A Case of Recurrent Superficial Acral Fibromyxoma

Superficial acral fibromyxoma (SAFM) is a rare myxoid tumor that was first described in 2001. The presence of a very slow growing solitary tender mass in the subungual area is the typical clinical feature at presentation. Histopathologically, SAFM is composed of stellate cells in a myxocollagenous matrix with a poorly circumscribed margin. This tumor is thought to be benign, but its natural course is not fully understood. We describe a 15-year-old patient with recurrent SAFM and discuss the proper treatment and follow up.     

Superficial acral fibromyxoma: report of two cases

Superficial acral fibromyxoma (SAFM) is a rare soft tissue tumor that has recently been delineated as a separate entity. We report 2 cases of SAFM and discuss its pathological features and differential diagnosis. Both patients had lesions on the toe. In 1 patient, the tumor was found after nail extraction, which had been performed for the treatment of onychomycosis, whereas in the other patient the tumor itself was the reason for seeking dermatological assistance. Biopsies from both cases demonstrated similar features. There was a moderately circumscribed, non-encapsulated tumor extending through the whole dermis. The neoplasm was composed of spindle and stellate cells with slight nuclear atypia arranged in a loose storiform, partly fascicular growth pattern. In 1 case, strands of cells with rather wavy nuclei were seen at the periphery of the tumor. Mitotic figures were scarce. The neoplastic cells were embedded in a myxoid stroma with increased numbers of small blood vessels and scattered mast cells. Immunohistochemically, the tumor cells showed weak focal positivity for CD34 and stained negatively for S-100 protein and alpha-smooth muscle actin. In 1 case epithelial membrane antigen (EMA) was negative, whereas in the second case focal expression of EMA by neoplastic cells was seen. Alcian blue staining revealed abundant mucinous material within the stroma. In conclusion, SAFM represents a distinct entity in the spectrum of cutaneous myxoid tumors. The differential diagnosis of SAFM includes various myxoid neoplasms and tumors with a predilection for distal parts of the extremities.     

Superficial acral fibromyxoma: clinical and pathological features

Superficial acral fibromyxoma (SAFM) is a recently recognized myxoid tumor that usually occurs on the fingers and toes of middle-aged adults. We report on the typical case of a 50-year-old woman with a SAFM in the right big toenail that had been slowly growing for more than 10 years. To our knowledge, this case is the first reported case for which clinical pictures are available. Histologically, the lesion was non-encapsulated and was composed of stellate and spindle cells, arranged in a myxoid matrix. No atypia or mitotic figures were found. Neoplastic cells showed positive staining for CD34 and negative staining for epithelial membrane antigen (EMA), actin, desmin, keratins, S100 protein, CD99, and HMB45. Differential diagnosis encompasses benign and malignant myxoid and spindle cells tumors such as myxoid neurofibroma, sclerosing perineurioma, superficial angiomyxoma, and several low-grade myxoid sarcomas.     

Superficial acral fibromyxoma

Superficial acral fibromyxoma (SAF), also known as digital fibromyxoma, is a rare soft tissue tumor with a predilection for acral surfaces. Superficial acral fibromyxoma classically presents as a pink to flesh‐colored nodule located on the subungual or periungual region of the hands or feet. It is typically slow‐growing and asymptomatic, which, coupled with its nonspecific clinical appearance, presents a diagnostic dilemma to the dermatologist. As these features overlap with those of a multitude of differential diagnoses, it is imperative to have a good understanding of the characteristics on which the diagnosis of SAF is based. Superficial acral fibromyxoma was initially described in 2001, since when several case reports and literature reviews have contributed to our current understanding of these tumors. In this article, we will review the history, clinical features, diagnosis, and management of SAF. It is our hope that this systematic approach will help to facilitate the recognition and management of this distinct dermatologic entity.     

Low‐grade fibromyxoid sarcoma of acral sites: Case report and literature review

Low‐grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue sarcoma that usually presents as a deep‐seated tumor in young adults; however, they can occur on superficial sites, mostly documented in pediatric age groups. LGFMS presenting on acral sites is not highly emphasized in the general pathology or dermatopathology literature. The case presented is that of a 30‐year‐old man with a foot mass that was removed 15 years earlier and subsequently recurred as two masses, the first occurring between the third and fourth toes/metatarsal region and the second over the lateral tarsal region. An excisional biopsy showed a relatively circumscribed, bland spindle cell proliferation with hypocellular and hypercellular zones. The cells showed minimal pleomorphism and lacked mitotic activity. Immunohistochemical analysis showed immunoreactivity for MUC4 and break‐apart fluorescence in situ hybridization was positive for FUS rearrangement, confirming the diagnosis of LGFMS. There are multiple spindle cell tumors that occur on acral sites which usually generates a list of differential diagnoses; however, LGFMS is not usually discussed in that anatomic location. Awareness of the occurrence of LGFMS on acral sites is important to avoid misdiagnosis of this deceptively benign‐appearing tumor.     

