CIC-rearranged sarcoma: a clinicopathological analysis of four cases / 中华病理学杂志

Objective: To investigate the clinicopathological features and differential diagnosis of CIC-rearranged sarcoma (CRS). Methods: Five CRSs of 4 patients (2 biopsies of pelvic cavity and lung metastasis from case 4) diagnosed in the First Affiliated Hospital of Nanjing Medical University were enrolled from 2019 to 2021. All cases were evaluated by clinical presentation, H&E, immunohistochemical staining and molecular analysis and the related literature was reviewed. Results: There were one male and three females, the age at diagnosis ranged from 18 to 58 (mean 42.5) years. Three cases were from the deep soft tissues of the trunk and one case from the skin of foot. Grossly, the tumor size ranged from 1 to 16 cm. Microscopically, the tumor was arranged in nodules or solid sheets. The tumor cells were typically round or ovoid, with occasional spindled or epithelioid morphology. The nuclei were round to ovoid with vesicular chromatin and prominent nucleoli. Mitotic figures were brisk (>10/10 HPF). Rhabdoid cells were seen in four of five cases. Myxoid change and hemorrhage were observed in all samples and two cases showed geographic necrosis. Immunohistochemically, CD99 was variably positive in all samples, while WT1 and TLE-1 were positive in four of five samples. Molecular analysis showed CIC-rearrangements in all cases. Two patients succumbed within 3 months. One had mediastinal metastasis 9 months after surgery. One underwent adjuvant chemotherapy and remained tumor-free 10 months after diagnosis. Conclusions: CIC-rearranged sarcoma is uncommon and shows aggressive clinical course with dismal prognosis. The morphological and immunohistochemical characteristics can largely overlap with a variety of sarcomas; hence, knowledge of this entity is vital to avoid potential diagnostic pitfalls. Definitive diagnosis requires molecular confirmation of CIC-gene rearrangement.

Estudio clínico, radiológico y derivación en tumores musculoesqueléticos / Diagnosis and referral of musculoskeletal tumors

Musculoskeletal tumors are uncommon. However, the true burden of all bone and soft tissue tumors of extremities is underestimated. Usually, the diagnosis of sarcomas is missed or delayed. Therefore, an adequate clinical and radiological assessment, along with the awareness and application of simple guidelines for referral to a specialized center, are of utmost importance. These are critical steps for an appropriate diagnosis and treatment of sarcomas, improving their prognosis.

Extremities Soft Tissue Sarcomas, more Common and as Dangerous as Bone Sarcomas / Sarcomas de partes moles nos membros, mais comuns e tão graves quanto os sarcomas ósseos

Abstract Musculoskeletal sarcomas are rare diseases that require attention. They often present high degree of malignancy at diagnosis and, if underestimated, they can evolve aggressively locally and systemically. They present as soft tissues arcoma and bone sarcomas, with soft tissue being four to five times more common. Most soft tissue sarcomas occur in the extremities. The most common subtypes in children and adolescents are rhabdomyosarcoma and synovial sarcoma; in adults, undifferentiated pleomorphic sarcoma, liposarcoma, leiomyosarcoma, myxofibrosarcoma and synovial sarcoma; all with a high degree of histological malignancy. Many soft tissue sarcomas are confused with benign soft tissue tumors, 100 times more common, so they are resected without the necessary planning, resulting in amputation of a limb that could have been preserved. As in all cancers, the most important prognostic factor is metastatic disease. When it is present, the overall survival rate falls around 20 to 30%. Survival rates are generally similar between bone and soft tissue sarcomas. So soft tissue sarcomas, in addition to being more prevalent, are as aggressive as bones arcomas, deserving a lot of attention from orthopedic surgeons, who are often the first line of care of carriers of these tumors.

