Dermatofibrosarcoma protuberans association with nuchal-type fibroma

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a hypercellular, storiform, CD34-positive low-grade sarcoma with honeycomb entrapment of fat, which typically involves the trunk and extremities. Nuchal-type fibroma (NTF ) is a paucicellular, CD34-positive fibrous tumor with fat entrapment, which may occur in both nuchal and extranuchal locations and in association with Gardner syndrome. METHODS: We report the association of DFSP with NTF in a 43-year-old male with no personal or family history of Gardner syndrome. RESULTS: The patient had a past history of a DFSP removed from his back, which recurred 2 years later and was re-excised. Additionally, the patient had a typical NTF, in the posterior neck, removed at the same time. Histopathologic examination of the recurrent back lesion demonstrated a composite lesion with typical appearances of DFSP, centrally, blending into an NTF-like appearance, peripherally. Both components expressed CD34 and CD99, and lacked elastin. A review of the microscopic slides of the patient's previously excised DFSP revealed an identical lesion with surrounding NTF-like areas. CONCLUSION: While an association between NTF and fibromatosis has recently been reported, this is to our knowledge the first report of an association between NTF and DFSP. The morphologic findings suggest that there may be a continuum between these two CD34-positive lesions that have a tendency to infiltrate adipose tissue and recur.     

Nuchal-type Fibroma of the Coccyx

Nuchal-type fibroma is a recently described, rare, tumor-like process of fibrous tissue occurring chiefly in the dorsocervical area. Nuchal-type fibromas in extranuchal locations are morphologically indistinguishable from lesions involving the nuchal region. Histopathologic findings of nuchal-type fibroma are poorly demarcated, dense collagen bundles with sparsely scattered fibroblasts, some interspersed adipose tissue, and entrapped nerve fibers. We report a patient with a mass on the coccyx area, and these were histopathologically consistent with nuchal-type fibroma. This tumor was surgically excised, and no recurrence had been noted during the two year follow-up period.     

Sudden Onset Vision Loss in an 8‐year‐old Female with McCune–Albright Syndrome

Abstract: We describe a case of an 8‐year‐old girl with large irregular café au lait macules on the right cheek and right lower extremity presenting with sudden onset vision loss and found to have polyostotic fibrous dysplasia on imaging. The classic triad of McCune–Albright syndrome is discussed along with the importance of recognition in patients with partial presentation. This case also highlights a rare and potentially devastating neurologic complication of McCune–Albright syndrome, as well as the need for early diagnosis and continual surveillance in these patients.     

Adenomatoid tumor of the skin: Differential diagnosis of an umbilical erythematous plaque

Adenomatoid tumors are benign tumors of mesothelial origin that are usually encountered in the genital tract. Although they have been observed in other organs, the skin appears to be a very rare location, with only one case reported in the literature to our knowledge. We report a second case of an adenomatoid tumor, arising in the umbilicus of a 44‐year‐old woman. The patient presented with an 8‐month‐old erythematous and firm plaque under the umbilicus. A skin biopsy showed numerous microcystic spaces dissecting a fibrous stroma and lined by flattened to cuboidal cells with focal intraluminal papillary formation. This little‐known diagnosis constitutes a diagnostic pitfall for dermatopathologists and dermatologists, and could be misdiagnosed as other benign or malignant entities. Through this case report, a practical approach and diagnostic keys have been devised to avoid misdiagnosis and overtreatment.     

Vohwinkel syndrome: treatment of pseudo‐ainhum

Background Vohwinkel syndrome or keratoderma hereditaria mutilans is a rare autosomal dominant palmoplantar keratosis which manifests in infants and becomes more evident in adulthood. Patients with this mutation present hyperkeratosis of the palms and soles, constricting bands of the digits, usually at the fifth, and starfish‐shaped hyperkeratosis on the dorsal aspects of the hands and feet. The disease mostly occurs in white women, where constricting fibrous bands appear on the digits and can lead to progressive strangulation and auto‐amputation (pseudo‐ainhum). Aim The treatment of this keratoderma is very difficult and tends to be symptomatic: topical keratolytics and systemic retinoids have been used to treat hyperkeratosis, but without consistent results. Reconstructive surgery is utilized for the treatment of pseudo‐ainhum. Results and Conclusion In this study, we present an additional case of Vohwinkel syndrome in which constrictive bands of the fifth digit in the left hand were treated with a cross finger flap, with a favorable outcome after 18 months of follow‐up.     

