Giant cell collagenoma: case report and review of the literature

BACKGROUND: Giant cell collagenoma (GCC) is a recently described cutaneous fibrous neoplasm that usually affects young to middle-aged adults. Despite its similar histological appearance with circumscribed storiform collagenoma, no association of GCC with Cowden's syndrome has been described so far. To the best of our knowledge only five cases of this rare fibrous tumor have been reported so far. METHODS: We report a case of a 79-year-old male patient presenting with a slow growing flesh-colored dome-shaped lesion in his left forearm, with a clinical diagnosis of fibroma. No stigma of Cowden's syndrome was depicted. RESULTS: The histological analysis showed a symmetrical and well-circumscribed flat-dome-shaped lesion covered by an atrophic overlying epidermis. The neoplasm was composed of hyalinized collagen bundles disposed in a whorled storiform pattern. Admixed with the collagen matrix, there were two distinct cell populations, one composed by spindle-shaped mononuclear cells, and the other composed by bizarre multinucleated giant cells. Immunohistochemical analysis showed positivity for vimentin and actin HHF35 in the mononucleated. The multinucleated cells only immunoexpressed vimentin. CONCLUSION: GCC is an unusual cutaneous fibrous tumor that should be differentiated from circumscribed storiform collagenoma, pleomorphic fibroma, regressive forms of dermatofibroma, and solitary myofibroma based on its histological features.     

席纹状胶原瘤1例

患者男,20岁,左侧鼻翼发疹7年,渐增大。皮肤科情况:左侧鼻翼有一枚直径约0.9 cm×0.9 cm大小皮色结节,表面光滑,质地坚韧。皮损组织病理示:肿瘤占据真皮全层,边界尚清,无包膜。瘤体主要由席纹状或旋涡状排列的胶原纤维束组成,结构致密,散布星状或梭形纤维母细胞。诊断:席纹状胶原瘤。     

席纹状胶原瘤

报告1例席纹状胶原瘤。患者男,46岁。背部肿物40年余,缓慢增长,无自觉症状。无系统性疾病病史。皮损组织病理检查示表皮轻度萎缩,真皮内可见一边界清楚的结节,无包膜,病变均匀、局限,由大量透明样变的胶原束组成,胶原束之间见许多裂隙,排列成席纹状。诊断:席纹状胶原瘤。     

Pacinian collagenoma

An unusual histological variant of collagenoma is described. A 36-year-old woman presented with a lump in the left hypothenar eminence. Histological examination revealed a well-delineated lesion composed of paucicellular collagen fibres arranged in concentric lamellations giving rise to an onion skin appearance. The overlying epidermis was thin and the lateral borders were demarcated by an epidermal collarette. Inflammation and xanthoma cells were absent and occasional capillaries were present. The lesion was positive for collagen stains, reticulin and CD34. This lesion represents an uncommon histological form of collagenoma or fibroma. It can be distinguished from histological look-alikes on the basis of the characteristic morphology and immunophenotype.     

Sporadic sclerotic fibroma of the oral soft tissues

Sclerotic fibroma (SF) is an uncommon, benign fibrous neoplasm that may present either as a sporadic, small, solitary cutaneous mass, in otherwise healthy individuals, or as solitary or multiple, discrete skin nodules in patients with Cowden syndrome. Oral SF has been reported in patients with Cowden syndrome; however we now report the first documented series of sporadic SF originating within the oral mucosa. We describe 5 cases of SF arising in 3 women and 2 men with an age range of 43 to 66 years. The buccal mucosa was the site of involvement in 4 patients and the lower lip in 1 patient. Microscopically, each of the tumors was characterized by an unencapsulated, well-circumscribed, hypocellular submucosal nodule that was sharply demarcated from the surrounding tissues. The neoplasms were primarily composed of thick collagen bundles that were occasionally arranged in a storiform pattern. Prominent clefts separated many of the collagen bundles. In all cases, spindle and stellate-shaped cells containing fusiform or stellate-shaped nuclei and inconspicuous nucleoli were found scattered throughout the lesion. Occasional stellate-shaped, multinucleated cells were also seen. Many of the cells also exhibited long dendritic cytoplasmic processes. The tumor cells strongly expressed CD34 and vimentin, and occasionally factor XIIIa, but were negative for markers of myofibroblastic, neural or melanocytic differentiation. These findings confirm that oral SF represents a unique entity and should be differentiated from more commonly occurring benign fibrous lesions of the oral soft tissues.     

Storiform collagenoma of the nail.

