¿Por qué la cirugía micrográfica de Mohs está infrautilizada en el tratamiento del dermatofibrosarcoma protuberans infantil? / Why Is Mohs Micrographic Surgery Underused in the Treatment of Dermatofibrosarcoma Protuberans in Children?

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Congenital multiple clustered dermatofibroma in a 12-year-old girl.

Congenital multiple clustered dermatofibroma (MCDF) is a rare, idiopathic, benign tumor presenting at birth as an asymptomatic hyperpigmented patch that is stable until puberty, at which time it enlarges and develops papules. Ultimately, MCDF appears to follow a stable, benign course. We present a case of a 12-year-old girl with congenital MCDF. To our knowledge, this is only the third reported case of congenital presentation of MCDF and the only case featuring atrophoderma-like depression.

Congenital multiple clustered dermatofibroma and multiple eruptive dermatofibromas--unusual presentations of a common entity.

Dermatofibroma is one of the most common entities seen in dermatology clinical practice. Several clinical subtypes have nevertheless been described, all of them of uncommon occurrence. The authors present two rare clinical variants of dermatofibromas: congenital multiple clustered dermatofibroma (the presented case is the 4th congenital case to be reported so far) and multiple eruptive dermatofibromas developing in the setting of a Sjögren's syndrome. Since the uncommon subtypes may not be clinically evident, dermatologists should familiarize themselves with their main features and we advise a high level of clinical suspicion in order to reach the correct diagnosis.

Congenital multiple clustered dermatofibroma and multiple eruptive dermatofibromas - unusual presentations of a common entity / Dermatofibroma multiplo agrupado congenito e dermatofibromas multiplos eruptivos - apresentacoes singulares de uma entidade comum

Dermatofibroma is one of the most common entities seen in dermatology clinical practice. Several clinical subtypes have nevertheless been described, all of them of uncommon occurrence. The authors present two rare clinical variants of dermatofibromas: congenital multiple clustered dermatofibroma (the presented case is the 4th congenital case to be reported so far) and multiple eruptive dermatofibromas developing in the setting of a Sjögren's syndrome. Since the uncommon subtypes may not be clinically evident, dermatologists should familiarize themselves with their main features and we advise a high level of clinical suspicion in order to reach the correct diagnosis.

O dermatofibroma é uma das entidades mais frequentemente observadas na prática clínica dermatológica. No entanto, além do dermatofibroma comum, vários subtipos clínicos de ocorrência incomum têm sido descritos na literatura. Os autores descrevem duas variantes clínicas raras de dermatofibromas: dermatofibroma múltiplo agrupado congênito (o caso apresentado é o quarto caso congênito reportado até hoje) e dermatofibromas eruptivos múltiplos no contexto de uma Síndrome de Sjögren. Estes diagnósticos menos comuns podem não ser clinicamente evidentes portanto os dermatologistas devem estar familiarizados com estas apresentações, sendo de suma importância um elevado índice de suspeita clínica.

Benign fibrous histiocytoma with osteoclast-like giant cells in an infant.

Benign fibrous histiocytoma is a common soft tissue tumor that usually occurs in adults and is relatively rare in childhood. This report describes a 7-month-old Caucasian boy with an enlarging firm congenital nodule on his occipital scalp. Histologic analysis revealed a benign fibrous histiocytoma with osteoclast-like giant cells. Benign fibrous histiocytoma with osteoclast-like giant cells is a rare histologic variant.

[Plexiform fibrohistiocytic tumour - case report of a rare low-malignant tumour with frequent localisation at the upper limb]. / Plexiformer fibrohistiozytischer Tumor. Fallbericht - Ein seltener niedrig-maligner Tumor mit vorwiegendem Befall der oberen Extremität.

A male child aged nine months was admitted due to a slow growing, painless resistance at the palmar aspect of the proximal phalanx of the index finger. The intraoperative aspect of the tumour showed a yellow to white soft tissue mass with signs of infiltration. After special histopathological stainings, the diagnosis of a plexiform fibrohistiocytic tumour was confirmed. In literature almost 100 cases were reported since first description by Enzinger and Zhang in 1988. In this paper, 41 of 65 described cases showed tumour localization in the upper limb. This is the second case ever published in Germany and the first with localisation to the hand. There is very little clinical experience with this tumour entity. Local recurrence, lymphatic and pulmonal metastases with lethal outcome in rare cases are described. In the presented case, after secondary radical excision of the tumour, the patient has been free of local recurrence and actually in complete remission for 14 months.

Histiocytic disorders with spontaneous regression in infancy.

The histiocytic disorders are uncommon, have a wide spectrum, and are poorly understood. We describe seven cases developing in infancy, seen during a period of 9 years at Asan Medical Center, Seoul, Korea. Clinically the patients had multiple papules over the face, trunk, and extremities that developed at birth or during infancy. Histopathologic examinations revealed an infiltrate of many histiocytic cells in the upper dermis with or without epidermotropism. Four cases were classified as congenital self-healing reticulohistiocytosis in that the histiocytes were identified as Langerhans cells by positive immunohistochemical staining for S-100 protein, ultrastructural studies showing many Birbeck granules, and spontaneous regression of the lesions within 1-4 months. One infant with a solitary lesion on the forehead was diagnosed as solitary, congenital, indeterminate cell histiocytoma because the histiocytic cells were S-100 protein positive, but meticulous ultrastructural studies did not detect Birbeck granules. The lesion was removed by shave excision. Two cases were classified as generalized eruptive histiocytoma. The histiocytic cells were S-100 protein negative and ultrastructurally Birbeck granules were absent. In one patient, eyeball- or popcornlike lysosomal structures were seen. The lesions regressed completely.

