Introduction: Primary cutaneous sarcomas are rare mesenchymal cancers with histopathological heterogeneity. Angiosarcomas are an uncommon cutaneous malignant sarcoma sub-type that effect mainly elderly male patients.Their location is primarily cephalic, and they present as multifocal lesions which lead to local edema, bleeding and ulceration. Aggressiveness, fast growth and undefined surgical margins explain the high rates of recurrence.We describe a case of primary angiosarcoma of the scalp in an elderly female patient, who was being treated with a multidrug therapy for lepromatous leprosy.The combination of angiosarcoma and leprosy is uncommon, and the absence of similar reports in the literature motivated the description of this case.

Keywords: SKIN NEOPLASMS, SARCOMA, LEPROSY, LEPROMATOUS

Dermatofibrosarcoma protuberans is a spindle cell neoplasm of intermediate malignancy, nonetheless it presents a high risk of local recurrence. It mainly affects the trunk and limbs, and is rare on the face. The treatment of the tumor can be carried out using conventional surgery with wide surgical margins or Mohs micrographic surgery. Radiation and chemotherapy can be indicated in selected cases. The present article reports the case of a 39-year-old male patient with a 0.7cm yellowish papule on the forehead, whose biopsy and immunohistochemistry confirmed the tumor's diagnosis. The lesion was treated through Mohs micrographic surgery with good aesthetic results.

Keywords: DERMATOFIBROSARCOMA; SARCOMA; MOHS SURGERY

Dermatofibrosarcoma protuberans is an uncommon and relapsing fibrohistiocytic tumor of intermediate malignancy. Its rarest variant is known as atrophic, morphea type or dermatofibrosarcoma non protuberans and its clinical diagnose is difficult. There are not well established dermatoscopic criteria, so the diagnosis is histopathologic. There are advances in immunohistochemical and genetic that help the diagnosis and complete surgical excision. It is reported a case of atrophic dermatofibrosarcoma in a 27 years patient ant it is reviewed the clinical, dermoscopic, histological and genetic aspects.

Keywords: DERMOSCOPY; NEOPLASMS; DERMATOFIBROSARCOMA

Kaposi's sarcoma is a rare multicentric neoplasm originating from endothelial cells, with cutaneous and extracutaneous manifestation. The present paper describes a case of an uncommon clinic variant of a Kaposi’s sarcoma in a 32 year-old male patient bearer of acquired immunodeficiency syndrome (AIDS), with regression after undergoing combined treatment with antiretroviral therapy and radiotherapy.

Keywords: sarcoma, kaposi; herpesvirus 8, human; acquired immunodeficiency syndrome; keloid

Angiosarcoma is a rare and aggressive vascular neoplasia that can occur anywhere in the skin integument, especially in the face and scalp. It has a branched character, and can clinically arise as a macula, papule, plaque or nodule of erythematous hue, with imprecise limits and expansive growth, and diagnosis based on the clinical-pathological correlation. In the present study, the authors report the case of a patient with exuberant cutaneous angiosarcoma on the face, where surgical resection was performed as the first therapeutic option.

Keywords: Dermatology; Hemangiosarcoma; Sarcoma; Vascular neoplasms

INTRODUCTION: Dermatofibrosarcoma protuberans (DFSP) is a local aggressive sarcoma that presents 60% of recurrences. Rarely it presents lung metastasis too.
METHODS: 41 years old man presented a soft tumor in his right hallux for two years. Histopathology has shown spindle-shaped cells arranged in a storiform manner, and immunohistochemistry was CD34 positive.
DISCUSSION: The literature describes some clinical types of DFSP. The most common are: confluent nodules forming a plaque, sometimes similar to keloids, tumoral lesions, and atrophic plaques.
CONCLUSION: DFSP cases simulating a subcutaneous cyst have been described in the literature; nevertheless, it is an unusual presentation of this tumor.

Keywords: Dermatofibrosarcoma; Sarcoma; Surgical Procedures, Minor

Dermatofibrosarcoma protuberans is a locally aggressive sarcoma of intermediate malignancy that predominates in the range of young adults to middle age. Congenital or childhood lesions are rare but have been reported. It can be misdiagnosed and incompletely excised in the early stages, which increases the risk of recurrence. The present article reports the case of a 24-year-old male patient presenting a hyperchromic macula in the abdominal region from birth that evolved into a tumor after several surgical approaches. Biopsy and immunohistochemistry confirmed the diagnosis of dermatofibrosarcoma protuberans, and the lesion was excised with a 3 cm margin.

