Clinical analysis of 81 cases of intravenous leiomyomatosis confined to pelvic cavity / 中华妇产科杂志

Objective:To investigate the clinical features, treatment and recurrence factors of intravenous leiomyomatosis (IVL) confined to the pelvic cavity after the operation.Methods:The clinical data of 81 patients who underwent surgery and were pathologically diagnosed with IVL at Women′s Hospital, School of Medicine, Zhejiang University from January 2014 to March 2021, were analyzed retrospectively to explore the influencing factors of postoperative recurrence of IVL, including age, gravidity and parity, surgical methods, intraoperative conditions and so on.Results:(1) Clinical features: the age of 81 IVL patients was (43.9±8.1) years old; increased menstrual volume in 26 cases (32%, 26/81), prolonged menstrual period in 31 cases (38%, 31/81), frequency and urgency to urinate in 4 cases (5%, 4/81), abdominal pain and abdominal distension in 8 cases (10%, 8/81), and pelvic masses in 34 cases (42%, 34/81). IVL was diagnosed right in 4 of 72 patients (6%, 4/72) underwent preoperative ultrasound, right in 11 of 51 patients (22%, 11/51) underwent magnetic resonance imaging (MRI), and right in 4 of 19 patients (4/19) underwent CT. (2) Treatment: all patients with IVL underwent surgical treatment. Surgical procedure: myomectomy in 37 cases, total hysterectomy and bilateral salpingectomy in 19 cases, total hysterectomy and bilateral salpingo-ophorectomy in 25 cases. Surgical approach: hysteroscopic operation in 6 cases, transabdominal operation in 52 cases, laparoscopic operation in 23 cases. Fifty-three cases underwent rapid intraoperative pathological examination, 17 cases (32%, 17/53) of them were diagnosed right as IVL. (3) Influencing factors of IVL postoperative recurrence: among 81 patients with IVL, 3 cases were lost to follow-up, and 8 cases (10%, 8/78) had recurrence during follow-up. Age<35 years, number of pregnancies<2, number of births<2, number of fibroids ≥10, abnormal appearance of fibroids (long, vermicular, beadlike, cystic, etc.) and IVL invasion into adjacent vessels were all the risk factors influencing postoperative recurrence of IVL (all P<0.05). Methods of operation, ovariectomy or not, IVL invasion or not, maximum diameter of IVL, abundant blood vessels near uterine fibroids were not associated with postoperative recurrence of IVL (all P>0.05). Conclusions:The clinical manifestations and preoperative auxiliary examination of IVL are lack of specificity. Doctors need to pay attention to young patients with uterine fibroids, and choose the appropriate surgical scope when the characteristic manifestations of IVL be found during the operation, or the right diagnoses of IVL in the surgery′s rapid intraoperative pathology be examined, and should remove the IVL lesions to reduce the recurrence as far as possible.

Adult diffuse hepatic hemangiomatosis

Abstract Diffuse hepatic hemangiomatosis (DHH) is an uncommon vascular lesion, though hemangiomas are the commonest benign tumors of the liver. The etiology is largely unknown to date; however, its association with giant cavernous hemangiomas (GCH) has been reported in the literature. We present herein, the case of a 37-year-old hypothyroid woman with abdominal fullness for 2 months. The contrast-enhanced computed tomography revealed multiple well-encapsulated lesions involving the liver lobes and was diagnosed as giant cavernous hemangiomas. Most of them, except the deep-seated ones, were enucleated. Histopathological examination highlighted the presence of GCH with irregular margin, replacement of hepatic parenchyma, and presence of multiple micro-hemangiomas suggesting the possibility of DHH further substantiated by retrospective radiological assessment. No extrahepatic vascular lesion was noted, and the post-operative recovery and follow-up were uneventful. Adult DHH is an uncommon entity. The diagnosis of DHH and its distinction from GCH are important from the management and prognostic point of view as recurrence, extrahepatic manifestations, features of consumption coagulopathy, and death from the complications are not uncommon.