Resumo Os sarcomas musculoesqueléticos são doenças raras que exigem atenção. Frequentemente, apresentam alto grau de malignidade ao diagnóstico e se subestimados podem evoluir de forma agressiva local e sistemicamente. Apresentam-se como sarcoma de partes moles e sarcomas ósseos, sendo os de partes moles quatro a cinco vezes mais comuns. A maioria dos sarcomas de partes moles ocorre nos membros. Os subtipos mais comuns nas crianças e adolescentes são o rabdomiossarcoma e o sarcoma sinovial, nos adultos o sarcoma pleomórfico indiferenciado, lipossarcoma, leiomiossarcoma, mixofibrossarcoma e sarcoma sinovial; todos de alto grau de malignidade histológica. Muitos sarcomas de partes moles são confundidos com tumores benignos de partes moles, 100 vezes mais comuns, por isso são ressecados sem o planejamento necessário, acarretando em amputação de um membro que poderia ter sido preservado. Como em todos os cânceres, o fator prognóstico mais importante é a doença metastática. Na sua vigência, a taxa de sobrevida global cai em torno de 20 a 30%. As taxas de sobrevida no geral são parecidas entre os sarcomas ósseos e de partes moles, portanto o sarcoma de partes moles, além de mais prevalente, mostra-se tão agressivo quanto os sarcomas ósseos, por isso merece muita atenção dos ortopedistas que frequentemente são a primeira linha de atendimento dos portadores destes tumores.

Sarcomas de partes blandas en pacientes menores de 2 años. Serie de casos / Soft tissue sarcomas in infants. Case series

Los sarcomas de partes blandas en menores de 2 años son infrecuentes. Durante 2007-2017, 445 pacientes con sarcomas de partes blandas fueron tratados en nuestra Institución; 6 (el 0,5 %) eran menores de 2 años. Se analizaron los resultados clínicos y oncológicos en este grupo. La edad media de diagnóstico fue 15 meses. Cuatro eran varones y 2, mujeres. El seguimiento promedio fue 29 meses. El fibrosarcoma (n = 4) fue la variedad más frecuente. Cinco fueron tratados con cirugía de conservación del miembro; al restante se le realizó amputación. Todos realizaron tratamiento adyuvante con quimioterapia. La supervivencia a 24 meses fue del 100 %. Dos pacientes presentaron recidiva local; ambos casos, antes de los 24 meses.El tratamiento quirúrgico asociado a la quimioterapia impresiona ser la mejor opción terapéutica. La proporción de recurrencia local es alta para este grupo de pacientes luego de la cirugía de conservación del miembro.

Soft tissue sarcomas in children under 2 years of age are infrequent. During 2007-2017, a total of 445 patients diagnosed with soft tissue sarcomas were treated at our institution, 6 (0.5 %) were under 2 years. We analysed clinical and oncologic outcomes in this select group. The mean age of diagnosis was 15 months. Four patients were male and 2 female. The mean follow-up time was 29 months. Fibrosarcoma (n = 4) was the most frequent diagnosis. Five patients were treated with limb salvage surgery, and the remaining one had to undergo amputation. All patients received adjuvant treatment with chemotherapy. The 24-month survival rate was 100 %. Two patients presented a local recurrence before 24-months follow-up. Surgical treatment associated with chemotherapy seems to be the best therapeutic option. Local recurrence rate after limb salvage surgery is high for this group of patients

When an unexpected diagnosis occurs: a vaginal premenopausal sarcoma / Quando um diagnóstico inesperado ocorre: um sarcoma vaginal na pré-menopausa

Abstract Vaginal cancer is a rare entity. The evidence on its management resides mostly in clinical cases or small case series. Of the histological types, the most frequent is the squamous cell carcinoma, followed by adenocarcinoma. But what to do when identifying an even more infrequent sarcoma in a premenopausal woman? In this study, we describe the case of a 53-year-old woman presenting with metrorrhagia for two months, who was evaluated after an intense episode. A necrotic and ulcerative vaginal swelling was documented and then submitted to biopsy, which revealed a vaginal sarcoma. The patient was referred to radiation therapy with 50 Gy (aiming to control the symptoms and to cause tumor reduction for posterior pelvic exenteration with intraoperative radiotherapy) and developed an extra-pelvic metastization at the end of the treatment, which caused a fast negative outcome. Despite the initial poor prognosis, a chemo-irradiation or primary surgery regimen might have achieved (although with greater side effects) a better survival. This case-report entails a discussion about the strategies to manage vaginal sarcoma in advanced stage and in premenopausal women.

