Undifferentiated pleomorphic sarcoma of skin in unusual locations: Report of two cases.

Undifferentiated pleomorphic sarcoma (UPS) of the skin is a rare soft tissue sarcoma subtype with a high risk of metastasis and local recurrence. Ultraviolet exposure plays a prominent role in its etiology. Herein, we present two rare cases of UPS of the skin with an occurrence in non-ultraviolet exposed locations and discuss the need of wide resection and the identification of the depth of the tumor. Due to the resection of the skin, resulting defect was undertaken with skin grafting. One case was extending to the fascia and, after the resection, vacuum-assisted closure therapy was used for the formation of granulation tissue. No recurrence was seen after three and four years for either cases. In conclusion, UPS is a rare, aggressive malignant tumor with a high local recurrence and metastasis rate. Suspicion of malignancy in a plaque-like or nodular lesion in the skin is crucial, and local recurrence and metastasis risk is significantly correlated to the success of wide excision.

Preoperative Evaluation of Myxofibrosarcoma: Prognostic Value and Reproducibility of Different Features on MRI.

BACKGROUND/AIM: Myxofibrosarcoma (MFS) is characterized by an infiltrative growth pattern. This study aimed to determine the correlation between overall survival (OS) and morphological features of MFS as well as examine the reproducibility of these findings on preoperative magnetic resonance imaging (MRI). PATIENTS AND METHODS: Fifty-eight MFS patients underwent preoperative MR imaging with the following features analysed: i) tumour size, ii) localization, iii) margins, iv) morphology, v) signal characteristics, vi) contrast enhancement, vii) presence and extent of perilesional oedema, and viii) presence of the tail sign. RESULTS: Only circumscribed perilesional oedema was associated with a significantly better survival compared to diffuse oedema (p=0.010), which was found in the majority of cases. The tail sign was found in less than 50% of the cases. Cohen's kappa coefficients confirmed a relatively high interrater variability. CONCLUSION: Perilesional diffuse oedema on MR imaging of MFS is significantly correlated with a poor overall survival. The interrater variability in interpretation of MR examinations varies from slight to substantial agreement. Preoperative MR imaging with detailed planning of the resection seem to be a logical approach to achieve negative resection margins and recurrence-free survival.

Clinico-epidemiological profile of breast cancer patients and the retrospective application of Gail model 2: An Indian perspective.

AIM: To study the clinical and epidemiological profile of patients of breast cancer presenting at our center at New Delhi, India and to evaluate the applicability of Gail model 2 as a means of measuring 5-year and lifetime risk in our already diagnosed cases of breast cancer. METHODS: This was a retrospective study conducted at Lady Hardinge Medical College Hospital in New Delhi, India, between January 2011 and July 2014. Two hundred and twenty two diagnosed cases of breast cancer were included. Information was collected retrospectively on a Performa from the medical record section and the Pathology department of the hospital.The predicted five-year and lifetime risk was calculated using GM2 prediction model from the NCI's breast cancer risk assessment tool website. RESULTS AND CONCLUSIONS: Breast cancer in India is a far more biologically aggressive disease than in the west with a widely different spectrum of presentation and behavior and late presentation in an advanced stage. The accepted risk factors routinely associated with breast cancer in western literature do not appear to be relevant in the Indian population. Accepted western models do not seem to apply in the Indian scenario.

Activation of the Akt/mammalian target of rapamycin pathway in myxofibrosarcomas.

