Circumscribed Storiform Collagenoma (Sclerosing Fibroma)

Storiform collagenoma as a clue for Cowden disease or PTEN hamartoma tumour syndrome

“…Microscopic examination ( fig 1) showed a paucicellular (although a cellular variant has been reported 10 ) well-circumscribed dermal nodule composed chiefly of collagen bundles with a distinctive interweaving, storiform pattern with prominent elongated clefts between them, typical of a storiform collagenoma. 11 Tinctorial stains showed absence of elastic fibres (Orcein and EVG) within the lesion and reticulin fibres were seen prominently (Reticulin). Immunohistochemistry showed negative staining with CEA, EMA or keratin staining.…”

“…Most of the cells within the nodule stained positively for vimentin, and factor XIIIa stain highlighted scattered dermal dendritic cells. 11 On the basis of similar fibrotic changes observed in dermatofibroma, it has been speculated that storiform collagenoma may have diverse origins. This issue has been resolved by showing that type 1 collagen synthesis indicates fibroblastic origin of storiform collagenoma.…”

“…13 14 Storiform collagenoma has been described in association with CD but can also arise sporadically. 11 It occurs most often in young and middle-aged adults 6 and usually presents as a slow growing solitary nodule up to 1 cm in diameter on the head and neck or upper extremities. Typical histopathological, histochemical and immunohistochemical appearances as described by Metcalf et al 11 were present in our patient.…”

Sclerotic fibroma (storiform collagenoma)‐like stroma in a fibroadenoma of axillary accessory breast tissue

“…Sclerotic fibroma (storiform collagenoma) is an uncommon skin neoplasm manifesting clinically as round to ovoid, skin‐colored papules. The lesion may be part of Cowden's disease (multiple hamartoma and neoplasia syndrome), an autosomal dominant disorder; or it can be found incidentally, without other manifestations of this syndrome …”

Lhermitte-duclos disease associated with Cowden disease

“…Benign fibroma and multiple sclerotic fibromas have also been reported recently. 25 Other skin complications are hemangioma, lipoma, neuroma, dermal fibroma, and so on. Multiple hamartomas appear in the thyroid, breast, gastrointestinal tube, genitourinary system, bones, and nervous system.…”