Leiomioma vascular que afecta el labio: reporte de un caso y un análisis actualizado de los 78 casos reportados en la literature / Vascular leiomyoma affecting the lip: report of a case and an updated analysis of the 78 cases reported in the literature / Leiomioma vascular que afeta o lábio: relato de um caso e análise atualizada dos 78 casos relatados na literatura

Resumen El leiomioma vascular (LV) es un tumor benigno de tejido blando que rara vez se observa en los tejidos orales. El objetivo de este trabajo es presentar un nuevo caso de leiomioma vascular del labio (VLL), describiendo sus características clínicas, microscópicas e inmunohistoquímicas, y revisar la literatura sobre el tumor. Una mujer de 27 años presentó una pequeña lesión dolorosa en la mucosa del labio inferior. La biopsia escisional reveló una masa encapsulada bien circunscrita formada por células pequeñas y fusiformes. El análisis de inmunohistoquímica reveló una expresión intensa y difusa de actina del músculo liso (AME) dentro de las células tumorales y la inmunorreactividad CD34 de las células endoteliales que recubren los espacios vasculares, lo que indica la presencia de vasos sanguíneos. Se estableció un diagnóstico final de VLL. No se observaron signos de recurrencia después de cinco años de seguimiento. Aunque VLL es una lesión rara, debe considerarse en el diagnóstico diferencial de las lesiones nodulares en los labios inferior y superior.

Abstract Vascular Leiomyoma (VL) is a benign soft tissue tumor rarely observed in oral tissues. The aim of this paper is to present a new case of vascular leiomyoma of the lip (VLL), describing its clinical, microscopical, and immunohistochemical features, and review the literature on the tumor. A 27-year-old woman presented with a small painful lesion on lower lip mucosa. Excisional biopsy revealed a well-circumscribed, encapsulated mass formed by small and fusiform cells. Immunohistochemistry analysis revealed intense and diffuse expression of smooth muscle actin (SMA) within the tumor cells and CD34 immunoreactivity of the endothelial cells lining the vascular spaces, indicating the presence of blood vessels. A final diagnosis of VLL was established. No signs of recurrence were observed after five years of follow-up. Although VLL is a rare lesion, it must be considered in the differential diagnosis of nodular lesions on lower and upper lips.

Resumo O Leiomioma Vascular é um tumor benigno de tecidos moles raramente observado nos tecidos da cavidade bucal. O objetivo desse artigo é apresentar um novo caso de um leiomioma vascular no lábio (LVL), descrevendo seus achados clínicos, microscópicos e imunoistoquímicos, e revisar a literatura sobre o tumor. Uma mulher de 27 anos de idade se apresentou com uma pequena lesão dolorosa na mucosa do lábio inferior. A biópsia excisional revelou um massa encapsulada e bem circunscrita, formada por células pequenas e fusiformes. A análise imunoistoquímica revelou expressão intensa e difusa de actina musculo liso (AML) nas células tumorais e imunorreatividade para CD34 em células endoteliais que revestem espaços vasculares, indicando a presença de vasos sanguíneos. O diagnóstico final de LVL foi estabelecido. Não foram observados sinais de recorrência após cinco anos de acompanhamento. Embora o LVL seja uma lesão rara, deve ser considerada no diagnóstico diferencial de lesões nodulares nos lábios inferior e superior.

Anomalías Vasculares de la Cavidad Oral: Revisión de la Clasificación y Tratamiento Aplicado a dos Casos Clínicos / Vascular Anomalies of the Oral Cavity: Review of Classification and Treatment Applied to Two Clinical Cases

RESUMEN: Las anomalías vasculares de cabeza y cuello son un grupo de lesiones que afectan vasos sanguíneos y linfáticos donde el tratamiento sigue siendo un desafío. La clasificación actualizada de anomalías vasculares de cabeza y cuello es la clasificación de Mulliken modificada, que las subdivide en a) tumores vasculares y, b) malformaciones vasculares. En este reporte, presentamos dos casos clínicos de pacientes de sexo masculino, con diagnóstico de anomalías vasculares que afectan al labio y paladar duro, diagnosticados como malformación arteriovenosa y malformación venosa, respectivamente. Dichas lesiones remitieron completamente mediante tratamientos conservadores (agentes esclerosantes) y/o quirúrgicos (exéresis quirúrgica completa de la lesión) logrando una remisión completa. Consecutivamente, presentamos una revisión de la literatura enfocado a la clasificación actual, enfoques terapéuticos actuales y futuros.