The term circumscribed storiform collagenoma was coined by Maize et al in 1989 to describe four dermal fibromas with pathologic features resembling the sclerotic fibromas associated with Cowden's disease. We report a subungual nodule with the pathologic features of circumscribed storiform collagenoma in a patient without Cowden's disease.     

A Case of Eruptive Collagenoma on the Left Calf

Eruptive collagenoma is an acquired connective tissue nevus without family history. It is typically described as numerous small papules or nodules on the trunk and arms with histopathological features of decreased or degenerated elastic fibers. We report a case of a 16-year-old male who presented with multiple asymptomatic 2 to 5 mm sized yellowish grouped papules on the left calf. Histopathologically, the lesion showed thickened homogenized collagen fibers highlighted by Masson trichrome stain and decreased and fragmented elastic fibers stained by Verhoeff-van Gieson stain. The skin lesion was diagnosed as eruptive collagenoma and no treatment was provided.     

Circumscribed storiform collagenoma (sclerosing fibroma)

In the past several years we have examined eight dermal nodules that have morphologic features identical to the nodules described in patients with Cowden's disease. The patients in this series had no other clinical manifestations of Cowden's disease. In an attempt to better define this distinctive entity, we subjected tissue sections to a battery of histochemical and immunohistochemical stains and examined tissue from one of the nodules ultrastructurally. Although we found similarities between these nodules and other common dermal fibrotic lesions, we believe that they are distinctive architecturally (they are sharply circumscribed and have a strikingly uniform storiform pattern) and immunohistochemically (with uniformly scattered factor 13a-positive cells). Because of the unique histologic features, we propose that the term "circumscribed storiform collagenoma" be applied to these nodules.     

Dermatofibroma with sclerotic areas resembling a sclerotic fibroma of the skin

BACKGROUND: Dermatofibromas are common benign tumors that occur as single or multiple nodules on the extremities in adults. Sclerotic fibroma of the skin (SFS) is a benign tumor characterized histopathologically by a well-demarcated, non-encapsulated dermal nodule composed of hypocellular, sclerotic collagen bundles with prominent clefts. The pathogenesis of these two conditions is still in dispute. METHODS: We present a case of dermatofibroma with sclerotic areas resembling a sclerotic fibroma of the skin and a review of the literature. RESULTS: The tumor showed a well-demarcated dermal, fibrocollagenous tumor with three different histopathological features. One-fourth of the lesion was consistent with dermatofibroma. Another area adjacent to dermatofibroma revealed hyalinized eosinophilic collagen bundles arranged in the characteristic interwoven pattern with prominent clefts, as is described in sclerotic fibroma of the skin. One-half of the lesion between the dermatofibroma and sclerotic fibroma showed transitional changes from dermatofibroma to sclerotic fibroma. CONCLUSION: According to these findings, the possibility that sclerotic fibroma is an ancient or degenerated stage of dermatofibroma cannot be completely ruled out, but some authors still consider that dermatofibroma and sclerotic fibroma of the skin are completely different neoplasms.     

发疹性皮肤胶原瘤1例

报告发疹性皮肤胶原瘤1例,患者男,38岁。背部,肩部出现多发性扁平丘疹,结节及斑块8年,皮损为正常肤色,直径数毫米至数厘米,部分融合成斑块,无自觉症状,无家族史,组织病理检查示,胶原纤维变厚,增殖,弹性纤维减少,诊断为发疹性皮肤胶原瘤。     

Eruptive collagenoma. Apropos of a case with ultrastructural study

A 29-year-old male patient was examined for numerous asymptomatic dermal nodules symmetrically distributed over the back and the shoulders. The nodules were firm, round to oval and varied in size from a few millimeters to 2 centimeters. They had appeared in normal skin without any known previous inflammation or injury, and they has been present without modification for nine years. At histological examination a dermal accumulation of abundant and large collagen bundles was observed. Elastic fibers seemed to be less numerous than normally. Alcian blue showed no increase in acid mucopolysaccharides. An ultrastructural study displayed normal, mature collagen fibers arranged in large bundles. Cutaneous collagenoma, initially described by Colomb as "eruptive collagenoma" is a rare disorder of unknown etiology occurring in young adults as symptomless dermal nodules without previous history of illness or injury and mostly localized to the back. No change is usually observed in these nodules for several years. Histologically, the nodules are characterized by a proliferation of collagen in the dermis; elastic fibers are generally reduced in number. Our ultrastructural study showed that proliferation of normal collagen fibers is the main morphological finding.     

