Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma - a rare vascular neoplasm with deceptive morphology and distinctive immunophenotype

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma is a rare, low grade vascular(endothelial) neoplasm typically presenting as multicentric, superficial to deep nodules in extremitieswith a slight tendency of affecting young adult males. We report a case of pseudomyogenichemangioendothelioma in a 15-year-old boy presenting initially with a 1 cm right thigh painlesscutaneous lump. The lump was excised with the clinical impression of a sebaceous cyst. On microscopy,a poorly circumscribed, mild to moderately atypical spindle cell lesion in fascicular and storiformpatterns with strikingly myoid-like eosinophilic cytoplasm was identified. The spindle cells werehighlighted by pancytokeratin AE1/AE3, CD31, and ERG with retained INI-1, while being negativefor MNF116, S100, CD34, EMA, desmin, SMA, caldesmon, myogenin, MyoD1, HHV-8 and CD163.Following the first diagnostic report, a positron emission tomography–computed tomography(PET-CT) scan revealed another 4 cm ill-defined nodule accompanied by a smaller adjacent 0.7cm ipsilateral satellite nodule within the right psoas muscle that displayed similar morphologyand immunophenotype as the cutaneous lump, supporting the multicentric feature of this uniqueentity. It is an uncommon yet increasingly recognised neoplasm of endothelial origin possessing amisleading myoid morphology and distinctive immunophenotype worth notifying.

A Case of Intraosseous Hemangioma of the Maxilla Bone / 대한이비인후과학회지

Intraosseous hemangioma is a rare tumor, occurring especially in the maxilla. Because hemangioma is a benign tumor with increased vascularity, some authors call it hamartoma. This tumor originates and expands into bony structures. We experienced a female patient with intraosseous hemangioma of the right maxilla. The tumor was completely removed through a gingivobuccal incision and the feeding vessel was embolized. We report this case regarding the radiologic work-up, differential diagnosis, pathology and treatment with a brief review of the literature.

Hibernoma: tumor retroperitoneal de grasa parda: Informe de un caso pediátrico / Hibernoma: brown fat retroperitoneal tumor: Report of a pediatric case

El hibernoma es un raro tumor benigno de los tejidos blandos, compuesto por grasa parda, tejido que predomina en animales que hibernan y de allí su nombre. Debido a su infrecuencia en Pediatría y a su diagnóstico complejo, presentamos un paciente de 3 meses de edad, con diagnóstico de tumor abdominal. Los exámenes por ecografía y tomografía computada demostraron un tumor retroperitoneal de carácter infltrativo, vascularizado y con características lipomatosas. La exéresis del tumor permitió arribar al diagnóstico histopatológico de hibernoma o tumor de grasa parda. En esta presentación se describen las características del hibernoma, como tumor infrecuente en niños, y se revisan los tumores de tejido graso, según su frecuencia en Pediatría.

Hibernoma is a rare benign tumor of soft tissue, composed of brown fat. This tissue is predominant in hibernating animals and hence its name. Because of its rarity in Pediatrics and diffcult diagnosis, we report a 3 month-old patient with a diagnosis consistent with an abdominal tumor. Ultrasound and computed tomography exams showed an infltrative retroperitoneal tumor, with hypervascular and lipomatous features. After tumor excision, histopathological exam confrmed the diagnosis of hibernoma or brown fat tumor. This presentation describes the characteristics of this type of tumor, rare in children, and reviews the fatty tumors, according to their frequency in pediatric patients.

Venous Hemangioma Mimicking Mediastinal Solid Mass: A case report / 대한흉부외과학회지

An occurrence of hemangioma in the mediastinum is a very rare, accounting for less than 0.5% of mediastinal tumors. Capillary hemangiomas and cavernous hemangiomas consists of over 90% of mediastinal hemangiomas. However, venous hemangioma has never been reported in South Korea and has also very rarely been reported worldwide. We found mediastinal solid mass, as an incidental finding during a follow-up chest CT scan of a 44-year-old female patient who had undergone colon cancer surgery. We performed a mediastinoscopic biopsy. We did a thoracotomy to remove this mass because hemorrhage and found a totally resected venous hemangioma.