Resumo O cancro vaginal é uma doença rara. A evidência para a sua abordagem reside fundamentalmente em casos clínicos ou pequenas séries de casos. Dentre os tipos de cancro histológicos, o mais frequente é o carcinoma espinocelular, seguido do adenocarcinoma. Mas o que fazer em presença de um sarcoma ainda mais raro numa mulher pré-menopáusica? No presente estudo, descrevemos o caso de uma mulher de 53 anos apresentando metrorragia por dois meses, avaliada após um episódio intenso. Foi então documentada uma tumefacção vaginal necrótica e ulcerativa, submetida a biópsia, que revelou um sarcoma vaginal. A paciente foi encaminhada para radioterapia com50 Gy (comos objetivos de controlo da sintomatologia e de redução tumoral para posterior exenteração pélvica com radioterapia intraoperatória) e desenvolveu, ao final do tratamento, umquadro demestastização extra pélvica, que ocasionou um desfecho negativo rápido. Apesar do mau prognóstico inicial, um esquema de quimiorradiação ou cirurgia primária poderiam ter alcançado (ainda que com maiores efeitos laterais) uma maior sobrevivência. Este estudo de caso aborda uma discussão sobre as estratégias de abordagem do sarcoma vaginal em estádios avançados e na mulher pré-menopáusica.

Evaluation of adjuvant chemotherapy in feline injection site-associated sarcoma / Avaliação da quimioterapia no tratamento do sarcoma de aplicação felino

Feline Injection Site-Associated Sarcoma (FISS) is a neoplasm that implies in reduction of quality of life and overall survival in feline patients. A retrospective study of 13 cases of FISS was conducted to evaluate the efficacy of surgical treatment associated to chemotherapy with doxorubicin or carboplatin. Local recurrence occurred in all patients. Patients treated with surgery and chemotherapy presented a longer overall survival and disease-free interval when compared to those that solely received surgical treatment, although no statistical significance was observed (p= 0.3360 and 0.7506, respectively). Surgery remains as the main option for FISS treatment. Further prospective studies with larger samples are warranted to investigate the benefit of chemotherapy for this neoplasm.(AU)

O Sarcoma de Aplicação Felino (SAF) é uma neoplasia associada a redução na qualidade de vida e sobrevida global. O objetivo deste estudo foi avaliar a eficácia da quimioterapia associada à cirurgia no manejo do SAF. Estudo retrospectivo de 13 pacientes com SAF submetidos à cirurgia isolada ou associada a quimioterapia com carboplatina ou doxorrubicina. Recorrência local ocorreu em todos os pacientes. Pacientes tratados com cirurgia e quimioterapia apresentaram maior sobrevida global e intervalo livre de doença quando comparados àqueles que receberam apenas tratamento cirúrgico, mas não foi observada diferença estatística (p=0,3360 e 0,7506, respectivamente). A cirurgia continua sendo a principal opção para o tratamento do SAF. Estudos prospectivos são necessários para investigação do real benefício da quimioterapia para esta neoplasia.(AU)

Implante de Port-o-Cath para quimioterapia antineoplásica em um canino: relato de caso / Implant of Port-o-Cath for antineoplastic chemotherapy in a canine: case report

O Port-o-Cath é um cateter venoso central totalmente implantável, o qual permite a infusão de quimioterápicos, hemoderivados, nutrição parenteral e coleta de sangue. Este trabalho relata o caso de um canino com um tumor no membro pélvico que foi tratado cirurgicamente e recebeu o cateter para a quimioterapia antineoplásica. A paciente possuía um sarcoma sinovial, tumor incomum em cães, que acomete normalmente as articulações e exige terapia multimodal. O tratamento foi feito em duas etapas; na primeira, o membro foi amputado e, na segunda, foi realizado o implante do Port-o-Cath e exérese de nódulo metastático. A cadela respondeu satisfatoriamente à cirurgia e realizou as sessões de quimioterapia com perfeito funcionamento do cateter, indo a óbito por outras causas 90 dias após o procedimento. O implante de Port-o-Cath para a quimioterapia é um grande aliado à terapia multimodal preconizada para o câncer, podendo o cateter ser considerado em pacientes que necessitem de medicação quimioterápica por longo período.(AU)