Constitutive activation of the mitogen-activated protein kinase signaling pathway in acral melanomas

One of the most attractive clinical targets for melanoma is the mitogen-activated protein kinase (MAPK) signaling pathway. In this study, we examined MAPK signaling activation in a total of 28 acral melanoma samples, consisting of 13 primary tumors and 15 metastases. In line with the previous reports, NRAS/BRAF mutations were rare; only one metastatic tumor had an NRAS E61R mutation, and one primary tumor and two metastases harbored BRAF V599E mutations. Western blot analyses, however, revealed phosphorylated extracellular signal-regulated kinase (ERK)1/2 proteins in 11 of 14 (78.5%) of the acral melanoma tumors. Furthermore, fluorescence in situ hybridization analyses revealed the prominent amplification of the cyclin D1 (CCND1) gene, which is an important down-stream effecter of the MAPK pathway, in 5 of 21 (23.8%) tumors examined. Interestingly, two of three tumors that were negative for phosphorylated ERK proteins according to western blot harbored CCND1 amplifications, suggesting that the increased gene dosage of CCND1 may exert effects similar to phosphorylated ERK proteins in cell growth. We conclude that, despite the low frequency of BRAF/NRAS mutations, the MAPK signaling pathway is constitutively activated in the majority of acral melanomas. This provides a rational basis to include acral melanomas into the clinical trials with MAPK inhibitors.     

Amelanotic Acral Melanoma Associated with KIT Mutation and Vitiligo

Amelanotic acral melanoma is rare and difficult to diagnose, both clinically and pathologically. KIT mutations are frequently found in acral melanomas and are considered a risk factor for poor prognosis. The presence of vitiligo in melanoma has been reported, and KIT is thought to be partly responsible for the dysfunction and loss of melanocytes observed in vitiligo. We report a case of amelanotic subungual melanoma with multiple metastases that was associated with KIT mutation and vitiligo. An 85-year-old man presented with a 3-year history of a tender erythematous ulcerated tumor on the left third fingertip and developed hypopigmented patches on the face and trunk. Histopathological examination of the ulcerative tumor showed aggregates of tumor cells that were pleomorphic epithelioid cells. Immunohistochemical staining of the tumor cells was positive for S100, HMB45, and c-Kit. Histopathological findings from the hypopigmented patch on the face were consistent with vitiligo. Mutation analysis showed a KIT mutation in exon 17 (Y823D). The patient had metastasis to the brain, liver, bone, and both lungs. The patient refused chemotherapy, and died 3 months after the first visit.     

Multinucleate cell angiohistiocytoma]

INTRODUCTION: Multinucleate cell angiohistiocytoma is a rare benign vascular proliferation. We report a case. CASE REPORT: A 75-year-old woman presented violaceous papules on the right thigh. Histological examination showed a proliferation of small blood vessels with multinucleated cells in the dermis and confirmed the diagnosis of multinucleate cell angiohistiocytoma. DISCUSSION: Multinucleate cell angiohistiocytoma is a rare benign vascular tumor first described in 1985 often confused with Kaposi's sarcoma. Clinically it is characterized by violaceous papules on acral sites and face in elderly women. Histological examination shows an increased number of blood vessels together with mononucleated and multinucleated histiocyte-like cells in the dermis. Reviewing the literature we describe the main clinical and histopathologic features of this disorder.     

Acral arteriovenous tumor developed within a nevus flammeus in a patient with Sturge-Weber syndrome

The Sturge-Weber syndrome consists of a large facial nevus flammeus in the distribution of the ophthalmologic division of the trigeminal nerve accompanied by ipsilateral leptomeningeal angiomatosis. Usually, when angiomatous nodules develop in a nevus flammeus of a patient with Sturge-Weber syndrome they are pyogenic granulomas. We describe an acral arteriovenous tumor developed within the nevus flammeus of a patient with Sturge-Weber syndrome. To our knowledge, acral arteriovenous tumor has not been previously described in the cutaneous vascular malformation of patients with Sturge-Weber syndrome. The development of acral arteriovenous tumor within the vascular malformation of a nevus flammeus in this patient with Sturge-Weber syndrome probably results from a vascular proliferation secondary to underlying arteriovenous shunts.     