ABSTRACT: Vascular anomalies of the head and neck are a group of lesions that affect blood and lymph vessels where treatment remains a challenge. The updated classification of head and neck vascular anomalies is the modified Mulliken classification, which subdivides them into a) vascular tumors and b) vascular malformations. In this report, we present two clinical cases of male patients, with diagnosis of vascular anomalies affecting the lip and hard palate, diagnosed as arteriovenous malformation and venous malformation, respectively. These lesions were completely treated with conservative (sclerosing agents) and/or surgical (complete surgical exeresis of the lesion) treatments, achieving a complete remission. Consequently, we present a review of the literature focused on the current classification, current and future therapeutic approaches.

Infantile Hepatic Hemangioma: Avoiding Unnecessary Invasive Procedures / 대한소아소화기영양학회지

Lobular capillary hemangioma in a patient with ankylosing spondylitis using adalimumab: an exuberant presentation

Abstract Lobular capillary hemangioma or pyogenic granuloma is a benign vascular tumor of the skin or mucous membranes. Most patients present a single lesion. It manifests clinically as an erythematous, friable, and fast-growing tumor. This report details a case with exuberant presentation in a patient with ankylosing spondylitis, using adalimumab. Factors triggering pyogenic granuloma are not well known. They may spontaneously regress, but most require treatment.

Lesiones vasculares en pacientes pediátricos asistidos en el Centro Hospitalario Pereira Rossell. Experiencia de dos años / Vascular lesions in pediatric patients assisted at the Pereira Rossell Children Hospital. Two years' experience / Lesões vasculares em pacientes pediátricos assistidos no Centro Hospitalar Pereira Rossell. Dois anos de experiência

Resumen: Introducción: las lesiones vasculares son un grupo heterogéneo de enfermedades que se distinguen por su presentación clínica, su evolución y su pronóstico. Es fundamental su correcto diagnóstico y clasificación con vistas a su seguimiento y tratamiento. Este estudio aporta datos epidemiológicos recabados en la Unidad de estudio y seguimiento de lesiones vasculares (UESLV) del Centro Hospitalario Pereira Rossell con el fin de aportar a los datos de Uruguay sobre estas lesiones, ya que hasta la fecha no hay estudios de éstas. Objetivo: describir el número de casos nuevos así como clasificar el tipo de lesión vascular que presentaron los niños asistidos en la UESLV del Centro Hospitalario Pereira Rossell durante el período comprendido entre mayo de 2015 y abril de 2017. Metodología: estudio observacional, descriptivo y transversal de los menores de 15 años con lesiones vasculares asistidos entre el 1º de mayo de 2015 y el 30 de abril de 2017 en la policlínica antes referida. Resultados: a 167 pacientes se les diagnóstico una lesión vascular. De estos, 83 presentaban tumores vasculares, 77 malformaciones vasculares, seis presentaban lesiones formando parte de un síndrome y un paciente presentaba una lesión vascular aún no clasificable.

Summary: Introduction : vascular lesions are a heterogeneous group of diseases that vary in their clinical presentation, evolution and prognosis. Their accurate diagnosis and classification are key in terms of follow-up and treatment. This study provides epidemiological data collected at the Vascular Lesion Study and Follow-Up Center of the Pereira Rossell Children Hospital (UESLV) and have the purpose of contributing to Uruguay's data collection regarding these lesions, since Uruguay does not have studies on them. Objective : to describe the number of new cases and to classify the type of vascular lesion presented in children assisted at the UESLV of the Pereira Rossell Children Hospital between May 2015 and April 2017. Methodology : observational, descriptive and transversal study performed on children under 15 years of age who showed vascular lesions between May 1, 2015 and April 30, 2017. Results : a total of 167 patients were diagnosed with vascular lesions, 83 had vascular tumors, 77 vascular malformations, 6 presented lesions as part of a syndrome and one patient had a vascular lesion not yet classified.

