Pleomorphic sclerotic fibroma: a case report and literature review.

Pleomorphic sclerotic fibroma is a benign neoplasm exhibiting features of sclerotic fibroma and pleomorphic fibroma. We report another such case. The tumor presented as a firm, 0.5-cm, flesh-colored papule on the forehead of a 72-year-old white man for an unknown duration. Histologic examination revealed a neoplasm in which the superficial portion showed features of a pleomorphic fibroma, the deeper portion showed features of a sclerotic fibroma, and a transitional area was present in between. We propose that pleomorphic fibroma, sclerotic fibroma, and pleomorphic sclerotic fibroma form a spectrum. Pleomorphic sclerotic fibroma may be used as a broad diagnostic term to encompass the spectrum.     

Pleomorphic hyalinizing angiectatic tumor of soft parts: report of a case and review of the literature

We present the case of a 69-year-old man with a subcutaneous pleomorphic hyalinizing angiectatic tumor of soft parts on the back. In addition to its rare localization, it showed unique clinical features of an exceptionally long history. Histological characteristics of this tumor showed thin-walled angiectatic blood vessels lined with a thick eosinophilic and hyalinotic amorphous material. Tumor cells are composed of pleomorphic cells. It was surgically removed, and there was no recurrence by 11 months postoperatively.     

Clear cell atypical fibroxanthoma - report of a case with review of the literature

Clear cell atypical fibroxanthoma (CCAFX) is a rare variant of atypical fibroxanthoma (AFX), a pleomorphic dermal tumour associated with a good prognosis. A 67-year-old man presented with a rapidly growing nodule on the ear, which had appeared over a 2-week period. Sections showed an ulcerated nodule composed of pleomorphic spindled and polygonal cells with clear cytoplasm, invested by a delicate vascular stroma, reminiscent of clear cell renal cell carcinoma. Numerous mitotic figures were seen. The tumour cells stained with vimentin, CD68 and CD99 and were cytokeratin-negative. The immunohistochemical and ultrastructural features supported a diagnosis of CCAFX. The diagnosis of CCAFX requires the exclusion of other pleomorphic clear cell tumours that can occur in the skin by using a combination of morphology, immunohistochemistry and electronmicroscopy. Murali R, Palfreeman S. Clear cell atypical fibroxanthoma - report of a case with review of the literature.     

Dermal pleomorphic liposarcoma resembling pleomorphic fibroma: report of a case and review of the literature

Pleomorphic liposarcoma (PLPS) is a rare, high‐grade sarcoma defined by the presence of pleomorphic lipoblasts. Constituting 5% of all liposarcomas, PLPS usually arises in deep soft tissues of the extremities, with rare occurrences in the dermis and subcutis. We describe a unique case of an 85‐year‐old Caucasian gentleman with a 1 year history of a pedunculated, pink, non‐tender papule on the dorsum of his left arm, measuring 1.0 cm in maximum dimension. Biopsy revealed a dermal collection of atypical epithelioid and spindle cells superimposed on a sclerotic background, resembling a pleomorphic fibroma on low power. On high power, a central focus of discrete adipocytic differentiation with pleomorphic lipoblasts was present. Tumor cells were positive for S‐100 and negative for desmin, actin, CD68, keratin, MART‐1 and CD34. Clinicopathologic findings were consistent with PLPS and the diagnosis was made. PLPS is rarely localized to the dermis and one with low power features resembling a pleomorphic fibroma has not been previously described in the literature.     