The Port-a-Cath is a totally implantable central venous catheter that allows the infusion of chemotherapeutic agents, blood products, parenteral nutrition, and blood sample collection. This work reports the case of a dog with a tumor in the pelvic limb that was treated surgically and received the catheter for cancer chemotherapy. The patient was diagnosed with synovial sarcoma, an uncommon tumor in dogs that usually affects the joints and requires multimodal therapy. The treatment was done in two steps, in the first the limb was amputated and in the second the Port-a-Cath implantation and the excision of the metastatic nodule were performed. The dog responded well to surgery and chemotherapy sessions held with proper functioning of the catheter, and died from other causes 90 days after the procedure. The implantation of Port-a-Cath for chemotherapy is a great ally of multimodal therapy recommended for cancer, so this catheter may be considered for patients requiring chemotherapy medication for a long period.(AU)

A massagem na criança com câncer: eficácia de um protocolo / Massage in children with cancer: effectiveness of a protocol

OBJETIVOS: a massagem é uma intervenção que pode contribuir para o alívio da dor, embora as evidências empíricas sejam escassas e contraditórias. O objetivo deste trabalho foi avaliar a eficácia de um protocolo de massagem no alívio da dor na criança internada, com patologia oncológica. MÉTODOS: através de um estudo randomizado e controlado com cegamento simples, foram estudadas 52 crianças, com idades compreendidas entre os 10 e os 18 anos, internadas num serviço de oncologia pediátrica. A intervenção consistiu na aplicação de um protocolo de massagem de três sessões com duração entre 20 a 30 minutos em dias alternados durante uma semana. A eficácia do protocolo foi medida através da avaliação da dor com a aplicação do Inventário Resumido de Dor e a eficácia de cada sessão de massagem pela Escala Visual Analógica (EVA). RESULTADOS: o protocolo de massagem apenas se revelou eficaz na diminuição da interferência da dor no andar (p < 0,05), apesar de ter contribuído para o alívio da dor e sua interferência nas atividades da criança. Após cada sessão de massagem a intensidade da dor sentida pela criança diminuiu (p < 0,001). CONCLUSÕES: apesar da reduzida dimensão da amostra, a massagem parece ser uma intervenção útil no alívio da dor da criança que sofre de patologia oncológica, embora permaneçam dúvidas quanto à eficácia deste protocolo de massagem. Todavia, os autores recomendam a sua utilização pela sua contribuição na promoção do bem-estar e qualidade de vida da criança.

OBJECTIVES: massage can help relieve pain, although empirical evidence is scarce and contradictory. This study aims to assess the effectiveness of a massage protocol in relieving pain in children hospitalized with cancer. METHODS: a randomized, controlled, and single-blind trial was performed in a sample of 52 children aged between 10 and 18 years who were hospitalized in a pediatric cancer ward. The intervention consisted of the implementation of a massage protocol with three sessions of 20 to 30 minutes on alternate days over a one-week period. The effectiveness of the protocol was evaluated by assessing pain using the Brief Pain Inventory (BPI), while the effectiveness of each massage session was measured using the Visual Analogue Scale (VAS). RESULTS: the massage protocol was only effective in reducing the interference of pain in walking (p < 0.05), although it also contributed to relieve pain and its impact on the children's activities. After each massage session, the intensity of the pain experienced by the child decreased (p < 0.001). CONCLUSIONS: despite the small sample size, massage therapy appears to be a useful intervention in reducing pain in children with cancer. However, there are still questions regarding the effectiveness of this massage protocol. The authors recommend its use due to its contribution to the promotion of the child's well-being and quality of life.