Resumo: Introdução : as lesões vasculares são um grupo heterogêneo de doenças que se distinguem pela sua apresentação clínica, evolução e prognóstico. O correto diagnóstico e classificação é fundamental, com vista ao monitoramento e tratamento. Este estudo fornece dados epidemiológicos coletados na Unidade de Estudo e Monitoramento de Lesões Vasculares (UESLV) do Centro Hospitalar Pereira Rossell, a fim de contribuir com dados do Uruguai sobre essas lesões, uma vez que não há estudos até o momento. Objetivo : descrever o número de casos novos e classificar o tipo de lesão vascular apresentada pelas crianças atendidas na Unidade de Estudo e Monitoramento de Lesões Vasculares do Centro Hospitalar Pereira Rossell no período de maio de 2015 a abril de 2017. Metodologia : estudo observacional, descritivo e transversal de crianças menores de 15 anos com lesões vasculares atendidas entre 1 de maio de 2015 e 30 de abril de 2017 na Unidade acima referida. Resultados : uma lesão vascular foi diagnosticada em 167 pacientes. Destes, 83 tinham tumores vasculares, 77 malformações vasculares, 6 tinham lesões que faziam parte de uma síndrome, e um paciente tinha uma lesão vascular que ainda não era classificável.

Cutaneous tumours of vascular origin: two rare entities

Skin and subcutaneous tissue are the most common location of benign and malignant vascular tumors. The benign or malignant nature of the lesion may not always be identified clinically because of the varying presentations. The histology of the lesion, especially in malignancies may not exhibit definite vascular pattern.  In such cases, clinical presentation has to be correlated with histopathological picture as well as immunohistochemical characteristics to reach a specific diagnosis. Judicious use of immunohistochemical markers proves to be invaluable.  Two rare cases of cutaneous vascular tumors which presented at our institution are cited, the diagnosis of which involved correlation of these modalities.

A cavernous hemangioma located in the axillary area: Challenges in preoperative diagnosis and operation

Cavernous hemangiomas are benign neoplasms of endothelial cells. Although this neoplasm has the potential to develop in all parts of the body, it rarely develops in the axilla; in fact, there are only two case reports of axillary cavernous hemangiomas in the literature. Here, we describe a third case, which occurred in a 30-year-old Korean woman. The patient presented with a palpable mass in the left axilla that was initially thought to be either a phyllodes tumor or a lymphoma based on imaging studies. However, the results of an excisional biopsy led to a diagnosis of cavernous hemangioma. Although uncommon, a cavernous hemangioma can be encountered unexpectedly, presenting as a mass in axilla. Although usually curative, surgery may be challenging not only because of the rarity of the condition, but also because of inconsistent preoperative findings and the involvement of large vessels.

Acquired elastotic hemangioma, a little-known entity: report of a case with 15 years of evolution

Abstract: Acquired elastotic hemangioma is a hemangioma variant first described in 2002. It is characterized by being a benign, solitary, slow-growing lesion, that appears in adulthood and is associated with solar exposure. It is a rare hemangioma variant with few cases reported in the literature. We present a case of acquired elastotic hemangioma on the back of the right hand and forearm in a male adult. Acquired elastotic hemangioma is a benign vascular proliferation associated with solar exposure, usually assymptomatic. It affects adults between 59 and 65 years of age. Histopatologically it is characterized by proliferation of small vessels in the upper dermis that are disposed parallel to the epidermis, and significant solar elastosis. The treatment is surgical, with no relapses reported.

An Aggressive Large Epithelioid Hemangioendothelioma of the Anterior Mediastinum in a Young Woman

Hemangioendothelioma is a rare vascular tumor with involvement of the liver, brain, long bones, and lung. Among the 6 histological subtypes, epithelioid hemangioendothelioma (EHE) is the most aggressive. Its occurrence in the mediastinum is quite rare, and very few cases have been documented. The reported cases in the literature have described difficulties in the preoperative diagnosis due to the unusual histological appearance of the tumor. Immunohistochemistry remains the mainstay for a definitive diagnosis. Due to its low incidence, there is no standard treatment for mediastinal EHE, but curative resection is the preferred treatment option where possible, with chemotherapy used as an adjuvant treatment or in cases of widespread inoperable disease. The present case study describes an aggressive EHE occurring in an 18-year-old woman in the anterior mediastinum.

