• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• v.42 no.5
• /
• pp.307-314
• /
• 2016

We report a case of retiform hemangioendothelioma (RH) located in the infratemporal fossa and buccal area in a 13-year-old Korean boy. The tumor originated from the sphenoid bone of the infratemporal fossa area and spread into the cavernous sinus, orbital apex, and retro-nasal area with bone destruction of the pterygoid process. Tumor resection was conducted via Le Fort I osteotomy and partial maxillectomy to approach the infratemporal fossa and retro-nasal area. The diagnosis of RH was confirmed after surgery. In the presented patient, surgical excision was incomplete, and close follow-up was performed. There was no evidence of expansion or metastasis of the residual tumor in the 8 years after surgery. In cases of residual RH with low likelihood of expansion and metastasis, even though RH is an intermediate malignancy, close follow-up can be the appropriate treatment choice over additional aggressive therapy. To date, 29 papers and 48 RH cases have been reported, including this case. This case is the second reported RH case presenting as primary bone tumor and the first case originating in the oromaxillofacial area.

• Tuberculosis and Respiratory Diseases
• /
• v.40 no.3
• /
• pp.301-307
• /
• 1993

Intravascular bronchioloalveolar tumor is now recognized as a pulmonary form of hemangioendothelioma(HE). HE is an unusual tumor of adult life which is characterized by proliferation of an "epithelioid" or "spindle" endothelial cell. In the lung it usually presents as multiple bilateral slowly growing nodules less than 2 cm in diameter. The aetiology and pathogenesis of this disease are unknown. Spindle cell HE occurs at any age, but approximately one half of patient are 25 years of age or younger and males are affected twice more frequently than females. On light microscopic examination, the tumor show mild cellular atypia, nearly absent mitoses and electron-microscopic studies reveal evidence of endothelial cell differentiation. Intracytoplasmic localization of Factor VIII-related antigen is demonstrated on immunohistochemical study, which confirmed the endothelial origin of the tumor. No effective therapy is yet known for HE, but survival of this tumor can be quite long. However, one half of the patient have died, usually of progressive pulmonary insufficiency. This 19-yr-old male complained of Rt. chest pain and intermittent hemoptysis. Simple chest film and chest CT scan showed the Rt. pleural effusion, variable sized bilateral pulmonary nodules, irregular large heterogenous tumor with well enhancement and extensive necrosis in the anterior mediastinum. The mediastinal mass was biopsied and diagnosed as spindle cell HE by light microscopic finding and immunohistochemical studies.

• Clinical and Experimental Pediatrics
• /
• v.54 no.6
• /
• pp.260-266
• /
• 2011

Purpose: Infantile hepatic hemangioendothelioma (IHHE) is the most common type of hepatic vascular tumor in infancy. We conducted this study to review our clinical experience of patients with IHHE and to suggest management strategies. Methods: We retrospectively analyzed the medical records of 23 IHHE patients (10 males, 13 females) treated at the Asan Medical Center between 1996 and 2009. Results: Median age at diagnosis was 38 days (range, 1 to 381 days). Seven patients (30%) were diagnosed with IHHE based on sonographically detected fetal liver masses, 5 (22%) were diagnosed incidentally in the absence of symptoms, 5 (22%) had congestive heart failure, 3 (13%) had skin hemangiomas, 2 (9%) had abnormal liver function tests, and 1 (4%) had hepatomegaly. All diagnoses were based on imaging results, and were confirmed in three patients by histopathology analysis. Six patients were observed without receiving any treatment, whereas 12 received corticosteroids and/or interferonalpha. One patient with congestive heart failure and a resectable unilobar tumor underwent surgical resection. Three patients with congestive heart failure and unresectable tumors were managed by hepatic artery embolization with/without medical treatment. At a median follow-up of 29 months (range, 1 to 156 months), 21 (91%) patients showed complete tumor disappearance or >50% decrease in tumor size. One patient died due to tumor-related causes. Conclusion: IHHE generally has a benign clinical course with low morbidity and mortality rates. Clinical course and treatment outcome did not differ significantly between medically treated and non-treated groups. Surgically unresectable patients with significant symptoms may be treated medically or with hepatic artery embolization.