Novos conceitos e revisão atualizada sobre sarcomas uterinos / New concepts and update review of uterine sarcomas

Realizou-se uma revisão sistemática das publicações dos últimos dez anos sobre os sarcomas uterinos. Este artigo traz novos conceitos, como a mudança do carcinossarcoma para carcinoma metaplásico. Evidencia-se a necessidade de individualizar o tratamento dos sarcomas, pois existem muitas diferenças de comportamento clínico, padrão metastático e prognóstico entre os diferentes tipos histológicos. Também foram enfocadas as controvérsias existentes quanto a extensão cirúrgica, a necessidade de ooforectomia e de esvaziamento ganglionar e o papel da adjuvância

It was performed a systematic review of publications for the past 10 years about uterine sarcomas. This article introduces new concepts, such as the change of carcinosarcoma to metaplastic carcinoma. This study highlights the need to individualize the treatment of uterine sarcomas, as there are many differences in clinical behavior, prognosis and metastatic pattern between the different histological types. Controversies have also been focused on the surgical extension, the need for lymph node dissection and oophorectomy and the role of adjuvant therapy

Adult soft-tissue sarcomas of the head and neck.

Background: Head and neck sarcomas are relatively rare tumors, accounting for only about 2% of all head and neck malignancies and for 4 to 10% of all soft tissue sarcomas. The rarity and more over the heterogeneity of these tumors make rigorous study of their clinical behavior difficult. Aims: We analyzed our single center experience of adult soft tissue sarcomas of the head and neck. Settings and Design: Our study included 27 patients of adult soft-tissue sarcomas of the head and neck who presented to our center between January 1996 and December 2005. Materials and Methods: Patient characteristics, clinical features, treatment modalities, clinical course, and long-term outcomes and its determinants were analyzed. Results: Surgery in the form of wide excision aimed at achieving negative margins was the main modality of treatment and adjuvant radiation was used in selected patients. The five-year disease-free survival was 64.5% and overall survival (OS) was 68%. Of the multiple potential factors analyzed, univariate analysis showed grade, margin status, and adjuvant radiation to be the important prognostic factors for the survival. The multivariate analysis however only showed margin status and adjuvant radiation to be prognostic. Conclusions: Surgery with negative margins is a key to long-term survival. Complete resection with adjuvant radiation in selected cases potentially decreases local recurrences and improves OS.

Clinicopathological study of patients with head and neck sarcomas / Avaliação clínico-patológica de pacientes com sarcomas em cabeça e pescoço

Sarcomas are rare tumors, mainly stemming from the embryonic mesoderm, with a high grade of morbidity and mortality. OBJECTIVE: To carry out a retrospective study of head and neck sarcoma cases between 1999 and 2008 in three specialized centers in the city of Fortaleza. MATERIALS AND METHODS: Data collection was based on the charts of the patients in the study. For statistical analysis purposes we used the chi-square associations and the z test for proportions. RESULTS: We found records of 36 patients, and the most affected ones were adult brown males, in the age range between 20 and 59 years - mean age of 39.7. The man/woman ratio was 1.76:1. The most prevalent histological type was the rhabdomyosarcoma and their most common locations were the face and the neck. Most of the sample was made up of live patients without evidence of the disease in the last visit - 41.6 percent of the cases. The most common treatment modes were the combination of surgery + radiotherapy + chemotherapy and surgery + radiotherapy, with 27.8 percent of the cases each. CONCLUSION: Sarcomas have a great histological variability and may have numerous locations. Since these are rare and not well-known lesions, new epidemiological studies must be carried out in order to enhance our understanding of the disease.

Sarcomas são tumores raros originados predominantemente do mesoderma embrionário e que apresentam elevado grau de morbidade e mortalidade. OBJETIVO: Realizar estudo retrospectivo dos casos de sarcomas em cabeça e pescoço no período de 1999 a 2008 em três centros especializados. MATERIAL E MÉTODO: A coleta de dados teve base nos prontuários dos pacientes estudados. Para análise estatística, foram utilizados os testes de associações qui-quadrado e o teste z para proporções. RESULTADO: Foram encontrados registros de 36 pacientes, sendo os indivíduos mais acometidos homens adultos, da raça parda, na faixa etária de 20 a 59 anos, com idade média de 39,7. A relação homem/mulher foi de 1,76:1. O tipo histológico mais prevalente foi rabdomiossarcoma e as localizações mais comuns foram face e região cervical. A maior parte da amostra foi de pacientes vivos sem evidência de doença na última consulta, com 41,6 por cento dos casos. As formas mais comuns de tratamento foram a combinação de cirurgia+radioterapia+quimioterapia e cirurgia + radioterapia, com 27,8 por cento dos casos cada. CONCLUSÃO: Os sarcomas apresentam grande variedade histológica e podem acometer diversas localizações. Por se tratar de lesões raras e pouco conhecidas, novos estudos epidemiológicos devem ser realizados para que se aumente o conhecimento sobre a doença.