The Akt/mammalian target of rapamycin (mTOR) pathway plays important roles in modulating cellular function in response to extracellular signals such as growth factors and cytokines. The Akt/mTOR signaling pathway is activated in certain kinds of sarcomas. Myxofibrosarcoma is a soft tissue sarcoma, characterized by abundant myxoid stroma and frequent local recurrence. Here, we conducted a large-scale examination of the clinicopathological and activation statuses of the Akt/mTOR pathways in myxofibrosarcoma. The phosphorylation status of Akt, mTOR, S6 ribosomal protein, and the eukaryotic translation initiation factor 4E-binding protein, and mitogen-activated protein kinase were assessed by immunohistochemistry in 101 formalin-fixed, paraffin-embedded samples, including 68 primary tumors in myxofibrosarcoma. Immunohistochemical expressions were confirmed by Western blotting with 20 frozen samples, which were paired with normal tissue samples. PIK3CA and AKT1 gene mutations were also analyzed using 12 primary tumor frozen samples. Immunohistochemically, phosphorylations of Akt, mTOR, S6 ribosomal protein, 4E-binding protein, and mitogen-activated protein kinase 1/2 were observed in 64.7%, 45.6%, 42.6%, 63.2%, and 64.7% of samples. Phosphorylated Akt/mTOR pathway proteins were correlated with one another and were also correlated with the phosphorylation of these proteins in the concordant recurrent tumors. Immunoblotting showed a high degree of phosphorylation in tumor samples, compared with that in normal tissue samples. Activation of the Akt/mTOR pathway was correlated with histologic grade and tumor progression. Mutational analysis failed to reveal any PIK3CA or AKT1 mutations around the hot spots. Activation of the Akt/mTOR pathway was associated with histologic malignancy and tumor progression in primary and recurrent myxofibrosarcoma.

Histiocitoma fibroso maligno. Un sarcoma excepcional en la glándula mamaria / Malignant fibrous histiocytoma. An exceptional sarcoma in the mammary gland

Introducción. El histiocitoma fibroso maligno constituye la neoplasia sarcomatosa más frecuente en los adultos, pero la mama es una localización excepcional. Presentamos el caso de una mujer que comenzó con una tumoración de crecimiento progresivo en la mama derecha. Caso clínico. Mujer de 68 años que consulta por autopalpación de un nódulo en la mama derecha que ha crecido de forma progresiva en los últimos meses. La mamografía y la ecografía muestran una imagen nodular con bordes bien definidos, situada en intercuadrantes superiores de mama derecha, sin adenopatías axilares. Se decidió intervención quirúrgica y el estudio histológico definitivo fue informado como neoformación mesenquimal fusocelular con patrón estoriforme. El estudio inmunohistoquímico fue compatible con un histiocitoma fibroso maligno. Conclusión. Es primordial el diagnóstico diferencial de esta entidad clínica debido a la variabilidad histológica de los tumores sarcomatosos. Sus características clínicas y radiológicas pueden hacerlo pasar desapercibido, pero su comportamiento agresivo hace necesario un diagnóstico precoz, lo cual permitirá un tratamiento adecuado para lograr el aumento en la supervivencia(AU)

Introduction. Malignant fibrous histiocytoma is the most common sarcomatous neoplasm in adults. Localization in the breast, however, is exceptional. We report the case of a woman who presented with progressive tumoral growth in the right breast. Case report. A 68-year-old woman consulted for a self-palpated nodule in the right breast that had grown steadily in the last few months. Mammography and ultrasound showed a nodule with well-defined borders, located in the upper inner quadrant of the right breast. There was no axillary lymphadenopathy. Surgery was performed and the histological examination gave a definitive diagnosis of mesenchymal spindle cell neoplasm with storiform pattern. Immunohistochemical analysis was compatible with a diagnosis of malignant fibrous histiocytoma. Conclusion. Due to the histological variability of sarcomatous tumors, differential diagnosis is paramount in malignant fibrous histiocytoma. Because of their clinical and radiological features, malignant fibrous histiocytoma can be overlooked. Because these tumors are aggressive, an early diagnosis is essential to allow appropriate treatment and to increase survival(AU)

Sarcoma pleomórfico em úlcera de Marjolin / Pleomorphic sarcoma in Marjolin's ulcer