Subungual pleomorphic fibroma

BACKGROUND: Pleomorphic fibroma is a benign fibroblastic tumor characterized by pleomorphic, hyperchromatic cells or giant multinucleated cells embedded in a collagenous stroma. These cytologic features may lead to an incorrect diagnosis of malignancy. Most cases reported in the literature are located on trunk or extremities; the presentation as a subungual mass is rare. METHODS: We report an unusual case of a subungual pleomorphic fibroma in 66-year-old woman. Clinical information was obtained. Histologic examination and immunohistochemical studies were performed. RESULTS: A 66-year-old woman presented with a longstanding (40 years), subungual mass that deformed the nail of the left middle finger. Microscopic examination revealed a paucicellular tumor composed of hyperchromatic spindled, pleomorphic, floret-like giant cells embedded in haphazardly arranged collagen bundles in the dermis. No mitotic figures were seen. The tumor cells were vimentin-positive but did not stain with antibodies to S-100, cytokeratin, smooth muscle actin, factor XIIIa or CD34 negative. The diagnosis of a pleomorphic fibroma was made. Follow-up shows no evidence of tumor, 36 months after excision. CONCLUSION: Pleomorphic fibroma of the subungual region is an unusual cutaneous tumor with histologic features that may cause confusion with true sarcomas. This is only the second case reported of a subungual pleomorphic fibroma. Pleomorphic fibroma should be considered in the differential diagnosis of pleomorphic subungual tumors.     

Progression of a myxoid pleomorphic fibroma to myxofibrosarcoma

A 75-year-old man presented with a protuberant stable lesion of many years on his left second toe. Histology on an incisional biopsy showed myxoid pleomorphic fibroma. Over the subsequent 21 months, the lesion enlarged and became exophytic and irregular. Repeat biopsy showed features consistent with a grade III myxofibrosarcoma. Treatment involved amputation of his toe. Six months later he remains well, with no evidence of local recurrence or systemic spread.     

Relación entre fibroxantoma atípico y sarcoma pleomórfico dérmico: histopatología de ambos y revisión de la literatura

The relation between atypical fibroxanthoma and pleomorphic dermal sarcoma has led to confusion and debate in the literature. Both tumors present on sun-exposed skin, typically on the head and neck, in patients of advanced age. Both are comprised of a variable mix of histiocytoid, spindle, epithelioid, and/or giant multinucleated cells with pleomorphic nuclei. No immunohistochemical diagnostic techniques have emerged to distinguish these tumors. Diagnosis is by exclusion. Histologically, atypical fibroxanthoma is seen as a well-circumscribed dermal nodule but there will be no evidence of extensive subcutaneous invasion, tumor necrosis, or lymphovascular or perineural invasion. Therefore, if any of the aforementioned features is present, the diagnosis would be pleomorphic dermal sarcoma. This narrative review of the literature aims to identify the distinguishing and overlapping histopathologic features of these 2 tumors as they have been described in case series.     

富含中性粒细胞的原发皮肤CD30阳性大细胞间变淋巴瘤二例

富含中性粒细胞的原发皮肤CD30阳性大细胞间变淋巴瘤是原发皮肤CD30阳性大细胞间变淋巴瘤的一种组织学亚型,具有较为独特和复杂的临床和组织学特点,容易误诊.我们报道2例富含中性粒细胞的原发皮肤CD30阳性大细胞间变淋巴瘤,患者均表现为具有脓性分泌物的红斑、结节,组织学除了大量表达CD30的多形性细胞外,尚可见大量的中性粒细胞.     

富含中性粒细胞的原发皮肤CD30阳性大细胞间变淋巴瘤二例

富含中性粒细胞的原发皮肤CD30阳性大细胞间变淋巴瘤是原发皮肤CD30阳性大细胞间变淋巴瘤的一种组织学亚型,具有较为独特和复杂的临床和组织学特点,容易误诊.我们报道2例富含中性粒细胞的原发皮肤CD30阳性大细胞间变淋巴瘤,患者均表现为具有脓性分泌物的红斑、结节,组织学除了大量表达CD30的多形性细胞外,尚可见大量的中性粒细胞.     

富含中性粒细胞的原发皮肤CD30阳性大细胞间变淋巴瘤二例

富含中性粒细胞的原发皮肤CD30阳性大细胞间变淋巴瘤是原发皮肤CD30阳性大细胞间变淋巴瘤的一种组织学亚型,具有较为独特和复杂的临床和组织学特点,容易误诊.我们报道2例富含中性粒细胞的原发皮肤CD30阳性大细胞间变淋巴瘤,患者均表现为具有脓性分泌物的红斑、结节,组织学除了大量表达CD30的多形性细胞外,尚可见大量的中性粒细胞.     

Recurrent pleomorphic solitary angiomyxoma of the face.

