Dermatofibrosarcoma protuberans: lo diagnosticamos en la edad pediátrica / Dermatofibrosarcoma protuberans: we diagnose it in the pediatric age

El dermatofibrosarcoma protuberans (DFSP) es un tumor cutáneo, de baja frecuencia, fibrohistiocítico, infiltrante, de lento crecimiento, de agresividad local, de malignidad intermedia; con escasas probabilidades de metástasis pero con alto índice de recurrencia local. El diagnóstico debe sospecharse y confirmarse con histología e inmunohistoquímica. El tratamiento de elección es con cirugía convencional y/o cirugía micrográfica de Mohs, con márgenes de 2-4 cm. Se considera que la prevalencia del DFSP en la edad pediátrica es baja, debido al escaso índice de sospecha. En el presente trabajo compartimos cinco casos de DFSP en la edad pediátrica, estudiados en el Hospital General de Niños Pedro de Elizalde. (AU)

Dermatofibrosarcoma protuberans (DFSP) is a cutaneous, low frequency, fibrohistiocytic, infiltrating, slow growing, local aggressiveness, intermediate malignancy tumor; with little chance of metastasis but with a high rate of local recurrence. The diagnosis should be suspected and confirmed with histology and immunohistochemistry. The treatment of choice is with conventional surgery and / or Mohs micrographic surgery, with margins of 2-4 cm. The prevalence of DFSP in pediatric age is considered to be low, due to the low index of suspicion. In this paper we share five cases of DFSP in the pediatric age, studied at the Pedro de Elizalde Children's General Hospital. (AU)

Cellular Plexiform Fibrohistiocytic Tumor / 대한피부과학회지

Plexiform fibrohistiocytic tumor (PFHT) is a rare mesenchymal neoplasm of intermediate malignancy and possibly of myofibroblast origin. It is morphologically divided into 3 groups: cellular, fibrous, and mixed. A 4-year-old girl presented with an irregular shaped subcutaneous mass on her left popliteal fossa for 6 months. The biopsy specimen showed multinodular tumor islands extending from dermis to subcutaneous layer, composed of histiocytes and osteoclast-like multinucleated giant cells, and circumscribed by fibrous tissue. Immunohistochemical staining was positive for CD68 in giant cells and histiocyte-like cells within tumor islands and faintly positive for smooth muscle actin around nodules. A cellular variant of PFHT was eventually diagnosed. Although PFHT comprises morphologically normal cells, it has the biological potential for malignant change and distal metastasis. Therefore, PFHT is categorized as a neoplasm of intermediate malignancy, and wide total excision with close follow-up is crucial.

Paediatric Primary Pachymeningeal Xanthogranuloma with Scattered Foci Displaying Reticulohistiocytoma-like Features

We report a unique case of a 4-year-old girl with an intriguing fibrohistiocytic tumour. Magnetic resonance imaging scans showed a dural mass of variegated intensity compressing the left occipital pole and apparently extending toward the superior sagittal sinus. Grossly, the cut surface of the surgical specimen was yellow, pale, and soft with reddish kernel-like crusts. Histologically, the yellow areas resembled cholesterol granulomas with widespread coagulative necrosis, cholesterol clefts, powdery calcification, foreign body-type giant cells, and foamy macrophages, while the scattered red spots contained numerous multinucleated giant cells of foreign-body and Touton types, the former with amphophilic to slightly eosinophilic cytoplasm. Immunoperoxidase reactions confirmed the expression of histiocytic markers and vimentin. As far as we know, no tumour displaying these peculiar morphological features has yet been described.

Congenital Juvenile Xanthogranuloma of Foot, a Nodular Lesion: An Unusual Case in 2-month-old Infant.

A 2-month-old infant presented with a circumscribed nodule on left foot since birth. Excision biopsy showed juvenile xanthogranuloma, an uncommon diagnosis in an unusual site; common sites being head and neck. Uncommon sites are groin, genital organs, limbs and even internal organs. It carries a favorable prognosis.

Tumor de partes blandas de bajo potencial maligno: caso clínico y revisión de la literatura / Soft tissue tumor of low potential malignancy: clinic case and review of literature

El tumor de células gigantes de partes blandas de bajo potencial de maligno, es una neoplasia poco frecuente, clasifica dentro de las lesiones fibrohistiocíticas. Histológicamente es un tumor con hallazgos idénticos al tumor de células gigantes del hueso. Presentamos un caso correspondiente a esta neoplasia. Se trató de paciente de 26 años con una lesión tumoral 20 cm x15 cm x15 cm, en ambas regiones lumbares y región sacra. El estudio histológico reveló una neoplasia con abundantes células gigantes, células fusiformes, hemosiderina y hueso metaplásico. Los estudios inmunohistoquímicos demostraron fuerte positividad de CD68 para las células osteoclásticas. La evolución del paciente fue favorable, sin evidencia de recidivas. Es imprescindible realizar el diagnóstico diferencial de este tumor con otras neoplasias con abundantes células gigantes, como el tumor de células gigantes de la vaina tendinosa y el fibrohistiocitoma maligno rico en células gigantes, el cual es un sarcoma de alto grado

