Dermatofibrosarcoma Protuberans Occurring in an Adult Presenting with Chronic Lymphedema: A Case Study and Literature Review.

BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a rare soft-tissue tumor typically located in the trunk. We report a unique case of DFSP in the right inguinal region of an adult presenting with chronic lymphedema. Only 1 case of DFSP and chronic lymphedema association has been previously reported in the literature. Since we could not provide adjuvant radiotherapy (RT), we conducted an extensive review of its application in similar cases, exploring various surgical treatments. CASE REPORT A 42-year-old Cameroonian man with unexplained chronic lymphedema presented with a tumor in the inguinal region of the affected limb. The patient underwent wide local excision (WLE) of the mass, including regional lymph node dissection. Pathological exam confirmed DFSP with a fibrosarcomatous component. Adjuvant RT was considered but not pursued due to the patient;s non-compliance. CONCLUSIONS This DFSP is reported for its rarity of site and the unique co-occurrence with chronic lymphedema. Considering both conditions are uncommon and the rarity of site of the DFSP, we assume that in this patient, chronic lymphedema was a contributing factor of occurrence of the DFSP. Remarkably, no prior reports have detailed an association between chronic lymphedema and DFSP onset. For that reason, we want to point out the value of better follow-up of chronic lymphedema and better knowledge of DFSP treatment options to improve patient healthcare and limit DFSP recurrence. In addition, we found adjuvant RT is an interesting treatment option that might be considered in all patients undergoing surgical excision, even in cases where negative surgical margins were achieved.

Cervical Erector Spinae Plane Block for Giant Dermatofibrosarcoma Protuberans of Neck and Chest Wall: A Case Report.

We encountered a giant dermatofibrosarcoma protuberans (DFSP) of the neck and chest wall which presented a challenge in terms of perioperative analgesia management. In recent years, erector spinae plane (ESP) block has emerged as an effective and safe analgesia technique for various surgical procedures as well as for chronic neuropathic pain without any untoward complications. A continuous lower cervical ESP block can be used successfully as an effective analgesic technique for extensive DFSP surgery involving the neck and chest wall area.

Melanotic Macule in Conjunction with a Giant Cell Fibroma.

INTRODUCTION: The aim of this study is to describe a case of a melanotic macule found in conjunction with a giant cell fibroma (GCF). For oral pigmented lesions without an identifiable etiologic factor, critical factors in determining the differential diagnosis are clinical history, symmetry, and uniformity of the lesions. Potential differential diagnosis includes racial pigmentation, endocrine disturbance, Peutz-Jeghers syndrome, trauma, hemochromatosis, oral malignant melanoma, or idiopathic etiology and melanotic macules. Melanotic macules are the most common solitary pigmented melanocytic lesions in the oral mucosa, corresponding to 86.1% of melanocytic lesions of the mouth. Giant cell fibromas are reactive connective tissue lesions in the oral cavity. They were first described as a distinct entity in 1974 by Weathers and Callihan and make up around 5 to 10% of all oral mucosa fibrous lesions. They are commonly mistaken for other growths, such as pyogenic granuloma and fibroma, and diagnosis is accurately based on its distinctive histopathology. This article presents the clinicopathologic findings of a 15-year-old Hispanic male presenting for biopsy of a melanotic macule on the mandibular anterior buccal gingiva. Histologic evaluation of the specimen revealed that the lesion also contained a GCF. Pathologic lesions of the mouth should be carefully diagnosed. Conventionally, histologic evaluation is the gold standard to produce a final diagnosis. As evidenced in this article, multiple lesions may exist in a site and may be mistakenly diagnosed as a single entity. CLINICAL SIGNIFICANCE: While each lesion has been reported individually, in reviewing the literature, no cases were reported in which both histopathologic findings of GCF and melanotic macule were present within the same lesion.

Oncocytoma as a growing FDG-avid adrenal mass on serial 18F-FDG PET/CT imaging: a potential pitfall in oncological evaluation / Masa suprarrenal captante de FDG que incrementa de tamaño en 18F-FDG PET/TC de seguimiento correspondiente a Oncocitoma: un posible pitfall en la evaluación oncológica

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Tumor de Bednar (dermatofibrosarcoma protuberans pigmentado) / Bednar Tumor (Pigmented Dermatofibrosarcoma Protuberans)

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Dermatofibrosarcoma Protuberans in a Patient With Cowden Syndrome: Revisiting the PTEN and PDGF Pathways.