A Case of Duodenal Gastrointestinal Stromal Tumor Mimicking a Vascular Neoplasm / 대한소화기내시경학회지

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal neoplasm of the gastrointestinal tract. They are preferentially located in the stomach and small intestine. However, the duodenum is an unusual location for GIST. Here we present a case of a 67-year-old woman with a GIST of the 3rd portion of duodenum mimicking vascular neoplasm as an obscure acute gastrointestinal bleeding. The upper gastrointestinal endoscopy and colonoscopy failed to find the lesion. Finally, a large protruded lesion with ulcer was found at the 3rd portion of duodenum using a colonoscope through the oral approach. A spurting bleeding was developed during hypertonic saline epinephrine injection for treatment of oozing bleeding at the margin of the ulcer. Abdominal 3D CT-angiography showed a round and hypervascular structure at the posterior wall of duodenum. A wedge resection of the third portion of the duodenum was performed. Microscopic findings revealed GIST.

Epithelioid hemangioma of the colon: a case report / Hemangioma epitelióide do cólon: relato de caso

CONTEXT: Epithelioid hemangioma or angiolymphoid hyperplasia with eosinophilia is an uncommon benign vascular neoplasm that is usually located on the face or neck. Exceptionally, it has been described affecting the colon, with only two such cases described in the worldwide literature. The aim here was to present a case of primary epithelioid hemangioma of the sigmoid colon with confirmation by immunohistochemical examination. CASE REPORT: A 37-year-old woman had had a complaint of intermittent abdominal pain for six months. Two months after the condition started, she began to present changes in her intestinal habit, with evacuations containing blood and mucus and a weight loss of 4 kg over this period. At physical examination, a palpable mass was noted in the lower left quadrant of the abdomen. Neoplasia of the colon was clinically suspected and she underwent colonoscopy. This demonstrated the presence of a vegetating sessile lesion of approximately 5 cm in diameter, at a distance of 36 cm from the anal margin. It occupied 80 percent of the intestinal lumen. A biopsy collected during the examination suggested a diagnosis of neoplasia of vascular origin. After surgical resection, histopathological examination of the resected specimen confirmed the diagnosis of epithelioid hemangioma of the colon, which was backed up by the immunohistochemical panel (factor VIII, Ki-67, CD-34). At present, three years after the surgery, the patient is asymptomatic, she has recovered her normal weight and she has normal findings from control colonoscopy. Despite the rarity of neoplasia of vascular origin, this possibility should be considered in the differential diagnosis for colorectal tumors.

CONTEXTO: Hemangioma epitelióide ou hiperplasia angiolinfóide com eosinofilia são neoplasias vasculares benignas raras, habitualmente localizadas na face e pescoço. O acometimento do intestino grosso é excepcionalmente descrito, existindo apenas dois casos descritos na literatura mundial. O objetivo deste artigo é apresentar um caso de hemangioma epitelióide primário do sigmóide com diagnóstico histopatológico confirmado por meio de estudo imunoistoquímico. RELATO DE CASO: Mulher de 37 anos apresentou queixa de dor abdominal de forte intensidade, intermitente, localizada no hipogástrio. Dois meses após o início do quadro, notou alteração do hábito intestinal, evacuações com sangue, muco e perda ponderal de 4 quilos no período. Ao exame físico abdominal, identificou-se massa palpável no quadrante inferior esquerdo. Com suspeita clínica de neoplasia de cólon foi submetida a colonoscopia, que demonstrou presença de lesão vegetante de aproximadamente cinco centímetros de diâmetro, ocupando cerca de 80 por cento da luz colônica. A biópsia mostrou a presença de neoplasia de origem vascular. Após a ressecção cirúrgica, o exame histopatológico do espécime extirpado estabeleceu o diagnóstico de hemangioma epitelióide do cólon, confirmado por meio de painel imunoistoquímico (fator VIII, Ki-67, CD-34). No momento, a paciente encontra-se bem, tendo recuperado o peso inicial três anos após a cirurgia e apresenta resultado de colonoscopia de controle normal. Não obstante a raridade, deve-se considerar a possibilidade das neoplasias de origem vascular no diagnóstico diferencial dos tumores colorretais.

Intravenous Leiomyomatosis extending to Right Atrium: A case report / 대한흉부외과학회지

Intravenous leiomyomatosis is a rare neoplasm characterized by intravenous growth of histologically benign smooth muscle cell tumor. We report a case of intravenous leiomyomatosis with right atrial extension in a 19-year-old woman. Various surgical techniques and approaches have been previously reported. In this case, the tumor was removed with a single-stage approach via laparotomy without cardiopulmonary bypass.