Granular cell atypical fibroxanthoma: case report and review of the literature

We present a case of granular cell atypical fibroxanthoma of the scalp. The neoplasm occurred as a tender nodule on the frontal scalp of an 82‐year‐old Caucasian man. Histology demonstrated a diffuse proliferation of atypical epithelioid cells with abundant granular cytoplasm. Many of the cells had large irregular nuclei and atypical mitotic figures were present. Immunohistochemical stains were positive for CD68 and procollagen 1 and negative for cytokeratin, HMB‐45 and S‐100 protein. The granular cell phenotype has been observed in other cutaneous neoplasms including granular cell tumors, dermatofibromas, dermatofibrosarcoma protuberans, fibrous papules, basal cell carcinomas, leiomyosarcomas, angiosarcomas and primitive polypoid granular cell tumors. We discuss the differential diagnosis and review the previously reported cases of this rare variant of atypical fibroxanthoma. Wright NA, Thomas CG, Calame A, Cockerell CJ. Granular cell atypical fibroxanthoma: case report and review of the literature.     

A Case of Subcutaneous Malignant Fibrous Histiocytoma Circumscribed by Fibrous Tissue

We report a case of malignant fibrous histiocytoma (MFH) located in the subcutaneous tissue on the right axilla. We excised the tumor sufficiently beyond the clinical margin. It was pathologically diagnosed as a storiform-pleomorphic type of malignant fibrous histiocytoma almost completely circumscribed by fibrous tissue, including fascicles of fibroblasts; this is a rare histological picture. The tumor has not recurred for three years. Although MFH frequently undergoes metastasis, the circumscribed-type subcutaneous MFH characteristic of superficiality and of histologically well-defined structure seems to have a relatively more favorable prognosis after adequate radical excision.     

Fibroelastolytic papulosis: histopathologic confirmation of disease spectrum variants in a single case

Fibroelastolytic papulosis is a rare, acquired fibroelastolytic disorder that presents clinically as white‐to‐yellow papules and plaques most commonly occurring on the neck of elderly patients. The term fibroelastolytic papulosis encompasses two closely related conditions previously described as pseudoxanthoma elasticum‐like papillary dermal elastolysis (PDE) and white fibrous papulosis of the neck (WFPN). Here we present a case of a 78‐year‐old white female with a several‐year history of numerous, asymptomatic 2–3 mm yellowish, non‐follicular papules distributed symmetrically over the posterior neck, axillae, arm and antecubital fossae. Histopathologic examination revealed thickened and clumped elastotic fibers admixed with thick, sclerotic appearing collagen bundles in the mid and deep reticular dermis. Rare melanophages, loss of vertically oriented elastic fibers and scattered elastotic globes were noted in the papillary dermis. Based on the shared clinicopathologic features showed in this case, strong consideration should be made for the additional inclusion of papillary dermal elastosis as existing along the disease continuum of fibroelastolytic papulosis. This occurrence of fibroelastolytic papulosis shows unique histopathologic findings of pseudoxanthoma elasticum‐like PDE, papillary dermal elastosis and WFPN, further supporting the theory that these entities exist as variants along the fibroelastolytic papulosis spectrum.     

Benign fibrous histiocytoma of the eyelid with an unusual clinical presentation

Benign fibrous histiocytoma is a common soft tissue tumor that can be deep or superficially located. Although the deep type of fibrous histiocytoma has a predilection for the orbit, the eyelids are an unusual location for the cutaneous type. A 42-year-old woman had bilateral yellowish nodular masses of the eyelids for two years. Pathological examination after excision revealed benign fibrous histiocytoma. Our case is an unusual clinical presentation of cutaneous fibrous histiocytoma as well as a rare location such as the eyelids.     

Benign fibrous histiocytoma of the foot: a literature review and case report

A rare case of benign fibrous histiocytoma involving the foot of a sixty-three-year-old white man is presented, with a review of the literature. The histopathologic appearance of benign fibrous histiocytoma, differential diagnosis, and surgical management are discussed. Fibrous histiocytomas are characteristically nonencapsulated tumors composed of a mixture of fibroblastic and histiocytic cells arranged in a storiform or cartwheel pattern.     

Ossifying plexiform tumor

We report a rare case of ossifying plexiform tumor in a 64‐year‐old female. The patient had a 2‐year history of gradual hardening of the right thumb pad and pain that radiated up the forearm. Physical examination showed a tender, mobile 2‐cm subcutaneous nodule distending the tip of the right thumb. The biopsy specimen showed a well‐delineated tumor with multiple lobules of epithelioid and spindled cells arranged in a plexiform pattern separated by fibrous bands and having foci of bone formation. The neoplastic cells had scant‐to‐moderate amphophilic cytoplasm with mild nuclear pleomorphism in a myxocollagenous background. No necrosis, mitoses or cytological atypicia were seen. The osteocytes present in the bone islands were bland, with occasional rimming osteoblasts. X‐ray showed stippled calcification in the soft tissue of the distal thumb without involvement of the phalanx. The patient is tumor free for 1 year after complete local excision. Only three cases of ossifying plexiform tumor have been reported. All previous cases and the current case presented as subcutaneous nodules on hand digits of females, measuring 1–2 cm in greatest dimension. Ossifying plexiform tumor appears to be a benign neoplasm with no reports of progression or metastasis.     