The historical background of solitary and multiple myxoid tumors of the dermis is accompanied by a confusing conglomeration of names. We report a case whose histologic features do not fit exactly with previous classifications. It appeared as a solitary angiomyxoma of the face containing stellate pleomorphic cells and exhibiting a locally infiltrative behavior with tendency for recurrences over more than 25 years. This recurrent pleomorphic solitary angiomyxoma appears to be distinct from cutaneous myxomas that exhibit a very low local recurrence rate and from malignant tumors with myxoid deposits. The treatment consisted of a wide surgical excision.     

Diffuse cutaneous reticulohistiocytosis in a child with tuberous sclerosis

Reticulohistiocytosis, a rare disorder occurring almost exclusively in adults, was seen in "pure" diffuse cutaneous form in an 8-year-old boy who had tuberous sclerosis since birth. The clinical features consisted of many papulonodular lesions, located mainly on the trunk and to a lesser extent on the head and limbs. Histologic findings were distinctive. Ultrastructural examination revealed the presence of pleomorphic cytoplasmic inclusions in almost all of the giant histiocytic cells of the infiltrate.     

Pleomorphic fibroma of the skin with MDM2 immunoreactivity: A potential diagnostic pitfall

Pleomorphic fibroma is a rare benign cutaneous neoplasm characterized by spindle‐shaped cells and multinucleated giant cells scattered throughout collagenous stroma. These morphologic features can lead to diagnostic confusion, including atypical lipomatous tumor as one consideration. In contrast to atypical lipomatous tumor, previous studies have found pleomorphic fibroma to be negative for MDM2 immunohistochemical staining and MDM2 gene amplification. Here, we present a case of pleomorphic fibroma of skin with nuclear MDM2 immunoreactivity in the absence of MDM2 gene amplification, underscoring the superiority of fluorescence in situ hybridization as a diagnostic test in this differential diagnosis. The RB1 locus is also explored for differential diagnosis with pleomorphic/spindle cell lipoma and related entities.     

Fibroepithelioma of pinkus with tumor giant cells

A case of fibroepithelioma of Pinkus with pleomorphic epithelial giant cells is reported. The lesion was an ovoid polypoid nodule measuring 4 mm x 3 mm x 2 mm and was located close to the right axilla in an 86-year-old woman. The immunohistochemical features of the epithelial giant cells indicate that most of these cells are not cycling. We suggest that these cellular changes may represent a senescent event. Giant cells showed a mean nuclear major diameter more than twice that of small cells. Flow cytometric study of the tumor showed a hypodiploid DNA content and an intermediate grade S-phase fraction of the aneuploid component. To the best of our knowledge, a pleomorphic variant of Pinkus fibroepithelioma has not been reported to date. In fibroepithelioma of Pinkus, the correct diagnosis depends primarily on the architectural pattern of the tumor rather than on its cytologic features.     

Cutaneous Metastatic Undifferentiated Pleomorphic Sarcoma from a Mediastinal Sarcoma

Undifferentiated pleomorphic sarcoma, known as malignant fibrous histiocytoma, is a malignant neoplasm that arises in both soft tissue and bones. In 2002, the World Health Organization declassified malignant fibrous histocytoma as a formal diagnostic entity and renamed it ''undifferentiated pleomorphic sarcoma not otherwise specified.'' It most commonly occurs in the lower extremities and rarely metastasizes cutaneously. We report a case of cutaneous metastatic undifferentiated pleomorphic sarcoma of the buttocks occurring in a 73-year-old man diagnosed with mediastinal sarcoma 4 years previously. He first noticed the mass approximately 2 months previously. Histological findings with immunomarkers led to a final diagnosis of cutaneous metastatic sarcoma from mediastinal undifferentiated pleomorphic sarcoma.     

Juvenile xanthogranuloma: a congenital giant form leading to a wide atrophic sequela

Juvenile xanthogranuloma is the most common non-Langerhans cell histiocytosis of infancy and childhood. It is a benign disorder that usually regresses spontaneously, more often without or with limited skin changes. The clinical features are very pleomorphic and recognition of the atypical presentations should facilitate the diagnosis. We report a 9-month-old boy who had an extensive, atrophic lesion on the proximal thigh that followed the course of the congenital giant form. The large size of the lesion does not alter the clinical course or result in extracutaneous involvement, which remains rare. However, uncommon clinical presentations raise the problem of differential diagnosis, requiring biopsy to eliminate other tumors with a less favorable prognosis.     