Kasabach-Merritt phenomenon in 9 neonates / 中国新生儿科杂志

Objective To study the clinical features,diagnosis and treatment in the neonates of Kasabach-Merritt phenomenn (KMP).Method To analyze retrospectively the data collected from the 9 neonates of Kasabach-Merritt phenomenon in our neonatal intensive care unit from January 2016 to March 2017.The data of their clinical presentation,imaging findings,treatment and outcomes were analyzed.Result Among the 9 cases,there were 6 males and 3 females,with onset age of 0 ～ 14 d.The hemangiomas were located over the body surface of the neck,back,trunk and limbs in 4 cases.The others were located in the deep organs in 5 cases.2 cases were found hepatic hemangiomas by fetal ultrasound and 3 cases had symptoms of poor response,dyspnea and nasal bleeding with occult onset.All the 9 cases had thrombocytopenia,and the average platelet count was 24.0 × 109/L.The imaging findings showed the changes of hemangiomas,and among them,3 cases were associated with arteriovenous fistula.In one case,the biopsy pathology diagnosis was Kaposiform hemangioendothelioma.Among 8 cases which was treated,2 cases had systemic medication,2 cases had interventional embolization and 4 cases were treated with combined therapy (interventional embolization together with medication),including 1 case of definitive surgical treatment.Finally 7 cases improved,1 case with severe thrombocytopenia and coagulation disorders did not improve and treatment abandoned and 1 case died before treatment.Conclusion The symptomatology of KMP is various,which is related to the location of the lesion.The imaging studies are helpful to diagnosis.Massive hemorrhage and multiple organ dysfunction can be fatal.The treatments are different among cases,for most cases with proper treatment the prognosis are good.

Rapidly involuting congenital hemangioma

Abstract: Rapidly involuting congenital hemangioma is a rare vascular tumor that generally has a good prognosis. The authors describe a case of a newborn girl with a left cervical vascular lesion. Image exams were performed, and the lesion slowly decreased, leaving redundant skin. Considering all of the findings, a final diagnosis of a rapidly involuting congenital hemangiomas was suspected.

Characteristics of ERG, Fli-1, CD34, CD31 and FⅧRAg expression in hepatic malignant vascular tumors / 中华病理学杂志

Objective@#To investigate the expression of ERG, Fli-1, CD34, CD31 and factor Ⅷ-related antigen(FⅧRAg) in hepatic malignant vascular tumors.@*Methods@#A retrospective analysis was conducted on 63 cases of primary hepatic malignant vascular tumors and 31 cases of hepatic other malignant spindle cell tumors collected during January 1986 to January 2014. EnVision method was used to detect the expression of ERG, Fli-1, CD34, CD31, FⅧRAg.@*Results@#Sixty-three cases of malignant vascular tumors, including 24 cases of angiosarcoma, 38 cases of epithelioid hemangioendothelioma and 1 case of hepatic Kaposi′s sarcoma. All of the cases were positive for ERG(100.0%, 63/63). Positive rate of Fli-1, CD34, CD31, FⅧRAg was 96.8% (61/63), 87.3% (55/63), 81.0% (51/63) and 41.3% (26/63), respectively. In other hepatic malignant spindle cell tumors, the positive rate of ERG, Fli-1, CD34, CD31 and FⅧRAg was 3.2% (1/31), 19.4% (6/31), 19.4% (6/31), 9.7%(3/31) and 3.2%(1/31), respectively.The sensitivity of ERG, Fli-1, CD34, CD31, FⅧRAg was 100.0%, 96.8%, 87.3%, 81.0% and 41.3%, respectively.The specificity was 96.8%, 80.6%, 80.6%, 90.3% and 96.8%, respectively.@*Conclusion@#ERG is a more sensitive and specific diagnostic marker for hepatic malignant vascular tumors in comparison to Fli-1, CD34, CD31 and FⅧRAg.

Study on the relationship between laparoscopic operation and pseudo vascular invasion in stage Ⅰ a endometrial carcinoma / 中华妇产科杂志