[Solitary congenital histiocytoma: two cases]. / Histiocytomes congénitaux : 2 cas.

BACKGROUND: Solitary histiocytoma is an uncommon form of Hashimoto-Pritzker syndrome and an exceptional type of histiocytosis with cells of undetermined origin. A solitary often ulcerated congenital nodule is generally observed. We report two cases, one of each form. CASE REPORTS: Both cases presented an ulcerative budding congenital tumefaction of the plantar aspect of the right foot for the first child and the parieto-axillary region in the second. Histology disclosed a granulomatous infiltrate of histiocytes positive for specific immunolabels (protein S100 and CD1a). In the first case, electron microscopy revealed histiocytes devoid of Birbeck granules and myelinoid bodies leading to the diagnosis of Langerhans histiocytosis with cells of unknown origin. In the second case, 18 p. 100 of the cells contained Birbeck granules. There has been no recurrence after a 5-year follow-up in a case. DISCUSSION: These cases recall the congenital nature of some types of solitary histiocytomas. Indeed, congenital Langerhans histiocytoma can occur as a unique nodule. The tumefaction may lie in any localization. Histological diagnosis is required. The benign nature of these lesions is confirmed by the absence of distant lesions and the lack of recurrence after complete excision. About a dozen cases have been reported. Most have been Hashimoto-Pritzker syndromes. Only one case has been reported with cells of undetermined origin. The diagnosis of histiocytosis with cells of undetermined origin is made when the ultrastructure study demonstrates the vacuity of the histiocyte cytoplasm. This condition is similar to Hashimoto-Pritzker syndrome by the absence of recurrence and systemic diffusion. It can however be observed in adults. The undetermined cell types would correspond different phases of Langerhans cell maturation or involution.

Congenital multiple clustered dermatofibroma.

Multiple clustered dermatofibroma (MCD) is a rare tumour which usually appears during the first and second decades of life. We report a man in whom the MCD was congenital, although during the first few years of his second decade it extended to involve a broad zone on the left hip, gluteal region and upper thigh.

Benign fibrous histiocytoma of the nasal cavity in a newborn: MR and CT findings.

Benign fibrous histiocytoma of the nasal cavity in a newborn is rare, and the MR imaging appearance of this entity has not been reported. We present the MR and CT findings in such a case and review the differential diagnosis for intranasal masses in the neonate.

Solitary congenital indeterminate cell histiocytoma.

BACKGROUND: Indeterminate cell proliferations are rarely described in the literature. We present a case of a patient who presented at birth with a solitary lesion that spontaneously regressed. OBSERVATIONS: Five cases of indeterminate cell proliferations have been described in the literature. Their clinical patterns display some common features including adult onset, usually with multiple lesions and a relatively benign clinical course. This patient presented at birth with a single lesion and spontaneous regression, as one would expect with congenital self-healing reticulohistiocytosis. Biopsy confirmed a dermal histiocytic nodule that was predominantly CD1 and S100 positive. Unlike patients with congenital self-healing reticulohistiocytosis, however, electron microscopy failed to demonstrate Birbeck granules. CONCLUSION: Indeterminate cell proliferations are a distinct entity. This presentation expands the range of clinical manifestations reported. It also suggests a close relationship between indeterminate cells and Langerhans cells.

A case of congenital angiofibroma.

A case of angiofibroma affecting the right maxilla of a new born baby is presented with histological evidence. We believe this to be the first congenital case of this condition to have been reported.

Congenital angiomatoid malignant fibrous histiocytoma. A light-microscopic, immunopathologic, and electron-microscopic study.

We present a case of a congenital angiomatoid malignant fibrous histiocytoma. This rapidly growing lesion, which was located in the subcutis of the left upper arm, was excised at the age of 8 1/2 months. The patient, a girl, was well and free of disease 10 months after surgical removal of the tumor. The tumor appeared grossly encapsulated. The gray-tan tissue contained cystic spaces filled with recent and organizing hemorrhages. Microscopically, the tumor was composed of solid masses of histiocyte- and fibroblast-like cells, inflammatory infiltrate, and multifocal irregular blood-filled spaces, which were predominantly devoid of endothelial cells. The tumor was studied immunohistochemically with antibodies specific for FVIII-related antigen, S-100 protein, epithelial membrane antigen, vimentin, desmin, alpha-1-antitrypsin, muramidase, laminin, and collagen type IV. Ulex europaeus lectin-I was also utilized. These studies, along with our ultrastructural findings, suggest that: (a) the tumor is composed of a mixture of mesenchymal cells; (b) an imperfect angiogenesis may be taking place, resulting in a wide spectrum of vascular structures; and (c) the cell of origin may be a pluripotent mesenchymal cell.

Congenital fibrosarcoma: a congenital fibrous histiocytoma.

Using immunoperoxidase PAP technique in 2 cases of congenital fibrosarcoma, a great number of cells showed positive stain for alpha-1-antitrypsin (A1AT) and alpha-1-antichymotrypsin (A1ACT), both considered to be good histiocytic markers. The ultrastructure in 1 case also provides evidence of histiocytic differentiation. These findings suggest that congenital fibrosarcomas are actually congenital fibrous histiocytomas and may explain the presence of inflammatory infiltrates as in some malignant fibrous histiocytomas.

Congenital fibrous lesion of the temporal bone.

A neonate had a destructive, fibrous lesion of the temporal bone. There was x-ray evidence of disseminated, lytic disease. A histologic diagnosis of malignant fibrous histiocytoma was made. No treatment was given, yet the child survived with spontaneous resolution of her lesions. We briefly discuss malignant fibrous histiocytoma and propose congenital generalized fibromatosis as an alternative diagnosis to explain the child's recovery.