, surgeons should be aware of this tumor, which requires complete surgical excision and follow-up to detect recurrence. To the best of our knowledge, this is the first reported case of superficial acral fibromyxoma with bone erosion in a great toe.

Superficial acral fibromyxoma in the heel with new vascular features on dermoscopy.

Superficial acral fibromyxoma (SAFM) is an uncommon mesenchymal soft tissue tumor with a predilection for the acral extremites and nail bed involvement. SAFM is diagnosed with clinicopathological and immunohistochemical examination. Awareness of this rare tumor is important because of amounts of benign and malignant neoplasms. We report a case of SAFM in a rare localization in the heel with a new finding on dermoscopy.

Superficial acral fibromyxoma: a case report with radiological review.

Superficial acral fibromyxoma is a benign, slow-growing soft-tissue neoplasm that has a predilection for the peripheries. Although considered benign, its risk of recurrence warrants consideration in the radiological differential diagnoses of acral tumors. To our knowledge, there has been minimal literature studying the radiological features of this entity. We present a case report with a discussion focusing on its clinical and radiological findings.

Superficial Acral Fibromyxoma: A Rare Soft Tissue Tumor.

Superficial acral fibromyxoma is a rare clinical entity, first described in 2001. It is a soft tissue tumor with a predilection for the fingers and toes. Since it was described, few cases have been reported. We present a case of an 88-year-old male with a history of a slow-growing lump in his left great toe after local trauma. The patient underwent surgical excision, and pathologic analysis revealed the diagnosis of superficial acral fibromyxoma. Although an unusual diagnosis, surgeons should be aware of this myxoid tumor, which requires complete surgical excision and short-term follow-up to detect recurrence.

CD34 negative superficial acral fibromyxoma: A rare case report.

Superficial acral fibromyxoma (SAF) is a slow growing soft tissue tumor that mainly appears in the acral areas. Here, we report a case of a SAF with distinctive immunophenotype charachteristics. An 18-year-old female was referred to our clinic with the complaint of painless subungual nodule of great toe for a few months. The diagnosis of SAF was made according to histopathology and immunohistochemical (IHC) study, however, the IHC assessment showed positive staining with vimentin, focal reaction with smooth muscle actin, negative reaction with CD34, and positive staining pattern with CD99. These IHC findings are unusual for SAF. This reported case of SAF supports the fact that, although CD34 expression is characteristic for SAF, it is not always present.

Superficial acral fibromyxoma: clinicopathological, immunohistochemical, and molecular study of 11 cases highlighting frequent Rb1 loss/deletions.

Superficial acral fibromyxoma (SAF) is an uncommon benign dermal mesenchymal lesion of adults with predilection for acral sites, in particular the nail region. To date, less than 300 cases have been reported. SAFs consistently express CD34, but other diagnostic markers or specific genetic alterations have not been established yet. We describe 11 SAFs occurring in 7 men and 4 women aged 37 to 86years (median, 48 years). Mean size was 6mm (range, 4-20mm). Affected sites were fingers (n=5), toes (n=3), heel (n=1), calf (n=1), and unspecified digit (n=1). None of 10 patients with available follow-up (2-60months; median, 24months) developed recurrence. Histology showed relatively hypocellular vaguely lobulated nodules composed of bland-looking spindled or stellate fibroblast-like cells arranged into storiform or loose fascicles within a variably myxoid, fibromyxoid, or collagenous vascularized stroma. Immunohistochemistry showed expression of CD34 (9/10) and focal weak reactivity for epithelial membrane antigen (2/11). None of the lesions expressed protein S100 (0/11), MUC4 (0/11), or STAT6 (0/11). Loss of Rb1 immunoexpression was observed in 9 (90%) of 10 cases. All 7 cases with successful RB1 fluorescence in situ hybridization testing showed RB1 gene deletions, which was variably associated with co-loss of the corresponding 13q12 signal (monosomy at the 13q region). To our knowledge, this is the first study investigating the expression status of the tumor suppressor Rb1 in SAF by immunohistochemistry and fluorescence in situ hybridization. Our results showed frequent Rb1 deficiency as a possible driver molecular event in SAF (seen in 90% of cases) indicating relationship of SAF to the RB1-deleted tumor family.

Clinico-pathologic, dermoscopic and ultrasound examination of a rare acral tumour involving the nail - case report and review of the literature.

There is a large spectrum of tumors presenting as nodular lesions that may affect the subungual space. We report the case of a 62-year-old woman presenting with a rapidly growing nodular lesion under the nail of the first left toe. Non-invasive examinations using dermoscopy, ultrasonography and elastography were performed for the preoperative assessment of the lesion. The biopsy of the lesion revealed superficial acral fibromyxoma, a benign tumor with predisposition for acral sites. The patient underwent radical surgery with wide resection margins. This is the first case report of a superficial acral fibromyxoma affecting the subungual region characterized by dermoscopic, ultrasonographic and elastographic features. We also performed a short review of the literature.