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In this article, we describe a case of conventional epithelioid hemangioendothelioma (EHE) arising within an extra-adrenal myelolipoma. This composite neoplasm arose in the mediastinum of a 51-year-old female. The tumor was composed of a large myelolipoma that contained nodules of EHE consisting of CD31-positive epithelioid endothelial cells that grew in solid cords and were enmeshed in a basophilic hyalinized stroma. Both EHE and myelolipoma are characterized genetically by alterations of WWTR1. We demonstrated the expression of CAMTA-1 chimeric protein by immunohistochemistry both in the neoplastic endothelial cells of EHE and some of the endothelial cells lining the blood vessels in the myelolipoma. To the best of our knowledge, this is the first report of a malignant vascular neoplasm arising in association with myelolipoma.

Extra-adrenal myelolipoma happens in adrenal glands, and the thoracic location is extremely unusual. This is the first study involving 36 of patients with thoracic myelolipoma of English literature by investigating the clinical data, pathologic findings, radiological manifestation, and treatment strategy of all patients. Imageologic diagnosis including computed tomography, magnetic resonance imaging, and positron emission tomography/computed tomography scans is useful to identify the feature of extra-adrenal myelolipoma. Pathologic analysis is an effective method to clarify the diagnosis. In view of the potential progressive enlargement of the lesion, most myelolipomas are removed by surgery, and this operation has frequently been accomplished by using video-assisted thoracic surgery., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Myelolipoma is a rare benign tumor composed of mature adipose tissue and normal hematopoietic tissue. Although surgical resection has been recommended due to the potential of progressive enlargement, the natural history of mediastinal myelolipoma has not yet been described. Herein we report a surgically resected mediastinal myelolipoma showing gradual enlargement over a period of 9 years., Case Presentation: A 70-year-old woman presented with a posterior mediastinal mass shadow detected by computed tomography (CT) examination. She had a medical history of sigmoidectomy for colon cancer 13 years previously. A CT scan showed a smooth, well-demarcated 2.8 × 2.1-cm paravertebral mass shadow, composed of a fat density area and a soft tissue density area, which showed gradual enlargement from a 1.6 × 1.0-cm nodule 9 years previously. This was not accompanied by chronic anemia or hematologic disease including thalassemia, and no abnormal accumulation was observed on bone marrow scintigraphy or fluoro-2-deoxyglucose positron emission tomography. With a clinical diagnosis of mediastinal myelolipoma, surgical resection was performed, and pathological examination confirmed the diagnosis., Conclusions: We experienced a rare case with mediastinal myelolipoma showing gradual enlargement, with a tumor doubling time of 1,212 days.

Symptomatic mediastinal location of an extra-adrenal myelolipoma is extremely rare. We describe a 56-year-old female with unusual pain in the right lower posterior chest radiated to the neck and to the upper abdomen. Chest CT showed a lesion of 3.5 x 2.2 cm in the posterior mediastinum. Video-assisted resection was performed and the final pathologic examination revealed the presence of a myelolipoma. Herein we discuss the clinical presentation, the differential diagnosis and treatment of mediastinal myelolipoma.

Myelolipoma is a benign tumour consisting of mature fat with scattered foci of haematopoietic elements resembling bone marrow. Extra-adrenal myelolipomas are an infrequent pattern of presentation. We report the case of a 64-year-old woman who presented a heterogeneous 1.4×2.5×3 cm paravertebral thoracic mass detected by chest tomography during the study of a leukocytosis of unknown aetiology. The CT findings and pathology revealed the diagnosis of myelolipoma.

Myelolipoma is a rare mesenchymal tumor composed of an admixture of fat and bone marrow-derived hematopoietic cells. It is typically an adrenal lesion, and mediastinal location is extremely unusual. We describe a 56-year-old man with nonspecific symptoms of cough and dyspnea and no underlying disease whose chest computed tomography showed a huge (25 x 15 cm) mediastinal tumor containing calcification. Surgical removal and subsequent pathologic examination of the tumor revealed myelolipoma. Primary myelolipoma should be considered in the differential diagnosis of large posterior mediastinal tumors containing calcification.

Extra-adrenal myelolipomas are rare, generally solitary, non-functioning tumors consisting of adipose tissue and hematopoetic elements. These lesions are not known to be associated with underlying hematologic disorders or documented bone marrow abnormalities. We describe the extremely rare occurrence of multifocal, extra-adrenal myelolipomas in a 35-yr-old male. A markedly hypocellular bone marrow is noted, despite normal peripheral blood counts. To our knowledge, this is the first case report of a markedly abnormal marrow examination in a patient with extra-adrenal myelolipoma, and implicates the extra-osseous masses as the driving force behind his normal hematopoesis.

Myelolipoma is a rare tumor, and a mediastinal location is extremely unusual. The main pathologic feature is the coexistence of mature adipose tissue and bone marrow cells; the presence of megakaryocytes is essential for diagnosis. The successful removal of a mediastinal myelolipoma in a 59-year-old man is described.

A 72-year-old man presented with a mediastinal mass on chest radiograph. Computed tomography and MR imaging showed that the mass consisted of both fatty and small nodular soft tissue components, highly suggestive of an extramedullary hematopoiesis or a myelolipoma. A CT-guided needle biopsy was next performed and confirmed the diagnosis. We discuss the CT and MR imaging appearances es of this tumor and usefulness of a CT-guided needle biopsy to avoid surgery in asymptomatic patients.

The authors report a case of extramedullary haematopoiesis, presenting in the form of several tumor masses in the posterior mediastinum and in the vertebral canal. The patient presented with oedema in his lower limbs due to compression of the inferior vena cava by the mediastinal masses. The asymptomatic mass in the vertebral canal was detected by magnetic resonance imaging. The patient was first operated on to avoid spinal cord compression by the tumor and subsequently to relieve compression of the inferior vena cava. The masses removed from the vertebral canal and the mediastinum were found on histologic examination to be extramedullary haematopoiesis. The cause of the disease was unknown in this case. Such disease is often disclosed in the course of hemolytic anaemias or other medullar deficiency diseases as a compensatory phenomenon when the bone marrow fails. An other explanation is that it is a manifestation of a myeloproliferative disorder in which heterotopic pluripotential cells are transformed into haematopoietic cells. The authors review 88 cases reported in the world literature.