Papulolinear Collagenoma with Arborizing Arrangement: Report of a Case

Abstract:  Connective tissue nevi of collagen type are now classified in four major subtypes. In addition to the clinicopathological features of papulolinear collagenoma, which is considered as a variant of isolated collagen harmatoma, the case we present has a unique arborizing pattern.     

Isolated Collagenoma: A Case Report with a Review of Connective Tissue Nevi of the Collagen Type

A 22-year-old male had had five skin colored tumorous growths on the mid-palm, ring and little fingers of the right hand for the last 15 years. Histopathological examination with hematoxylin and eosin and special stains confirmed the diagnosis of connective tissue nevi of collagen type. There were no associated cutaneous or systemic findings; thus the case was designated as isolated collagenoma. A review of connective tissue nevi of the collagen type is presented.     

Segmental storiform collagenomas: Expanding the spectrum of PTEN hamartoma tumor syndrome in children

A 4‐year‐old girl with autism spectrum disorder and congenital heart disease presented to dermatology clinic for evaluation of skin growths present since infancy. Physical examination was significant for macrocephaly and agminated skin‐colored to pink papulonodules in a segmental distribution on the right lower back and buttocks, biopsy of which showed storiform collagenomas (sclerotic fibromas). Genetic testing revealed a pathogenic missense mutation in the PTEN gene, and a diagnosis of PTEN hamartoma tumor syndrome was made. The segmental nature of her storiform collagenomas is unique, to our knowledge, and may be explained by a postzygotic second‐hit PTEN mutation, contributing to the growing spectrum of clinical findings associated with PTEN hamartoma tumor syndrome.     

Multiple periorbital fibrous histiocytomas: a light and electron microscopic study.

Recurrent, bilateral, multiple, periorbital fibrous histiocytomas occurred in a 62-year-old woman. There were no associated serum lipid abnormalities. Histologically, the tumor was composed of an admixture of lipid-laden histiocytes and fibroblasts with a variable amount of collagen production. In many areas the fibroblasts were arranged in a storiform pattern. No cytologic evidence of malignancy was observed. Ultrastructural studies of the tumor showed histiocytic cells with a variable amount of lipid in the cytoplasm and fibroblasts with mild to moderate collagen production. Differential diagnosis and histogenesis are discussed.     

Sclerosing dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans is a low-grade sarcoma of the skin with well characterized clinical and histopathological features. The lesion frequently appears as a plaque on the trunk or on the extremities of middle-aged adults. Microscopically, the tumor is composed of monomorphous spindle cells arranged in storiform pattern and embedded in a sparse to moderately dense fibrous stroma. We report on two unusual cases of dermatofibrosarcoma protuberans in which neoplastic cells were diffusely replaced by sclerotic tissue in more than 50% of the entire tumor. In both cases, no external trauma or radiotherapy were recorded. Since tumor regression has been defined as a loss of tumor mass in the absence of any treatment (or trauma) we believe that sclerosis in DFSP may represent a manifestation of regression and not, as previously suggested, a new variant of the neoplasm.     

Atypical fibroxanthoma with prominent sclerosis

BACKGROUND: Malignant cutaneous spindle cell lesions with marked sclerosis are uncommon. Only a few cases of cutaneous leiomyosarcoma and dermatofibrosarcoma protuberans with sclerosis have been published. METHODS: We report a case of atypical fibroxanthoma (AFX) with prominent sclerosis and hyalinization occurring on the scalp of an 81-year-old male. RESULTS: Histopathologic examination revealed an exophytic, well-delineated, focally ulcerated tumor arising in sun-damaged skin. The lesion was composed of atypical spindle cells arranged in a fascicled and vaguely storiform pattern. Occasional multinucleated giant cells were present. The tumor cells were strongly positive for CD99 (O13), vimentin, and smooth muscle actin, and focally positive for CD68. There was striking sclerosis with hyalinization throughout the lesion. CONCLUSIONS: Rarely, AFX may exhibit marked sclerosis with areas of complete replacement of tumor by hyalinized collagen. In a small biopsy, such hyalinization may be a diagnostic pitfall leading to an erroneous diagnosis.     

Progress of an isolated collagenoma during pregnancy

Collagenomas are connective tissue naevi composed predominantly of collagen. Isolated collagenomas are usually localized to a single body region, acquired, and of rare occurrence. We describe a patient with an isolated collagenoma that showed an increase in size during pregnancy and regressed afterwards.     

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.