Neoadjuvant chemoradiation therapy for soft tissue sarcomas of the extremities

INTRODUCTION AND OBJECTIVE: Neoadjuvant and adjuvant therapies for soft tissue sarcomas of the extremities are still controversial. The aim of this study was to analyze the results of a protocol of neoadjuvant chemoradiation therapy for extremity sarcomas. METHODS: A retrospective analysis was carried out in a consecutive series of 49 adult patients with advanced extremity soft tissue sarcomas that could not be resected with adequate margins during the primary resection. All patients were treated with a protocol of preoperative radiation therapy at a total dose of 30 Gy, concomitant with doxorubicin (60 mg/m²) chemotherapy. The main endpoints assessed were local recurrence-free survival, metastasis-free survival and overall survival. The median follow-up time was 32.1 months. RESULTS: The five-year local recurrence-free survival, metastasis-free survival and overall survival rates were 81.5 percent, 46.7 percent and 58.3 percent, respectively. For high-grade tumors, the five-year metastasis-free and overall survival rates were only 36.3 percent and 41.2 percent, respectively. Severe wound complications were observed in 41.8 percent of the patients who underwent surgery. These complications precluded adjuvant chemotherapy in 73.7 percent (14/19) of the patients eligible to receive it. CONCLUSIONS: In this study, neoadjuvant chemoradiation therapy was associated with a good local control rate, but the distant relapse-free rate and overall survival rate were still poor. The high rate of wound complications modified the planning of adjuvant treatment in most patients.

O uso da ventilação mecânica não-invasiva nos cuidados paliativos de paciente com sarcoma torácico metastático: relato de caso / The use of noninvasive mechanical ventilation in the palliative care of a patient with metastatic thoracic sarcoma: case report

JUSTIFICATIVA E OBJETIVOS: Os cuidados paliativos se baseiam na prevenção e alívio do sofrimento, identificando, avaliando e tratando a dor e outros problemas físicos, psicossociais e espirituais. Quadros súbitos de dispnéia são freqüentes em pacientes oncológicos em fase terminal. Nestes casos, a ventilação mecânica não-invasiva pode ser uma boa opção no manuseio da dispnéia, promovendo conforto e permitindo o contato do paciente com seus familiares. O objetivo deste estudo foi apresentar os benefícios obtidos com a ventilação mecânica não-invasiva no paciente sob cuidados paliativos. RELATO DO CASO: Paciente com 29 anos, em pós-operatório imediato de cesariana, admitida na unidade de terapia intensiva (UTI) para tratamento de insuficiência respiratória aguda. À radiografia de tórax visualizava-se massa pulmonar a direita. Após investigação clínica e de imagem foi diagnosticado sarcoma torácico metastático em fase avançada, sendo indicadas pela equipe de Oncologia medidas e cuidados paliativos. Para alivio da dispnéia, a equipe multidisciplinar da UTI optou pelo uso de ventilação mecânica não-invasiva (modalidade CPAP + PSV), permitindo a interação da mãe com o bebê e familiares. CONCLUSÕES: No contexto de cuidados paliativos, a VMNI mostrou-se ser um método capaz de contribuir para o controle da dispnéia provendo conforto e alívio ao paciente.