PTEN hamartoma tumor syndrome, of which Cowden syndrome (CS) is the most recognized variant, is characterized by multiple benign and malignant tumors of ectodermal, mesodermal, and endodermal origins, secondary to germline mutation in the phosphatase and tensin homolog (PTEN) gene. Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive malignant fibroblastic/myofibroblastic tumor of the skin, characterized by the t(17:22)(q22:q13) translocation resulting in fusion of the COL1A1 and PDGFB genes. An association between CS and DFSP has not been reported in the literature to date. The authors have encountered a male patient with CS and a history of DFSP that developed adjacent to a sclerotic fibroma on the parietal scalp, both excised at age 7. He presented at age 21 with an enlarging pink nodule at the same site on the parietal scalp. Excision revealed a dermal and subcutaneous storiform spindle cell proliferation with fat entrapment and positive staining for CD34, consistent with DFSP. Fluorescence in situ hybridization confirmed PDGFB gene rearrangement. PTEN expression in the patient's recurrent DFSP was nearly absent when compared with that of sporadic DFSP. To our knowledge, this is the first report of DFSP in a patient with CS. Although the association is likely to be coincidental, the authors revisited the PTEN and the PDGF pathways to speculate any possible interplay of the 2 conditions on a molecular level.

Dermatofibrosarcoma protuberans: série de 27 casos consecutivos / Dermatofibrosarcoma protuberans: a single series of 27 consecutive cases

INTRODUÇÃO: A dermatofibrosarcoma protuberans (DFSP) é um tumor maligno de baixo grau de partes moles caracterizado por infiltração local agressiva e propenso a recidiva local. Este estudo retrospectivo analisou resultados clínicos, taxas de recidiva e sobrevida após tratamento cirúrgico de DFSP. MÉTODOS: Pacientes submetidos a cirurgia para erradicação completa do tumor, e subsequente seguimento por exame clínico e vigilância ultrassonográfica de locais primários do tumor e linfonodos correspondentes para detectar recidiva local ou distante. A cirurgia, invariavelmente, incluiu grande excisão do tumor, seguida por procedimentos de reconstrução diferente como enxerto de pele em 23 casos (85%), retalho local em 2 pacientes (7.4%), retalho livre em 1 caso (4%), enquanto a principal sutura foi realizada apenas em um caso (4%). RESULTADOS: Foi necessária segunda cirurgia em 9 casos (33%) para atingir margens livres mínimas de 2-3 cm. Outros tratamentos cirúrgicos foram utilizados, como cirurgia micrográfica de Mohs, ou terapias adjuvantes, como radioterapia e quimioterapia. Em nossa série de pacientes o intervalo livre de recidiva variou de 1 a 10 anos, com média de 6 anos. A recidiva local ocorreu em 3 pacientes (11%), e necessitou de outras excisões cirúrgicas extensas. Um total de 27 pacientes não desenvolveu metástase distante durante o seguimento. CONCLUSÃO: A excisão extensa é efetiva para disponibilizar local confiável para controlar a doença, porém somente se limitadas por margens livres de 2-3 cm e confirmadas por patologista. A vigilância clinica e ultrassonografia durante o seguimento permite identificação precoce de eventuais recidivas locais e envolvimento de linfonodos.

INTRODUCTION: Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade malignant tumor of soft tissues characterized by aggressive local infiltration and propensity for local recurrence. This retrospective study analyzed clinical outcomes, recurrence and survival rates after surgical treatment of DFSP. METHODS: Patients underwent surgery to complete eradicate tumor, and subsequently a close follow-up by clinical examination and ultrasounds surveillance of primary tumor site and corresponding lymph nodes, to detect local or distant recurrence. Surgery invariably included wide excision of tumor, followed by different reconstructive procedures as skin grafting in 23 cases (85%), local flap in 2 patients (7.4%), free flap in 1 case (4%), while primary closure was performed only in one case (4%). RESULTS: Second surgery was needed in 9 cases (33%) to achieve minimum free-margins of 2-3cm. Other surgical treatments like Mohs Micrographic Surgery, or adjuvant therapies, like radio- or chemotherapy were not used. Free-recurrence lapse among this series of patients varied from 1 to 10 years, with a medium period of 6 years. Local recurrence occurred in 3 patients (11%), and required a further extended surgical excision. A total of 27 patients did not develop distance metastasis during the follow-up. CONCLUSION: Extended excision is effective to provide a reliable local control of disease, but only if free margins limiting 2-3 cm is confirmed by pathologist. Clinical and ultrasound surveillance during close follow-up provide early detection of eventual local recurrence and of lymph nodes involvement.

Unusual bleeding of a giant cell fibroblastoma: a soft tissue sarcoma of the skin mimicking metastatic melanoma.

A 56 year-old man presented to the emergency department after a spontaneous bleeding of a giant mass located on the right axilla. Clinical diagnosis was recurrent hemorrhagic nodular melanoma. Ten months previously a malignant melanoma had been removed from the dorsum by radical excision and surgical margins had been disease-free (MM: Breslow IV, Clark IV, lung and lynphnode metastases). The patient required immediate emergency surgical intervention to prevent death by hemorrhagic shock. The tumor was bleeding and the patient required a transfusion. Subjective symptoms included pain in palpation and spontaneous hemorrhage, poor general appearance, pale skin, BP 80/40 mmHg, HR 100/min with overall symptoms of hypovolemic shock. At the time of surgery, radical tumor excision was performed with an approximately 3 cm circumferential gross tumor free margin. The resultant defect was reconstructed by pectoral rotation fascio-cutaneous flap. The histological diagnosis demonstrated an undifferentiated high-grade pleomorphic sarcoma with microscopic tumor free margins.