Objective To explore relations between pseudo vascular invasion (PVI) and laparoscopic operation in patients with stage Ⅰa endometrial carcinoma. Methods Details of stage Ⅰa endometrial carcinoma patients′ clinical information and pathological sections who received operation treatment between January 2008 and October 2015 were obtained from the Dalian Maternal and Child Health Hospital. There were 515 cases entrolled into this study, including 332 (64.5%) cases performed surgery by laparoscopy and 183 (35.5%) cases undergone abdominal surgery. The vascular invasion rate and the microscopic manifestations of PVI in two groups were retrospectively compared and analyzed. The follow-up period ended February 2017, the adjuvant treatment and prognosis of patients with lympho-vascular space invasion (LVSI) and PVI were analysed. Results (1) Seventy-five of 515 cases with stage Ⅰa endometrial carcinoma, there were seven-five cases found with tumor cells in lympho-vascular space, including 52 cases of LVSI and 25 cases of PVI (2 cases of both). The incidence of cancer emboli in lympho-vascular space and LVSI in laparoscopic group and laparotomy group were respectively 15.4%(51/332) vs 13.1%(24/183) and 9.6%(32/332) vs 10.9%(20/183), there were no significant differences (all P>0.05). But the rate of PVI between the two group was significantly different [6.3% (21/332) vs 2.2%(4/183), P=0.036], laparoscopic group was higher than that in laparotomy group. (2) Of 332 cases in laparoscopic group, 21 cases was found PVI, containing 8 cases showed tumor tissue with mesenchyme or papillary structures located in large thick wall vessels of the outer lateral myometrium, 13 cases showed the tumor tissue was away from the vessel wall. Among 183 cases in laparotomy group, 3 cases with PVI which the tumor tissue was away from the vessel wall, and 1 with interstitial tissue. (3) During the follow-up period, 7 of 75 cases (both LVSI and PVI) were lost, and 66 cases were relapse-free survival, 2 cases recurred at fifth months and died respectively at 7, 20 months after the operation. This 2 cases were both PVI of laparoscopic group, and the pathological type was endometrioid adenocarcinoma grade 3, and adjuvant treatment was not accepted after the operation. The relapse-free survival with LVSI was 49 months and 46 months for cases with PVI. Conclusions Laparoscopic operation could increase PVI in patients with stageⅠa endometrial carcinoma, while does not increase LVSI. It is not yet possible to demonstrate that PVI affects the prognosis of patients.

Study on the relationship between laparoscopic operation and pseudo vascular invasion in stage Ⅰ a endometrial carcinoma / 中华妇产科杂志

Objective To explore relations between pseudo vascular invasion (PVI) and laparoscopic operation in patients with stage Ⅰa endometrial carcinoma. Methods Details of stage Ⅰa endometrial carcinoma patients′ clinical information and pathological sections who received operation treatment between January 2008 and October 2015 were obtained from the Dalian Maternal and Child Health Hospital. There were 515 cases entrolled into this study, including 332 (64.5%) cases performed surgery by laparoscopy and 183 (35.5%) cases undergone abdominal surgery. The vascular invasion rate and the microscopic manifestations of PVI in two groups were retrospectively compared and analyzed. The follow-up period ended February 2017, the adjuvant treatment and prognosis of patients with lympho-vascular space invasion (LVSI) and PVI were analysed. Results (1) Seventy-five of 515 cases with stage Ⅰa endometrial carcinoma, there were seven-five cases found with tumor cells in lympho-vascular space, including 52 cases of LVSI and 25 cases of PVI (2 cases of both). The incidence of cancer emboli in lympho-vascular space and LVSI in laparoscopic group and laparotomy group were respectively 15.4%(51/332) vs 13.1%(24/183) and 9.6%(32/332) vs 10.9%(20/183), there were no significant differences (all P>0.05). But the rate of PVI between the two group was significantly different [6.3% (21/332) vs 2.2%(4/183), P=0.036], laparoscopic group was higher than that in laparotomy group. (2) Of 332 cases in laparoscopic group, 21 cases was found PVI, containing 8 cases showed tumor tissue with mesenchyme or papillary structures located in large thick wall vessels of the outer lateral myometrium, 13 cases showed the tumor tissue was away from the vessel wall. Among 183 cases in laparotomy group, 3 cases with PVI which the tumor tissue was away from the vessel wall, and 1 with interstitial tissue. (3) During the follow-up period, 7 of 75 cases (both LVSI and PVI) were lost, and 66 cases were relapse-free survival, 2 cases recurred at fifth months and died respectively at 7, 20 months after the operation. This 2 cases were both PVI of laparoscopic group, and the pathological type was endometrioid adenocarcinoma grade 3, and adjuvant treatment was not accepted after the operation. The relapse-free survival with LVSI was 49 months and 46 months for cases with PVI. Conclusions Laparoscopic operation could increase PVI in patients with stageⅠa endometrial carcinoma, while does not increase LVSI. It is not yet possible to demonstrate that PVI affects the prognosis of patients.