• Advances in pediatric surgery
• /
• v.14 no.2
• /
• pp.134-143
• /
• 2008

Infantile hepatic hemangioendothelioma (IHHE) is the most common benign vascular hepatic tumor in children. We analyzed the 17-year experience of IHHE. The medical records of 16 patients (M:F=8:8) treated at the Department of Pediatric Surgery and the Department of Pediatrics Seoul National University Children's Hospital between January 1991 and January 2008 were reviewed retrospectively. Mean age at presentation was 87 days (1 day - 551 days). Seventy five percent of patients were diagnosed with imaging study and 25 % with biopsy. Major symptoms were hepatomegaly (N=5), palpable abdominal mass (N=4) and congestive heart failure (N=3). Six patients had no symptoms. Kasabach-Merritt syndrome was combined in one patient. Nine patients (56.3 %) underwent operation and 2 patients (12.5 %) underwent only medical treatment. Clinical observation was tried on 5 patients (31.3 %) without any treatment. Operation was performed on the patient with clinical symptoms or on patients where the differentiation between begin and malignant could not be determined. Patients who had clinical symptoms but tumor was unreresectabile were treated medically. Among the 5 patients who had been observed for their clinical course, 2 patients showed complete regression and the tumors of the remaining 3 patients were regressing. Clinical symptoms, the age at presentation, the size of tumor and ${\alpha}$-FP, all had no significant statistical relationship with the time required for complete tumor regression. There was no relationship between the size change of the tumor and the change of ${\alpha}$-FP level. Only the size of tumor was related with clinical symptoms. One patient died of post-operatvie bleeding. Treatment plan was determined by the extent of the tumor and the presence of clinical symptoms. Observation was enough for the patients without clinical symptoms and complete resection was curative for patients with clinical symptoms. Medical treatment is an alternative for the patient whose tumor is unresectable.

• Tuberculosis and Respiratory Diseases
• /
• v.44 no.6
• /
• pp.1396-1402
• /
• 1997

Epithelioid Hemangioendothelioma(EH) is a rare vascular tumor, originating from endothelial cells. The principal locations are lung, soft tissue, bone and liver. This tumor is of borderline malignancy, relatively benign course. In the lung, the tumor is often multifocal, bilateral and frequently lead to the mistaken diagnosis of metastatic carcinoma. Although EH of the lung is relatively slow growing tumor, extensive pulmonary involvement, systemic metastasis, mainly to the liver have been documented. A 26-year-old man with EH involving the lung and liver was reported. Chest X-ray and chest CT showed multiple nodules in both lung fields and Abdominal CT multiple round low densities in liver. Transbronchial lung biopsy was performed. The patient diagnosed as EH by light microscopic finding and immunohistochemical study for Factor VIII-related antigen.

• Journal of the Korean Society of Radiology
• /
• v.83 no.4
• /
• pp.958-964
• /
• 2022

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of borderline or low-grade malignancy, and its prognosis is unpredictable. Herein, we describe the case of a 47-year-old asymptomatic female with a diagnosis of multinodular PEH. During a 7-year follow-up, the nodules with large size and high ¹⁸F-fluorodeoxyglucose uptake in the initial study showed progression with increasing sizes; however, most small nodules (size < 1 cm) demonstrated spontaneous regression with peripheral rim or nodular calcification. The patient underwent surgical resection for an enlarged nodule. Of note, it is unusual for an individual to have mixed progression and regression concomitantly, which may be helpful in predicting the prognosis.