BACKGROUND AND OBJECTIVES: Palliative care is based on prevention and relief of suffering, identifying, assessing and treating pain and other physical, psychological, social and spiritual problems. Sudden dyspnea is frequently observed in terminal oncologic patients. In these cases, noninvasive ventilation can be an adequate option to control dyspnea promoting comfort and allowing patient interaction with their relatives. The aim of this article was to present the benefits of noninvasive ventilation in the palliative care setting. CASE REPORT: The case of a 29 year old patient, admitted in intensive care unit (ICU), after cesarean section delivery, for clinical treatment of acute respiratory failure is reported. Chest X-ray showed pulmonary mass in the right lung. After clinical and image investigation, metastatic thoracic sarcoma was diagnosed and palliative cares were introduced. The ICU interdisciplinary team chose to use noninvasive ventilation (modality CPAP + PSV) to relieve dyspnea and discomfort, as well as to allow interaction with her baby and family. CONCLUSIONS: Palliative care with noninvasive ventilation contributed to increase comfort of the patient by controlling dyspnea.

Kaposi Sarcoma: Changing Trend in Calabar; South Eastern Nigeria

Background/objective: Kaposi sarcoma (KS) is now the most frequently reported malignant skin tumour in some areas of Africa and was endemic in Africa before the advent of the human immunodeficiency virus (HIV) infection. The prevalence has increased with the emergence of HIV infection. The objective of this report is to describe the frequency; current clinical pattern; and anatomic distribution of KS in Calabar; south-eastern Nigeria and compare this with total malignant skin tumour. Method: All the patients with histologic diagnosis of KS presenting to the University of Calabar Teaching Hospital from January 2005 and December 2006 were analyzed as part of the wider study of malignant skin tumorus. Diagnosis of HIV was based upon enzyme linked immunosorbent assay. Results: In our study; there were 11 patients (7 males and 4 females); with a male: female ratio of 1.75: 1. This was the commonest malignant skin tumor (38) followed by squamous cell carcinoma (SCC) (34.5) and the age ranged from 21 - 60 years (mean 42.9years). Nine patients (81.8) were HIV positive including the 4 females (age ranged from 21 -45 years) and 2(18.2) HIV negative; aged 59 and 60years. The lower limb was the commonest site (50). Atypical lesions involved the eyelids/ nose and penis. Conclusion: KS is now the commonest malignant skin tumour in our region with the HIV related KS as the commonest clinical type. Successful prevention and treatment of HIV infection would reduce the prevalence of this tumour

Description of a neural sheath tumor of the trigeminal nerve: immunohistochemical and electron microscopy study

CONTEXT: Malignant neural sheath tumors of the trigeminal nerve affecting the nasal cavity and the paranasal sinuses are extremely rare. With conventional optical microscopy, their identification is difficult, and it is necessary to confirm them by means of electron microscopy and immunohistochemical techniques. CASE REPORT: The patient was a 41-year-old woman with a ten-month progressive history of pain followed by painful edema in the left facial region, and with symptoms of bleeding, secretion and nasal obstruction. Studies with imaging methods suggested the presence of an expansive process in the left nasal and paranasal cavities. In the biopsy, the histopathological findings from optical microscopy were suggestive of a tumor of neural origin in the trigeminal nerve. Immunohistochemical and electron microscopy studies confirmed that it was a malignant tumor of the neural sheath of the trigeminal nerve. We describe the clinical, radiological, and histological features of this tumor and review the literature.

CONTEXTO: O tumor maligno da bainha neural do trigêmeo que acomete a cavidade nasal e os seios paranasais é extremamente raro. A microscopia óptica habitual sua identificação é difícil, sendo necessária a confirmação através de microscopia eletrônica e de técnicas imunoistoquímicas. RELATO DE CASO: A paciente era uma mulher de 41 anos com história de 10 meses de progressiva dor seguida de edema álgico em região facial à esquerda, e de sintomas de sangramento, secreção e obstrução nasal. Estudos com métodos de imagem sugeriam a presença de processo expansivo em cavidades nasais e paranasais à esquerda. A biopsia, os achados histopatológicos à microscopia óptica foram sugestivos de tumor de origem neural no trigêmeo. Estudos imunoistoquímico e por microscopia eletrônica confirmaram se tratar de neoplasia maligna da bainha neural do trigêmeo. Descrevemos as características clínicas, radiológicas e histológicas deste tumor, e revisamos a literatura.