Aberrant Melan-A expression in atypical fibroxanthoma and undifferentiated pleomorphic sarcoma of the skin

Background: Atypical fibroxanthoma (AFX) is a distinctive clinicopathologic entity presenting on sun‐damaged skin of the elderly. Its behavior is benign if strict diagnostic criteria are applied. Tumors showing invasion of deeper structures or perineural/lymphovascular invasion are best regarded as undifferentiated pleomorphic sarcoma of the skin. The diagnosis requires immunohistochemical studies to exclude melanoma, squamous cell carcinoma, angiosarcoma and leiomyosarcoma. Methods: Two AFX and one undifferentiated pleomorphic sarcoma showing aberrant expression of Melan‐A were identified. Clinical data were obtained and histopathological features, immunohistochemical profile and electron microscopy were assessed. Results: All tumors arose on sun‐damaged skin of elderly males. Two AFX showed pushing growth into superficial subcutis only. The undifferentiated pleomorphic sarcoma was characterized by infiltrative growth into galea as well as perineural invasion. Multifocal expression of Melan‐A and MART‐1 was largely limited to tumor giant cells in the absence of S100 or HMB‐45 labeling. No melanosomes or premelanosomes were identified by electron microscopy. Conclusions: Aberrant expression of Melan‐A and MART‐1 in AFX and undifferentiated pleomorphic sarcoma of the skin represents an important diagnostic pitfall with potential for misdiagnosis as melanoma. Melan‐A expression limited to tumor giant cells, in the absence of S‐100 positivity, is a helpful diagnostic feature. Thum C, Hollowood K, Birch J, Goodlad JR, Brenn T. Aberrant Melan‐A expression in atypical fibroxanthoma and undifferentiated pleomorphic sarcoma of the skin.     

Histopathological evolution of a cutaneous myxofibrosarcoma

A 54-year-old woman presented with a 6-month history of a tender and swollen plaque appearing as a panniculitis affecting the left pretibial area. The initial histopathology revealed dermal and subcutaneous inflammation with interstitial histiocytes and mucinosis suggestive of either granuloma annulare or necrobiosis lipoidica. Over the subsequent 6 months the plaque grew progressively, despite treatment with topical corticosteroids under occlusion. Distal to the plaque reduced sensation developed in the limb. Biopsies of the nodular areas now revealed a dense dermal infiltrate of atypical spindle cells within a prominent myxoid stroma and a pleomorphic multinucleated epithelioid cell component. These features were those of an intermediate grade myxofibrosarcoma. This soft-tissue sarcoma may initially masquerade clinically as a panniculitis and a granulomatous process on biopsy as in our patient, leading to a delay in surgical therapy.     

Low‐grade fibromyxoid sarcoma with nuclear pleomorphism arising in the subcutis of a child

Low‐grade fibromyxoid sarcoma (LGFMS) represents a rare soft tissue tumor that was first characterized in 1987. LGFMS usually presents as a large, deeply situated mass in adults and is characterized by deceptively bland histopathologic features. LFGMS is less common in superficial soft tissue and in children. It is distinctly uncommon for LGFMS to exhibit nuclear pleomorphism. Herein, we present a case of a 10‐year‐old male who presented with a subcutaneous back mass that displayed features typical for LGFMS as well as scattered large, hyperchromatic and pleomorphic nuclei. The constellation of clinicopathologic features, including the young age of the patient, the small size and superficial location of the tumor and the presence of scattered nuclear pleomorphism are all unusual features for LGFMS. Fluorescent in situ hybridization (FISH) with a break‐apart probe for FUS revealed the presence of a FUS gene rearrangement confirming the diagnosis of LGFMS. This case highlights the importance of maintaining a high index of suspicion for LGFMS even in the context of small, superficially‐located tumors, pediatric patients or tumors with scattered nuclear pleomorphism.