McCune-Albright syndrome: a case report in a male

McCune-Albright syndrome (MAS) is a rare, heterogenous, clinical condition caused by a rare genetic mutation. The disorder is more common in females and is characterized by a triad of cutaneous, bone and endocrine abnormalities. We describe a male patient with MAS having multiple café-au-lait macules and deforming polyostotic fibrous dysplasia involving long bones of the limbs, skull and spine without any endocrine abnormality. Severe bone deformities involving almost all bones have not been described previously and this prompted us to present the current case.     

Fibrofolliculoma in a patient with tuberous sclerosis complex

The occurrence of multiple fibrofolliculomas or trichodiscomas on the face and neck is clinically characteristic of Birt‐Hogg‐Dubé (BHD) syndrome, whereas the development of multiple fibrous papules or angiofibromas on the face is an important clinical sign of tuberous sclerosis complex (TSC). It has been suggested that a relationship exists between BHD syndrome and TSC and there was a case of BHD syndrome reported recently, in which multiple fibrous papules or angiofibroma lesions were observed. This report describes the first case, to our knowledge, of fibrofolliculoma in a patient with TSC.     

Aneurysmal Benign Fibrous Histiocytoma with Atrophic Features

Aneurysmal benign fibrous histiocytoma is an uncommon pathologic variant of dermatofibroma. In addition to the features of a typical dermatofibroma, it has large cleft-like or cavernous blood-filled spaces with numerous hemosiderin pigments. It should be differentiated from angiomatoid malignant fibrous histiocytoma, malignant melanoma, and vascular tumors such as Kaposi''s sarcoma and angiosarcoma. Atrophic dermatofibroma is also a rare variant of dermatofibroma, and the combination of aneurysmal and atrophic features is rarer still. We report a case of aneurysmal benign fibrous histiocytoma with atrophic features in a 27-year-old male who had a grayish-brown atrophic patchy lesion on his back for 2 years.     

Primary Cutaneous Solitary Fibrous Tumor on the Back

Solitary fibrous tumors (SFT) are uncommon mesenchymal tumors. SFT have several synonyms including localized fibrous tumor, benign mesothelioma, localized fibrous mesothelioma, and submesothelial fibroma. SFT usually occur in the pleura or other serosal surfaces, but SFT can also develop in extrapleural areas including the nasal cavity, orbit, retroperitoneum, and pelvis. Cutaneous SFT is extremely rare, and more likely to occur in the head and neck region. Histologically, this tumor can mimic a variety of benign and malignant tumors such as dermatofibroma, dermatofibrosarcoma protuberans, spindle cell lipoma or other mesenchymal tumors. Most cases of SFT show non-aggressive clinical courses, with low recurrence rates. Herein, we describe a case of primary cutaneous SFT which presented with huge mass on the back.     

Localized vulvar bullous pemphigoid of childhood: A rare cause of persistent vulvar erosions in children

Localized vulvar bullous pemphigoid of childhood is an excessively rare variant of bullous pemphigoid and affects almost exclusively young girls of 7‐12 years of age. In contrast to adult‐onset bullous pemphigoid, a prompt response to potent topical corticosteroids is observed in the majority of cases, with a favorable prognosis and rare relapses. We report the case of a 7‐year‐old girl who presented with this condition. Our case reinforces the recognition of this rare subtype of childhood bullous pemphigoid as a distinct entity and enlightens the importance of performing a cutaneous biopsy when clinically indicated.     

An unusual histopathologic feature of angiomatoid fibrous histiocytoma – A case report and molecular study

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor with intermediate malignant potential. It predominantly affects children and young adults and is most often located in the extremities. The atypical pattern of AFH may create significant diagnostic difficulty. We present a case of AFH diagnosed via molecular confirmation by fluorescence in situ hybridization (FISH) and also review cases of AFH with atypical histologic presentations in Taiwan. We present this case to remind clinicians that identification of gene fusions by molecular testing is a valuable diagnostic tool for AFHs, especially in cases with atypical histopathological presentations.     

A Case of Perifollicular Fibroma

Perifollicular fibroma (PFF) is a very rare proliferative lesion of the perifollicular sheath. A flesh-colored, dome-shaped papulonodule is usually located on the face or the neck. These papulonodules may be single or multiple. Histologically, the lesion consists of a concentric arrangement of cellular fibrous tissue around a normal hair follicle. We describe here an unusual case of PFF in a 41-year-old female who had an asymptomatic small skin colored papule on the scalp, and this is an uncommon location for PFF.