The giant cell tumor of soft tissues of low potential malignancy is a very rare tumor. It’s classified in fibrohistiocytic neoplasm and has features identical to giant cell tumor of bone. We present a clinical case for this less frequent malignancy. Patient 26 years old man with a tumor of 20 cm x 15 cm x 15 cm, in both lumbar and the sacral regions. Histological examination revealed a neoplasm with abundant giant cells, spindle cells, hemosiderin and metaplastic bone. The immunohistochemistry studies practice showed strong positivity of CD68 for the osteoclastic cells. The patient outcome was favorable, without evidence of recurrence. It is essential to make the differential diagnosis of this kind of tumor with other neoplasm with abundant giant cells, such as the giant cell tumor of the tendon sheath and malignant giant cell, also malignant fibrous hystiocitoma, which is rich in giant cells and high grade sarcoma

Clinical Study on Malignant Fibrous/Fibrohistiocytic Tumors / 대한피부과학회지

BACKGROUND: Malignant fibrous/fibrohistiocytic tumors are uncommon soft tissue tumors which gives dermatologists special attention on differential diagnosis. However, there has not yet been a multicenter study on these tumors in Korea. OBJECTIVE: The aim of this study was to investigate the epidemiology and clinical features of malignant fibrous/ fiborhistiocytic tumors. METHODS: A total of 62 patients from 11 training hospitals who had been confirmed with malignant fibrous/ fibrohistiocytic tumor were studied. A retrospective analysis of hospital records served as the data source for this study. RESULTS: Among patients with malignant fibrous/fibrohistiocytic tumors, the most common tumor type was dermatofibrosarcoma protuberance followed by malignant fibrohistiocytic. The male to female ratio among subjects was 1.38 to 1 and the mean age was 44 years old. Of the common complaints recorded, asymptomatic mass was the most frequent. The mean size of the subjects' tumors was 2.9 cm in the long axis and 2.3 cm in the short axis with a mean tumor thickness of 2.1 mm. The most common site for tumors was the back followed by the thigh. The recurrence rate after primary treatment was 14.5% and metastasis developed in 5 of 62 patients. CONCLUSION: This study is expected to be helpful for understanding the clinical and pathological characteristics of malignant fibrous/fibrohistiocytic tumors.

Fibrohistiocitoma de laringe / Laryngeal fibrohistiocytic tumor

Presentamos el caso de un paciente varón de 49 años, portador de fibriohistiocitoma maligno de la laringe. Hacemos un análisis de su presentación clínica, la terapéutica y su evolución. Se revisó la literatura, donde se encuentra unos pocos casos publicados hasta la fecha en esa localización.

We report the case of a 49-year-old male patient, with a laringeal malignant fibrohistiocitoma. We found a few cases reported in this localization.

Nódulos fibro-histiocíticos no baço de uma cadela: relato de caso / Splenic fibroistiocytic nodules in a bitch: case report

Descreve-se a ocorrência de múltiplos nódulos fibro-histiocíticos no baço de uma cadela Rottweiler, de sete anos. O diagnóstico foi feito por histopatologia e confirmado por imunoistoquímica positiva para ED1, CD18, vimentina e lisozima. O animal foi sacrificado três meses após o diagnóstico, por severo agravamento dos sinais clínicos.

Multiple fibrohistiocytic nodules in the spleen of a 7-year-old female Rottweiler were reported. The diagnosis was made by histology and immunohistochemistry; cells were positive for ED1, CD18, vimentin and lysozyme. The dog was euthanatized three months after the tumor was diagnosed, due to severe clinical signs.

Plexiform Fibrohistiocytic Tumor of the Neck: A Case Report

Plexiform fibrohistiocytic tumor (PFT) is a rare, low-grade soft tissue tumor that occurrs primarily in children and young adults. The most common location of PFT is the upper extremity, and there are very few reports of PFT in the neck. We report here on a case of PFT presenting as a painless subcutaneous nodule in the neck of a 46-year-old woman. Histologically, this subcutaneous tumor was composed of a plexiform proliferation of histiocyte-like cells and fibroblast-like cells along with a few multinucleated osteoclast-like giant cells. Immunohistochemically, the tumor cells were positive for vimentin, CD68 and smooth muscle actin (SMA).