Post traumatic amelanotic subungual melanoma

Subungual melanomas are rare; a delay in the diagnosis is common and is associated with advanced stage. Part of the reason for a delay in presentation to the physician is that patients often attribute the lesion to trauma. Trauma may play a role in the pathogenesis or just draw attention to a skin tumor that may be more susceptible to injury. We report a case of subungual melanoma that was observed in an 86 year old man. The preceding trauma history and misleading clinical appearance delayed the diagnosis slightly. Biopsy of every nodular acral tumor is very important. A direct role of the trauma in the pathogenesis of melanoma remains unclear.     

Acral lentiginous melanoma simulating "clear cell sarcoma of tendon and aponeuroses"

Clear cell sarcoma was closely mimicked in metastatic tumor deposits from two patients with acral lentiginous malignant melanoma. A subcutaneous deposit composed of glycogen-laden spindle cells dominated the presenting clinical picture in one patient. In the other, primary acral melanoma resembled a histiocytic tumor and metastatic tumor simulated clear cell sarcoma. The cases illustrate the pleomorphism that may be encountered in malignant melanoma.     

Zirkumskripte akrale Hypokeratose

Circumscribed acral hypokeratosis is a rare chronic disorder of cornification that occurs predominantly in women. Lesions are solitary and do not respond to any local conservative treatment. They have to be differentiated by biopsy from other non-healing lesions and tumors in acral skin. Clinically lesions appear as sharply circumscribed reddish macules. The histologic hallmark is a circumscribed loss of the entire stratum corneum, which can be best demonstrated at the border of the lesion as a contrast to the perilesional broad stratum corneum typical for acral sites.     

A case of docetaxel-induced erythrodysesthesia

Chemotherapy-induced acral erythema (CIAE) is a rare cutaneous reaction to high-dose chemotherapy, clinically featuring painful erythema on the palms and soles. Docetaxel (Taxotere), an anticancer agent, is known to cause various reactions, including CIAE. We experienced a case of docetaxel-induced acral erythema with facial edematous erythema that coincidentally emerged and regressed with appearance and disappearance of the acral lesions. Docetaxel-induced acral erythema exhibits a widespread distribution and intense sensations of intolerable pain and numbness. Therefore, some authors use the term erythrodysesthesia instead of acral erythema. We speculated that the facial erythema might be part of the spectrum of erythrodysesthesia. Our case was finally diagnosed as decetaxel-induced erythrodysesthesia. Although CIAE is self-limiting, the patients frequently require treatment because of intolerable pain. Reported treatments for CIAE include topical or systemic steroids, elevation of the legs, and application of cold compression to the lesion. In our case, application of a steroid ointment with the occlusive dressing technique (ODT) alleviated the clinical manifestations and was also prophylactic for the erythrodysesthesia.     

Acral,Superficial Spreading Melanoma Arising on Melanocytic Nevus in a Pregnant Woman: A Case Report with Review

We are reporting a case of superficial spreading melanoma (SSM) on left palm of a 37-year-old pregnant housewife. She had a small acquired melanocytic nevus on her left palm since childhood, which changed its consistency and color in the last 4 months. Dermoscopy of the lesion indicated malignant changes. The lesion was managed surgically using split-thickness skin graft. The histopathology report was suggestive of SSM with positive HMB-45 cells. SSM is very rare on the acral site, and it is very difficult to differentiate it from acral lentiginous melanoma. The rarity of the site (acral nonchronic sun damage) with evolution during pregnancy and importance of management approach are reasons for publishing this case.     

Paraneoplastic acrokeratosis: Bazex syndrome

Paraneoplastic acrokeratosis, or Bazex syndrome, is a rare, distinct dermatosis characterized by psoriasiform acral hyperkeratosis. In most cases, it is a specific cutaneous sign of an occult squamous cell carcinoma of the upper aerodigestive tract that has metastasized to cervical lymph nodes. We report the fifth American case of paraneoplastic acrokeratosis. The patient's skin disease was more remarkable for its hyperpigmentation than its hyperkeratosis. Nearly all of the hyperpigmentation resolved, but the nail dystrophy persisted seven months after the tumor had been treated using surgery and radiation.     