Unusual presentation of dermatofibrosarcoma protuberans in a male patient's breast: a case report and review of the literature.

We herein present a case report and literature review of dermatofibrosarcoma protuberans in the breast of a male patient. A 27-year-old man presented with a painless lump in his right breast with areas of bluish skin discoloration. The diagnostic work-up comprised clinical examination, ultrasonography, core biopsy, mammography, and magnetic resonance imaging. After surgical excision, the preoperative diagnosis of dermatofibrosarcoma protuberans was proven by pathological examination and immunohistochemistry. The patient was still free of recurrence 1 year after surgical excision. This extremely rare case is, to the best of our knowledge, the fifth such case reported in the literature.

Giant Hemosiderotic Dermatofibroma: The Largest Giant Dermatofibroma Reported to Date.

Dermatofibroma (DF) is a relatively common benign fibrohistiocytic soft tissue tumor. It has a slightly greater incidence amongst females and typically presents itself during the midadult life as a slowly growing, firm dermal nodule, usually smaller than 2 cm in diameter, on the lower extremities. Giant DF is a rare clinical variant of DF characterized by unusually large size (>5 cm), which mimics malignant soft tissue tumor clinically. Twenty-six cases of giant DF have been reported so far. One of these giant DFs was a giant hemosiderotic DF. We report herein a case of a 47-year-old woman who presented with the largest DF reported in the literature to date. It was hemosiderotic.

Coexistence of giant cell fibroblastoma and encephalocele.

Giant cell fibroblastoma (GCF) is a rare soft tissue tumour that occurs almost exclusively in children younger than 10 years of age and is mostly located in the superficial soft tissues of the back and thighs. We present a rare case of GCF with encephalocele in a 1.5-year-old boy who presented with a swelling in the occipital area of the scalp since birth. CT scan suggested encephalocele without any suspicion of a mass lesion. On histopathology, an ill-defined proliferation of fibroblasts in a heavily collagenised and focally myxoid stroma was seen containing numerous multinucleated cells having a floret-like appearance along with mature glial tissue bordering a cystic space. Immunohistochemically, the stromal cells were positive for both, vimentin (diffuse) and CD34 (focal) thereby confirming the histological diagnosis of GCF. This case highlights the unusual coexistence of GCF with congenital defects and its histogenetic resemblance to dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans on the right neck with superior vena cava syndrome: case report and literature review.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon dermal soft tissue tumour of intermediate malignancy. A 44-year-old man presented to the hospital with a large lesion on the right upper chest and neck. Despite eight previous surgical excisions, the tumour had continued to recur. Contrast-enhanced computed tomography showed recurrence of the tumour, associated with superior vena cava (SVC) syndrome. He declined radical surgical resection of the recurrent tumour, which may have required right upper limb amputation. Targeted therapy with sunitinib malate was therefore introduced. This case demonstrates the recurrent nature of DFSP and the association of this lesion on the upper chest/neck with SVC syndrome. Primary wide radical resection is essential for better local control and to avoid the development of SVC syndrome.

Dermatofibrosarcoma protuberans / Dermatofibrosarcoma Protuberans

El dermatofibrosarcoma protuberans es el sarcoma de piel más frecuente aunque su incidencia es muy baja comparada con otros tumores cutáneos. Se presenta clínicamente en forma de placa indurada de crecimiento lento sobre la que aparecen nódulos a medida que el tumor progresa. Se localiza inicialmente en la dermis desde donde infiltra el tejido celular subcutáneo, la fascia, el músculo e incluso el hueso. La translocación COL1A1-PDGFB es específica del dermatofibrosarcoma protuberans y sirve de ayuda en el diagnóstico de determinados casos. Según la revisión de las series publicadas en la literatura, el porcentaje de recidivas con cirugía micrográfica de Mohs es mucho menor que el encontrado cuando se emplea cirugía convencional con márgenes amplios. Para casos metastásicos o en aquellos donde la cirugía pueda ser mutilante se dispone recientemente del imatinib, fármaco de la familia de los inhibitores de la tirosina quinasa (AU)

Dermatofibrosarcoma protuberans is the most common skin sarcoma, although its incidence is very low compared with other skin tumors. It presents as a slow-growing indurated plaque on which nodules develop over time. The lesion arises in the dermis but can invade subcutaneous tissue, fascia, muscle and even bone. COL1A1-PDGFB translocation is specific to dermatofibrosarcoma protuberans, and the presence of this fusion contributes to diagnosis in certain cases. A review of the literature provides evidence that recurrence is much lower after Mohs micorgraphic surgery than after conventional wide local excision. In the case of metastatic disease or when surgery would be mutilating, another recently approved treatment is the tyrosine kinase inhibitor imatinib (AU)