• Neonatal Medicine
• /
• v.15 no.2
• /
• pp.183-189
• /
• 2008

Infantile hepatic hemangioendotheliomas (IHHEs) are benign vascular tumors, but can be associated with the life-threatening complications, such as congestive heart failure, disseminated intravascular coagulation, and massive bleeding. Various therapeutic options have been developed and the treatment response depends on the patient's clinical status and the nature of the lesion. In the case of a symptomatic IHHE, a non-invasive and precise diagnosis should be performed promptly before the therapeutic method is chosen. Additionally, it should be kept in mind that the residual lesions have malignant potential. We report a case of a congenital giant IHHE that was successfully reduced in size by interferon-$\alpha$ and completely removed by surgical tumor resection with a hepatic lobectomy.

• Journal of Chest Surgery
• /
• v.30 no.6
• /
• pp.631-635
• /
• 1997

혈관에서 기원되는 종양은 혈관외피 세포로부터 발생되는 종양 혈관외피종과 glomus tumor)관 혈관내피 세포로부터 발생되는 종양(를상피 혈관내피종)의 두가지로 구분된다. 이들은 매우 드물게 발생되며 악성종양의 특성을 가지고 있는데, 폐에서 발생된 경우에는 무증상의 작은 종괴로부터 증상이 있는 커다란 종양 의 형태로 나타난다. 최근 저자들은 단일성의 유상피 혈관내피종과 혈관외피종 각각 1례를 치험하였기에 문헌고찰과 함께 보 고하는 바이다. a 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.

• Tuberculosis and Respiratory Diseases
• /
• v.53 no.1
• /
• pp.56-65
• /
• 2002

A pulmonary epithelioid hemangioendothelioma (PEH) is a rare tumor with a vascular origin. A PEH can arise in many organ systems, such as the lung, liver, bone and soft tissues. It is a borderline malignancy but the clinical course is usually benign. In this report, we describe three cases of PEH. Case 1, a 61-year-old man, had nonspecific chest discomfort and the chest X-ray showed a solitary lung nodule. This nodule was diagnosed by an open lung biopsy. The pathologic findings including abundant necrosis, mitosis and hyperchromatic and pleomorphic nuclei, indicated a malignancy. The electron microscopic study showed Weibel-Palade bodies and the immunohistochemical stain for CD31 showed a positive reaction in the tumor cells, and linear staining along the vascular lumina. Case 2, a 34-year-old man, was admitted for an evaluation of multiple small nodules, incidentally detected a screening chest X-ray. The nodules were diagnosed by a immunohistochemical stain for the factor VIII-related antigen. Case 3, a 34-year-old woman, complained of left pleuritic chest pain. A simple chest film and the chest CT scan revealed multiple bilateral nodules and a left pleural effusion. An immunohistochemical stain for the factor VIII-related antigen was used to diagnose the nodules. Forth-one months after the diagnosis, she died of pulmonary insufficiency.

• Tuberculosis and Respiratory Diseases
• /
• v.47 no.5
• /
• pp.691-696
• /
• 1999

Epithelioid hemangioendothelioma(EH) is a rare pulmonary vascular malignancy. Clinically, EH has been considered as an indolent, generally non-aggressive tumor. We report a case of EH which was confirmed by open lung biopsy. A 34-year-old woman was admitted for further evaluation of multiple small(less than 2cm in size) nodules, incidentally detected on screening chest radiograph. The chest CT showed multiple, relatively well-marginated, variable sized nodules at both whole lung. Transbronchial lung biopsy and transthoracic needle aspiration were nondiagnostic and open lung biopsy was performed from right middle lobe of lung. On light-microscopic examination, the nodules were composed of a poorly cellular hyaline core and a more cellular peripheral zone which extended into air space in a micropolypoid fashion and obliterated blood vessels. The tumor cells at the peripheral zone had intracytoplasmic vacuoles which suggested primitive, vascular differentiation. Immuno-histochemical study revealed the cellular area which gave positive reaction to factor VIII-related antigen. She received no specific therapy after open lung biopsy and chest X-ray films had showed no change for about two years.