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Hemangioendothelioma

Hemangioendothelioma is used to describe a group of vascular neoplasms that may be considered benign or malignant in their activity. They have been described as masses that fall between a hemangioma and angiosarcoma. They are vascular tumors that commonly present with an enlarging mass and have been reported in the head and neck, intestines, lungs, lymph nodes, pleura, retroperitoneum, stomach, and many other body sites. Surgical resection, radiotherapy, and chemotherapy have all been used to treat these masses.

Types of Hemangioendotheliomas

  • Epithelioid
  • Kaposiform
  • Retiform
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Pulmonary epithelioid hemangioendothelioma : an unusual case and a review of the literature - selected reports
From CHEST, 2/1/04 by Paul Cronin

We describe a case of pulmonary hemangioendothelioma, previously known as intravascular bronchoalveolar tumor, in a 35-year-old woman with an initial diagnosis made by transbronchial biopsy. This is a rare disease, with approximately 50 cases described in the literature. All previous cases have been diagnosed by surgical lung biopsy. Although our patient underwent thoracoscopic lung biopsy, the diagnosis was initially made on transbronchial biopsy; to our knowledge, this has not been previously described in the English-language literature. We also described findings on high-resolution CT, both typical and atypical relative to previously published reports. This tumor can affect multiple organs. The prognosis is very unpredictable, with life expectancy ranging from 1 to 15 years. There is no single effective treatment, though spontaneous regression and response to chemotherapy and interferon are reported.

Key words: epithelioid hemangioendothelioma; high-resolution CT; transbronchial biopsy

Abbreviations: EHE = epithelioid hemangioendothelioma; GGO = ground-glass opacity; HRCT = high-resolution CT

**********

We describe a case of pulmonary epithelioid hemangioendothelioma (EHE), previously known as intravascular bronchoalveolar tumor, in a 35-year-old woman with an initial diagnosis made with transbronchial biopsy. This is a rare disease, with approximately 50 cases described in the literature. (1) All previous cases have been diagnosed by surgical lung biopsy. Although our patient underwent thoracoscopic lung biopsy, the diagnosis was initially made by transbronchial biopsy. We also described findings on high-resolution CT (HRCT), both typical and atypical relative to previously published reports. This tumor can affect multiple organs, and has a prognosis that is unpredictable, with life expectancy ranging from 1 to 15 years. Our patient eventually died of progressive respiratory failure.

CASE REPORTS

Our patient is a 35-year-old woman referred with an 18-month history of shortness of breath and dry cough. She was previously treated for presumed asthma with bronchodilators, which initially improved her symptoms. However, over the 3 months prior to presentation, she had increasing shortness of breath. She had no prior medical history, was a nonsmoker, and denied any history of exposure to pets or birds. Her mother had lung cancer, but there was no other family history of cancer. On physical examination, she was not clubbed and there were no abnormally enlarged lymph nodes. Lung examination revealed only inspiratory crackles at the bases without wheezing or evidence for consolidation. Her pulmonary function tests revealed FE[V.sub.1] of 1.47 L, (39% of predicted), FVC of 1.5 L (40% of predicted), total lung capacity of 2.95 L (56% of predicted), and diffusion capacity of the lung for carbon monoxide of 10.20 mL/mm Hg/min (41% of predicted).

The working diagnosis was that of an interstitial lung disease or hypersensitivity pneumonitis. CBC count and liver function test results were normal, as was a hypersensitivity pneumonitis screen, Aspergillus precipitins, autoantibodies, and angiotensin-converting enzyme. HRCT and bronchoscopy were performed.

HRCT showed several, approximately 5-ram, well-demarcated noncalcified nodules predominantly in the lung bases, as well as interlobular septal thickening and small pleural effusions. However, there were also mosaic areas of ground-glass opacity (GGO) diffusely in both lungs, and airspace disease predominantly within the left lower lobe. There were no enlarged lymph nodes. The appearance was believed to be most compatible with either an infectious or inflammatory etiology (Fig 1).

[FIGURE 1 OMITTED]

Bronchoscopy was performed with BAL and transbronchial lung biopsy from the middle lobe and the right lower lobes, respectively. The transbronchial lung biopsy showed histologic features of EHE. Immunohistochemical stains demonstrated that the cells were positive for CD31, equivocally positive for CD34, and negative for cytokeratin, supporting a diagnosis of EHE (Fig 2, top and center). This was also confirmed with a left upper lung lobe thoracoscopic wedge biopsy (Fig 2, bottom). Again, histologic features were that of EHE with confirmatory CD31, CD34, factor VIII, and CD1A immunohistochemical stains.