Surgical Treatment of Pulmonary Artery Sarcoma: One case report / 대한흉부외과학회지

We report a rare case of pulmonary artery sarcoma mimicking pulmonary artery thromboembolism in a 57-year-old man who suffered with 2-month dyspnea and exacerbated for 1 week. He was transferred from private clinic and he was diagnosed as acute pulmonary artery thromboembolism on the basis of chest CT. Chest CT, pulmonary artery angiogram, and perfusion scan were examined. We performed surgical excision with aid of CPB. The final pathologic report was that the mass was a pulmonary artery sarcoma. We experienced one case of pulmonary artery sarcoma and reported it with reference.

Primary Pulmonary Artery Osteosarcoma Mimicking Acute Pulmonary Artery Embolism: A case report / 대한흉부외과학회지

The estimated relative incidence of primary pulmonary sarcoma to lung cancer is 0.4%. Furthermore, osteogenic sarcoma of the pulmonary artery is extremely rare. We report a case of a 63-year-old woman who visited our emergency room with the chief complaints of chest pain, dyspnea and dizziness. On echocardiography, right heart failure due to acute pulmonary artery embolism was diagnosed and we performed emergency operation. After opening the main pulmonary artery trunk, we found a mass attached to the arterial wall and massive thrombi around the mass. The mass was diagnosed as primary pulmonary artery osteosarcoma through postoperative evaluation. The patient received chemotherapy and radiotherapy. The patient is alive without specific symptoms 16 months postoperatively.

Primary Pulmonary Artery Osteosarcoma Mimicking Acute Pulmonary Artery Embolism: A case report / 대한흉부외과학회지

The estimated relative incidence of primary pulmonary sarcoma to lung cancer is 0.4%. Furthermore, osteogenic sarcoma of the pulmonary artery is extremely rare. We report a case of a 63-year-old woman who visited our emergency room with the chief complaints of chest pain, dyspnea and dizziness. On echocardiography, right heart failure due to acute pulmonary artery embolism was diagnosed and we performed emergency operation. After opening the main pulmonary artery trunk, we found a mass attached to the arterial wall and massive thrombi around the mass. The mass was diagnosed as primary pulmonary artery osteosarcoma through postoperative evaluation. The patient received chemotherapy and radiotherapy. The patient is alive without specific symptoms 16 months postoperatively.

Pulmonary Artery Sarcoma: One Case Report / 대한흉부외과학회지

Pulmonary artery sarcoma is a rare disease and hard to diagnose; therefore, suspicion is very important for the diagnosis and treatment. Surgical resection is almost always needed because of progressive right heart failure. Adjuvant chemotherapy and radiation therapy are still controversial. We report a case of a 42-year-old man who had a right pulmonary arterial tumor. Curative resection was impossible because the tumor invaded the left pulmonary artery. Palliative endarterectomy was performed followed by radiation therapy. The patient refused the chemotherapy. Until the postoperative 6th month, the residual tumor was stable. However, 15 months later, follow-up chest computed tomography revealed a metastatic pulmonary nodule at left lower lobe and the increased residual tumor. The patient received chemotherapy with limited tumor response. The metastatic nodule and residual tumor did not increase but bone scan revealed a rib metastasis at postoperative 24 months. He will be receiving additional chemotherapy.

Surgical Experience of Pulmonary Vascular Tumor: 2 cases / 대한흉부외과학회지

Tumors of vascular origin are subdivided into two groups: those composed of pericytes (hemangiopericytoma and glomus tumor), and those composed of endothelial cells(hemangioendothelioma). They are uncommon, potentially malignant tumors, and in the lung, the tumors may present as a small asymptomatic nodule or a large symptomatic lesion. Recently we experienced two cases of solitary pulmonary vascular tumors(epithelioid hemangioendothelioma and hemangiopericytoma), and reviewed them with references

A Case of Spontaneous Hematomyelia

This is a report of 23 year old soldier who developed sudden onset of flaccid paraplegia and loss of all sensory modalited below the level of T4. The clinical entities of hematomyelia were sudden onset of pain, repidly developing pyramidal signs and paraplegia. The mainly involving sites of this disease were cervical and thoracic regions. The causative factors were hemorrhagic diasthesis, vascular malformation and inflammatory process as well as trauma and neoplasms affecting the spinal cord. We experienced a case of hematomyelia which did not combine with vascular anomaly, inflammatory process, hemophilia or trauma history.