Úlcera de Marjolin é uma transformação maligna em tecido cutâneo cronicamente inflamado ou traumatizado, que ocorre especialmente após queimaduras. O carcinoma de células escamosas é o tipo histológico mais encontrado nas úlceras de Marjolin, seguido de carcinoma basocelular e melanoma maligno. Sarcomas em úlcera de Marjolin são raros, correspondendo a aproximadamente 5% dessas degenerações malignas. Neste artigo é descrito o caso de paciente do sexo feminino, vítima de queimadura há 42 anos, com grande ulceração em dorso. A biópsia dessa ulceração evidenciou sarcoma pleomórfico de alto grau em úlcera de Marjolin. A paciente foi submetida a ressecção da ulceração e enxerto de pele no local, seguidos de radioterapia e quimioterapia adjuvantes. Em 3 anos de seguimento, a paciente não apresentou recidiva da neoplasia. Úlceras de Marjolin são neoplasias malignas de comportamento agressivo, com alto índice de metástases regionais. A importância de seu entendimento está na necessidade de prevenção das mesmas, com o tratamento adequado dos pacientes queimados, evitando-se a cicatrização por segunda intenção. Sarcomas em úlcera de Marjolin são considerados raros, com poucos casos relatados na literatura, o que demonstra a importância deste relato.

Marjolin's ulcer is a malignant transformation of traumatized or chronically inflamed cutaneous tissue that occurs after burns. The most common histological type of carcinoma found in Marjolin's ulcers is squamous cell carcinoma, followed by basal cell carcinoma and malignant melanoma. Sarcomas in Marjolin's ulcers are rare, representing approximately 5% of these malignant degenerations. In this report, we describe the case of a female patient who was burned 42 years prior, with a large ulceration on her back. Biopsy of the ulceration showed a high-grade pleomorphic sarcoma in the Marjolin's ulcer. The patient underwent resection of the ulceration and a skin graft followed by radiation therapy and adjuvant chemotherapy. In 3 years of follow-up, the patient had no tumor recurrence. Marjolin's ulcers are aggressive and have a high rate of regional metastases. It is important that clinicians develop an understanding of their prevention by properly treating burns. Sarcomas in Marjolin's ulcers are rare and few cases have been reported in the literature, which demonstrates the importance of this report.

Mediastinal pleomorphic sarcoma in an immunodeficient patient: case report and review of the literature.

Pleomorphic sarcoma, widely known as malignant fibrous histiocytoma (MFH), is a soft tissue sarcoma. The occurrence of this malignancy in the mediastinum is rare. To our knowledge, only 13 cases of MFH of the mediastinum have been previously reported. Furthermore, only three cases of MFH in patients infected with human immunodeficiency virus (HIV) have been previously described. Here we present a 44-year-old African-American male who complained of epigastric pain radiating to the right chest. On admission, a chest radiograph revealed a widened mediastinum, and chest computerized tomography (CT) identified a large mass in the posterior mediastinum. Histologic diagnosis revealed a high-grade MFH. He was also incidentally diagnosed with HIV infection. The rarity of this malignancy and uncommon site of presentation in association with an immunodeficient state makes this case unique. This is the first report in the literature of an HIV-infected patient presenting with this uncommon tumor in the mediastinum.

Benign vs malignant soft tissue neoplasms: limitations of magnetic resonance imaging.