Consumption of the epidermis in acral lentiginous melanoma

Background: Consumption of the epidermis (hereafter, consumption), namely thinning of the epidermis with attenuation of basal and suprabasal layers and loss of rete ridges adjacent to collections of melanocytes, has been used to differentiate invasive melanoma from Spitz nevi. Evaluation of 213 invasive melanomas, including only two cases of acral lentiginous melanoma (ALM), showed that the frequency of consumption increases with increasing tumor thickness. Methods: We evaluated consumption in 52 acral melanomas relative to age, gender, Breslow depth, tumor thickness (based on the 2010 American Joint Commission on Cancer guidelines), Clark level, mitoses, ulceration, vertical‐growth phase, regression, tumor‐infiltrating lymphocytes and anatomical site. Results: Consumption was more frequent in ALM with increasing Breslow depth (p = 0.01), and in the presence of ulceration (p = 0.0078); in all cases with ulcer, consumption was found adjacent to the ulceration. There was no statistically significant difference in consumption in nail melanomas in comparison to melanomas of acral skin other than the nail. Conclusions: These results support the hypothesis that epidermal thinning in consumption represents an early phase of ulceration. No statistically significant difference in consumption was found between nail melanomas and melanomas of acral skin other than the nail, probably because of similar tumor thickness in both groups. Ohata C, Nakai C, Kasugai T, Katayama I. Consumption of the epidermis in acral lentiginous melanoma.     

Acral Lentiginous Melanoma Developing during Long-standing Atypical Melanosis: Usefulness of Dermoscopy for Detection of Early Acral Melanoma

Clinical guidelines suggest that suspicious pigmented lesions of the plantar or palmar area require biopsy for early detection of acral melanoma. We present here a case of acral lentiginous melanoma in which various melanocytic atypia was observed at each biopsy site, including focal melanocytic proliferation. We suggest that this atypical melanosis is part of a contiguous phase of invasive tumor growth, which is known as the very early stage of melanoma in situ. In addition, noninvasive dermoscopy has been effective for the early discovery of hidden lesions of acral melanoma.     

Acral arteriovenous tumor.

A unique, superficial, lobulated, benign vascular tumor of the skin, found in the acral areas of adult males, is described. The combination of three different elements--arterial, venous, and transitional vascular channels--make up the predominant histologic features of this tumor. The transition of fibromuscular channels indicates a venous lesion, but the acral location of the lesions and the structure and staining characteristics suggest that the lesion is a hamartomatous proliferation of the Sucquet-Hoyer canal of the true glomus.     

Acral melanoma foot lesions. Part 2: clinical presentation,diagnosis, and management

Acral melanoma (AM) is a rare subtype of cutaneous malignant melanoma found on acral skin, primarily on the soles of the feet. Although rare, it is the most common subtype of melanoma found in patients of African or Asian ethnicity and has a poor prognosis, often because of the more advanced stage of presentation at diagnosis. In the second of this two‐part series, we review the clinical presentation, histopathology, diagnosis and management of AM. Clinically, AM presents as a variegated lesion with blue–black pigment and irregular borders on acral skin. A parallel‐ridge pattern is a very specific dermoscopic finding for AM. The differential diagnoses of AM include acral naevus, pyoderma gangrenosum, pyogenic granuloma, verrucous carcinoma and peripheral neuropathy‐induced foot ulcers. If there is a clinical suspicion of AM, an excisional biopsy should be taken. Once diagnosis is confirmed by histology, surgical excision is the standard treatment. Overall, dermoscopy and histopathology are key tools in the diagnosis of AM. A greater emphasis on melanoma screening and awareness is essential in minority populations to improve survival outcomes in AM.     

Acral erythema with oral methotrexate in a child

Abstract:  Acral erythema is a rare cutaneous reaction that has been associated with various chemotherapy regimens. Most occurrences have been described in adult patients. Recently, methotrexate has been implicated in the development of acral erythema; however, pediatric reports are few. All of the reports in the pediatric and adult literature have described patients receiving moderate to high-dose intravenous methotrexate. Here, we describe an instance of standard-dose oral methotrexate-associated acral erythema in a young girl with acute lymphoblastic leukemia, and review the recent literature as it relates to pediatric patients.