Keywords: Dermatofibrosarcoma; Neoplasms, Fibrous Tissue; Sarcoma

Undifferentiated sarcomas are neoplasms of soft tissues derived from the mesoderm. We report a case of undifferentiated pleomorphic sarcoma, its dermoscopy, staging, and therapeutic approach.

Keywords: Dermoscopy; Sarcoma; Soft Tissue Neoplasms

O queratoacantoma (QA), apesar de frequente na prática diária, é um tumor que gera questionamentos. O aspecto mais intrigante associa-se à sua posição no limite entre malignidade e benignidade. A abordagem do tumor, bem como sua classificação, é controversa. Na definição da conduta, é importante levar em conta o potencial de transformação para carcinoma espinocelular. Desse modo, a excisão cirúrgica é a terapia de escolha, sempre que possível. Algumas situações merecem atenção adicional, como a imunossupressão associada. Neste artigo, relatamos um caso de QA gigante em um paciente com síndrome da imunodeficiência adquirida tratado com cirurgia micrográfica de Mohs.

Keywords: Ceratoacantoma; Retalhos Cirúrgicos; Neoplasias Nasais; Neoplasias Cutâneas; Cirurgia de Mohs; Síndrome de Imunodeficiência Adquirida; HIV; Imunossupressão

Malignant fibrous histiocytoma (MFH) or undifferentiated pleomorphic sarcoma (UPS) is a sarcoma capable of invading adjacent structures. It is a mesenchymal neoplasia that predominates in men between the sixth and seventh decades of life. It is located mainly in the lower limbs and may affect the head and neck, trunk, and retroperitoneum, presenting a tendency to recurrence and local metastasis. This report aims to present a case of MFH in the ankle of a 49-year-old woman with an adjacent bone invasion, which evolved with transtibial amputation. Clinical, radiological, histopathological, and therapeutic aspects were addressed, highlighting the importance of early diagnosis.

Keywords: Amputation; Malignant fibrous histiocytoma; Sarcoma

Low-grade fibromyxoid sarcoma (LGFS) is a rare, indolent spindle cell sarcoma with a risk of recurrence and metastasis. We report the case of a 34-year-old man with a tumor in his thumb that had recurred after excision eight years ago, with slow and progressive growth. The pathological examination was suggestive of SFBG, and we proceeded with the disarticulation of the phalanx. The patient remains in follow-up, with no evidence of metastases. Although rare, it is essential to recognize the diagnosis of an LGFS with its potential risk of recurrences and metastases, especially as a differential diagnosis of acral soft tissue tumors.

Keywords: Dermatology; Neoplasms; Sarcoma

Extraskeletal Ewing sarcoma (EES) is a rare tumor that affects men between 10-20 years old and represents 25% of Ewing sarcomas. We report a case due to the previous diagnosis of a fast-growing lipoma to highlight the importance of histopathological diagnosis. A 14-year-old boy presented 10x10 cm tumor in the right shoulder for a year with ultrasonography suggesting lipoma. The lesion was excised and EES was confirmed by immunohistochemical analysis. EES diagnosis in young people is challenging compared to subcutaneous tumors, and histopathology is essential. The rapid progression of the tumor and high metastatic rates highlight the significance of early treatment.

Keywords: Sarcoma, Ewing; Neuroectodermal tumors; Sarcoma

Fibromyxosarcoma is a rare subtype of soft tissue sarcoma characterized by the presence of spindle cells and myxoid matrix. We report the case of an older patient who presented with progressive growth of a nodule in the interscapular region, and further investigation confirmed the diagnosis of high-grade fibromyxosarcoma. Notably, this neoplasm more commonly occurs in the lower limbs and may exhibit recurrence associated with increased histological grade. Therefore, early diagnosis and treatment are crucial for mitigating morbidity and mortality. We emphasize the significance of diagnosing an atypical case and providing timely therapeutic intervention, contributing to the optimization of clinical outcomes.

Keywords: Sarcoma; Neoplasms, Connective and Soft Tissue; Solitary Fibrous Tumors