AIMS: Various features have been described in the literature to differentiate benign from malignant lesions. The aim of the present study was to study the accuracy of each of these features and that of magnetic resonance imaging (MRI) in diagnosing malignant lesions. MATERIALS AND METHODS: Fifty-five consecutive patients presenting with neoplastic (both benign and malignant) lesions diagnosed clinically and on ultrasound were studied and their MRI features were compared with the findings on surgical exploration and histopathologic examination. RESULTS: There were 32 (58%) benign and 23 (42%) malignant masses. Malignant masses were more common in patients older than 20 years (83%), and these had symptoms of less than 6 months duration (75%), as against benign lesions. The swelling was painful in 8 malignant masses and these were more common in the upper limbs (61%). Various features of malignant lesions were size more than 5 cm in 83%, change in signal intensity from homogenous on T1-weighted images to heterogenous on T2-weighted images in 74%, irregular margins in 74%, and heterogenous contrast enhancement in 91%. The accuracy of these features was 76%, 58%, 78%, and 60%, respectively. Most benign and malignant lesions were intramuscular in location. A significant number (38%) of benign lesions were located in the intermuscular facial plane. Definitive diagnosis was made in 42% of the lesions. CONCLUSIONS: MRI is an excellent modality for evaluating soft tissue neoplasms; however, prediction of a specific diagnosis and differentiation of malignant and benign lesions is not always possible.

Pleomorphic malignant fibrous histiocytoma/undifferentiated high-grade pleomorphic sarcoma of the scrotum in a patient presenting as fournier gangrene: a case report.

Pleomorphic malignant fibrous histiocytoma (MFH), also known as undifferentiated high-grade pleomorphic sarcoma according to the latest World Health Organization classification, is a diagnosis of exclusion and extremely rare in adult scrotal/paratesticular region. Clinical presentation of scrotal/paratesticular pleomorphic MFH is usually a painless and gradual scrotal swelling. We report a case of scrotal MFH in a 63-year-old man who presented as Fournier gangrene after 10-month painful scrotal swelling and multiple procedures. The specimen of emergent debridement was submitted for pathologic and bacteriologic examination. Microscopically, the lesion had marked architectural and cytologic pleomorphism. The neoplastic cells were positive for vimentin, but negative for all lineage-specific markers. Fluorescence in-situ hybridization showed an aneuploid karyotype and negative results for lipomatous tumor abnormalities. Bacterial cultures of the specimen showed extensive growth of virulent polymicrobes. The diagnosis of scrotal/paratesticular pleomorphic MFH with concurrent Fournier gangrene was made. Thoracic computed tomography scan showed bilateral multiple pulmonary nodules. The patient died 1 month later.

Sarcoma pulmonar primario / No disponible

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Un nuevo caso de histiocitoma fibroso maligno dependiente de cápsula renal / A new case of malignant fibrous histiocytoma arising from the renal capsule

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Characterization and signaling in a primary human malignant fibrous histiocytoma cell line.

Sarcomas are rare tumors, and malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma found in adults. Increasing evidence suggests a possible role for AKT activation in soft tissue sarcoma. In the present study, we established a primary human MFH cell line (named MSUMFH cells) from a fresh surgically resected MFH tumor. These cells morphologically resembled human normal fibroblasts that are the presumptive cells of origin of MFH tumor cells. As there is, unfortunately, no standard marker other than morphology to identify MFH at the cellular level, we compared MSUMFH cells to primary nonmalignant fibroblasts and the primary tumor specimen to characterize its signaling. AKT was hyperactivated in both the MSUMFH cell line and original primary MFH tumor cells compared to normal fibroblasts. The AKT hyperactivity in the MSUMFH cell line was not accompanied by activation of focal adhesion kinase (FAK) or downregulated expression of PTEN, each of which is a putative upstream regulator of AKT. In contrast, this AKT hyperactivation required PI-3K and Src in MSUMFH cells. This PI-3K and Src-dependent AKT-activated MSUMFH cell line that we established in this study may be beneficial for the future cell-based study of MFH biology.

Abdominal aorta and inferior vena cava thromboses in advanced stage of malignant fibrous histiocytoma.

Asymptomatic simultaneous thrombosis of abdominal aorta and inferior vena cava is a rare complication in advanced malignancy. We described an incidental finding of this clinical entity in our patient who presented with advance stage of malignant fibrous hystiocytoma of soft tissue and pathological fracture. The radiological evaluation with spiral computed tomography scan of abdominal aorta and inferior vena cava are presented and the subsequent management highlighted.