[FIGURE 2 OMITTED]

As this disease can be multifocal or can metastasize, a standard helical contrast-enhanced chest and abdominal CT was also performed, to better stage the disease. There was no evidence of either multiorgan disease or metastases.

DISCUSSION

Dail et al (2) first described EHE in 1975. Initially, this was believed to be an aggressive titan of bronchoalveolar cell carcinoma that invaded adjacent blood vessels, hence the name intravascular bronchoalveolar tumor. Corrin and coworkers (3) employed immunohistochemical techniques to demonstrate that the tumor cells were from a lineage capable of differentiation along endothelial lines. Weldon-Linne et al (4) confirmed this using electron microscopy and applied immunohistochemistry. Electron microscopic features, and immunochemistry showing diffuse cytoplasmic staining of the malignant cells with factor VIII-related antigen confirmed the endothelial lineage of tumor cells. Weiss et al (5) were the first to offer the term epithelioid hemangioendothelioma. It is a rare vascular tumor of borderline or low-grade malignancy. It can arise from many organ systems, including liver, bone, and soft tissues simultaneously or sequentially. When this occurs, it may be difficult to determine if the tumor is multicentric or is a primary lesion with metastases to other tissues. Rarely, the lungs are involved, with only 50 cases of pulmonary EHE described in the literature. (1)

EHE is often diagnosed incidentally, as patients are usually asymptomatic or have minor symptoms at the time of diagnosis. It is four times more common in women than men, with patients ranging in age from 12 to 60 years, with a mean onset of 35 years; 50% are < 40 years old. Patients may live for up to 15 or 20 years. However, cases in which the behavior is more aggressive have been reported. (2,6) Patients with fibrinous pleuritis and extrapleural proliferation of tumor cells or spindle tumor cells generally have a much worse prognosis. (6) Kitaichi and coworkers (6) investigated 21 Asian patients with pulmonary EHE and found two of the three patients with pleural effusion died within 1 year, while the 16 patients with no effusion were alive > 1 year later, and two patients with fibrinous pleuritis and extrapleural proliferation of tumor cells died within 2 years, while only one of 14 patients lacking such manifestations died within the same period. However, they also found partial spontaneous regression in three asymptomatic patients 5 years, 13 years, and 15 years after detection.

Dail and colleagues (2) reviewed 20 patients and found those with extensive intravascular, endobronchial, or pleural spread had a worse prognosis, as did those with those with liver nodules and peripheral lymphadenopathy. (6) Boudousquie and coworkers (8) described several clonal abnormalities in a series of patients with EHE, including a complex unbalanced translocation between chromosome 7 and 22 with multiple breakpoints, a robertsonian translocation of chromosome 14 and loss of the Y chromosome; monosomy for chromosome 11 was noted in a subset of the tumor cells.

The most characteristic feature of EHE on chest radiograph or CT is the presence of multiple perivascular nodules with well- or ill-defined margins in both lungs. The nodules range in size up to 2 cm, but most are < 1 cm in diameter. They are usually found in relation to small and medium-sized vessels and bronchi. (7) In a review of 20 eases, Dail et al (2) showed 20% of patients had < 10 nodules, 25% had [greater than or equal to] 20 nodules, and the rest were somewhere in-between. This presentation is often mistaken for metastatic carcinoma, which is the initial radiologic interpretation in nearly all cases. (1) However, they show little or no growth on serial chest radiographs or CTs. Other differential diagnoses for this radiologic presentation include chronic granulomatous disease, less commonly multiple benign tumors, as they are quite rare, and vasculitis or bronchiolitis rarely. (2) While histologic calcification and ossification is common, radiologic calcification is not. Ross et al (9) reviewed 36 cases and found that 9% of patients had hilar lymph node enlargement and 10% bad pleural effusions. Hilar lymph node metastases have been described, (2) as has interlobular septal thickening. (7) Patients with multiorgan disease or metastatic disease within the liver and hilar lymph nodes have a worse prognosis. CT should be performed to evaluate the liver and intrathoracic lymph nodes as well as the pulmonary parenchyma in all patients.