Intrathoracic hemangioendothelioma presenting as refractory unilateral pleural effusion and thrombocytopenia

A variety of diseases are associated with the development of unilateral pleural effusion. Although unilateral pleural effusion is common, refractory unilateral pleural effusion is rare. It is important to make an accurate diagnosis using proper diagnostic tools. Thrombocytopenia is one of the rare conditions occurring from various diseases such as severe infection or autoimmune diseases. It can be life-threatening if accurate diagnosis and treatment are delayed and be a clue to accurate diagnosis in differential diagnosis from refractory pleural effusion. Kasabach-Merrit syndrome (KMS) is often accompanied by extensive vascular tumors and characterized by consumptive coagulopathy with profound thrombocytopenia. It is also important to have a high index of suspicion for the diagnosis. We report a case of KMS in a 2-month-old female infant with a vascular tumor on her left intrathoracic cage, who had presented refractory unilateral pleural effusion and thrombocytopenia. Initially, the patient was diagnosed as having complications of severe infection, and a chest tube was inserted for aggressive treatment. However, her unilateral pleural effusion persisted, and thrombocytopenia and hypofibrinogenemia were refractory. Chest imaging revealed an infiltrating large vascular tumor involving the cardiac border, diaphragm, and chest wall. The patient’s unilateral pleural effusion was misidentified as an infectious condition at the initial stage. As a result of the ultrasonography-guided biopsy, it was revealed to be Kaposiform hemangioendothelioma. The patient was cured after treatment for KMS.

Renal hemangiopericytoma: case report and literature review / Hemangiopericitoma renal: relato de caso e revisão de literatura

Hemangioperycytoma is a rare perivascular tumor that seldom involves the urogenital system. This tumor often appears with an unspecific clinical picture, and sometimes is associated with hematuria or hypertension. Diagnosis is based on a combination of histological and immunohistological findings. We report a case of a 52-year-old patient with renal hemangiopericytoma who underwent surgical treatment at our service. This report also includes a literature review on the subject.

Hemangiopericitoma é um raro tumor perivascular que raramente envolve o sistema urogenital. Esses tumores geralmente se manifestam com quadro clínico inespecífico, por vezes associado a hematúria ou hipertensão. O diagnóstico baseia-se numa combinação de alterações histológicas e imuno-histológica. Este artigo relatou o caso de uma paciente de 52 anos de idade com um hemangiopericitoma renal submetida a tratamento cirúrgico em nosso serviço e incluiu uma revisão de literatura sobre o assunto.

Fibrosarcoma de arteria pulmonar simulando un síndrome de Hughes Stovin / Pulmonary artery sarcoma mimicking a Hughes Stovin syndrome. Report of one case

Pulmonary artery sarcoma is an uncommon entity with high mortality. Its clinical presentation is usually indistinguishable from pulmonary embolism, which leads to a significant delay in diagnosis. Hughes-Stovin syndrome is characterized by venous thrombosis and aneurysms of the pulmonary or bronchial artery. We report a 59 year-old female with a history of recurrent pulmonary embolism. In the last thromboembolic episode a pulmonary artery aneurysm was found on a CT scan. The patient was operated performing a left inferior lobectomy. The patient died five days after surgery and the pathological examination of the surgical piece revealed a pulmonary artery sarcoma.

Case for diagnosis

Intravascular papillary endothelial hyperplasia is a benign vascular lesion caused by proliferation of endothelium. It is reactive to thrombotic or inflammatory stimuli in the vessel wall.We report the case of a 14-yearold male patient with a violet-colored erythematous tumoral lesion of progressive growth in the occipital region. The diagnosis of intravascular papillary endothelial hyperplasia (IPEH) was confirmed by clinical and histopathological findings. Total lesion exeresis was performed with no recurrence up to date. IPEH presents clinical importance due to its clinical and histological resemblance to angiosarcoma. In order to differentiate it from angiosarcoma, distinguishing features of the benign disease should be considered, such as lack of cellular atypia and rare mitotic activity.Prognosis is good.