A Case of Myxoid Plexiform Fibrohistiocytic Tumor / 대한피부과학회지

A plexiform fibrohistiocytic tumor is a low-grade malignant mesenchymal neoplasm of myofibroblastic origin, with the capacity for biphasic differentiation toward a fibroblastic or histiocyte-like morphology. We report a case of myxoid plexiform fibrohistiocytic tumor occurring on the left upper arm in a 34-year old man. The lesion was a yellowish-to-pinkish color, rounded, pedunculated tumor with a short pedicle, measuring 1x1x1.5cm. Histopathological examination revealed a multinodular biphasic proliferation of fibroblast- like and histiocyte-like cells with a few osteoclast-like giant cells. This case is notable for the rare myxoid changes. To the best of our knowledge, this is the first report of a plexiform fibrohistiocytic tumor in the Korean dermatological journals.

Metastatic Adenocarcinoma Histologically Mimicking Fibrohistiocytic Tumors

Cutaneous metastasis from visceral malignancies shows diverse manifestations. Very rarely, cutaneous metastasis mimicks benign mesenchymal tumors. We describe a case in which metastasis from gastric adenocarcinoma mimicked fibrohistiocytic tumors. The diagnosis of cutaneous metastasis was confirmed by the presence of atypical cells and signet-ring cells, positive staining for mucin and positive immunohistochemical stainings for keratins, carcinoembryonic antigen(CEA), and lysozyme within the tumor cells infiltrated in the irregular, intertwining bundles of collagen in the reticular dermis.

Immunohistochemical Study of Fibrohistiocytic Tumors of the Skin

BACKGROUND: Histologic distinction between various fibrohistiocytic tumors of the skin may sometimes be difficult. Recently, several immunohistochemical markers of "histiocytes and "facultative fibroblasts" have been introduced and used for the study of some fibrohistiocytic tumors of the skin. OBJECTIVE: The purpose of this study is to determine whether immunostatining with MAC 387, antibodies to S-100 protein, factor Xllla(FXllla) and CD 34 allows distinction between various fibrohistiocytic tumors of the skin in formalin-fixed, paraffin-embedded specimens. METHODS: Paraffin-embedded specimens of dermatofibroma, keloid, hypertrophic scar, dermatofibrosarcoma protuberans(DFSP), neurofibroma, and juvenlie xanthogranuloma were investigated with MAC 387, antibodies to S-100 protein, CD 34 and FXllla using avidin-biotin -peroxidase complex method. RESULTS: In all fibrous dermatofibromas (n=26), 20-90% of constituent cells were positive for FXllla. Focal or diffuse CD 34 reactivity was present in DFSP (n=2). Weak reactivity for CD 34 and consistent labeling for S-100 protein were found in neurofibromas (n=5). Tumor cells showed negative for FXllla, CD 34 and S-100 protein in keloids (n=2), hypertrophic scars (n=6), and juvenile xanthogranulomas (n=5). MAC 387 did not label tumor cells of the fibrobistiocytic tumors we have studied. CONCLUSION: Immunostaining of paraffin-embedded specimens with antibodies to S-100 protein, FXllla and CD 34 may be useful in the differential diagnoses of fibrohistiocytic tumors of the skin.

The Study of Proliferating Cell Nuclear Antigen (PCNA) Reactivity in Fibrohistiocytic Tumors

Fibrohistiocytic tumors are a diverse group of benign and malignant soft tissue lesions, including dermatofibroma, dermatofibrosarcomaprotuberans, and malignant fibrous histiocytoma. On the clinical point of view, the distinction between benign and malignant lesions and malignancy grading is far more important. Therefore, we investigated 23 fibrohistiocytic tumors, using PCNA (PC10) which was a useful marker of proliferating activity, to differentiate the benign lesions from the malignant and correlate with other prognostic factors including tumor necrosis. cellularity, histologic grade, and mitotic counts. The results obtained were as follows 1) Positive tumor cells were clearly identified by the characteristic diffuse or granular nuclear staining. 2) The number of PCNA-positive tumor cells were 2.16+/-2.39% in dermatofibroma, 16.12+/-7.38% in dermatofibrosacoma protuberans, and 28.02+/-17.47% in the malignant fibrous histiocytoma. The numbers of PCNA-positive tumor cells in the malignant lesions higher than in the benign (p5 cm) and recurred or metastatic cases of MFH were more the high PCNA index (more than 20%) than the low index (less than 20%) groups. 4) PCNA index in MFHs had positive correlation with the number of mitotic counts (r=0.7582, p<0.001), cellularity (r=0.5908, p<0.05) and histologic grade (r=0.4164, p<0.05). These results suggested that reactivity on PCNA might assist in the distinction between benign and malignant lesions in fibrohistiocytic tumors, and could be a useful prognostic factor in the patients with malignant fibrous histiocytoma.