Our patient's HRCT showed several, approximately 5-ram, well-demarcated noncalcified nodules predominantly in the lung bases, and a small right pleural effusion, findings compatible with those previously described in the literature. However, there also were mosaic areas of GGO diffusely in both lungs and interlobular septal thickening, and airspace disease predominantly within the left lower lobe. Mukundan and colleagues (1) reported GGO as well as interlobular septal thickening on HRCT in one patient with pulmonary EHE; in their patient, GGO and interlobular septal thickening predominated, mimicking the appearances of diffuse interstitial lung disease, and nodules were not present. They attributed the atypical radiologic appearance to the pathologic finding of small and infiltrating nodular tumor proliferation within the lumen of arteries and veins, rather than the more typical pattern of interstitial nodules. To our knowledge, our patient's HRCT findings represent the first reported case of pulmonary EHE with both typical CT features such as multiple nodules and pleural effusions, and less typical CT features such as GGO and interlobular septal thickening. Airspace disease is not previously described.

Although the transbronchial lung biopsy specimen showed histologic features of EHE, all previously reported cases of pulmonary EHE in the literature were diagnosed using open-lung or thoracoscopic biopsy specimens. For this reason, our patient went to surgery and underwent a thoracoscopic wedge resection of the left upper lobe that confirmed the diagnosis of EHE.

Because of the rarity of this condition, there is no clear standard for treatment, but partial spontaneous regression has been described in three patients, between 5 years and 15 years after detection. (5) Complete response to six courses of carboplatin plus etoposide chemotherapy in a patient with pleural EHE is also described, with full remission at 18-month follow-up. (10) There are several case reports of patients with EHE achieving partial or more complete remission after treatment with interferon. (11-13) Two of these patients had pulmonary involvement as part of more widespread disease. (14)

Our patient was referred to an oncologist specializing in the management of soft-tissue sarcomas. She initially received parenteral treatment with interferon-[alpha]2b, 1.5 million units three times weekly, and remained stable, with dyspnea and restrictive pulmonary function. However, her disease progressed and she was started on gemcitabine and docetaxel chemotherapy. An episode of respiratory failure requiting intubation and hospitalization developed after the first cycle. Further treatment with gemcitabine and docetaxel chemotherapy was attempted, but tachypnea subsequently developed and the patient was again admitted to hospital and intubated. After recovering from this, due to the difficulty with IV chemotherapy, the patient was started on an oral regimen including cyclophosphamide and etoposide. Her condition deteriorated further, and she died 9 months after her initial diagnosis.

CONCLUSION

In summary, we describe a case of pulmonary EHE diagnosed by transbronchial biopsy. We conclude this diagnosis can be made by transbronchial biopsy, and open-lung or thoracoscopic biopsy is not necessarily required, potentially sparing patients the morbidity and mortality associated with surgical lung biopsy. We also describe HRCT features, both typical (such as multiple nodules and pleural effusions) and less typical (airspace disease, GGO and interlobular septal thickening). We conclude that CT features of infectious or inflammatory lung diseases are compatible with a diagnosis of pulmonary EHE.

ACKNOWLEDGMENT: We thank Dr. A Flint, Department of Pathology, University of Michigan Health Systems, for assistance with the pathology illustrations.

REFERENCES

(1) Mukundan G, Urban BA, Askin FB, et al. Pulmonary, epithelioid hemangioendothelioma: atypical radiologic findings of a rare tumor with pathologic correlation, J Comput Assist Tomogr 2000; 24:719-720

(2) Dail DH, Liebow AA, Gmelich JT, et al. Intravascular, bronchiolar, and alveolar tumor of the lung (IVBAT): an analysis of twenty cases of a peculiar sclerosing endothelial tumor. Cancer 1983; 51:452-464

(3) Corrin B, Manners B, Millard M, et al. Histogenesis of the so-called "intravascular bronchioloalveolar tumour." J Pathol 1979; 128:163-167

(4) Weldon-Linne CM, Victor TA, Christ ML, et al. Angiogenic nature of the "intravascular bronchioloalveolar tumor" of the lung: an electron microscopic study. Arch Pathol Lab Med 1981; 105:174-179

(5) Weiss SW, Ishak KG, Dail DH, et al. Epithelioid hemangioendothelioma and related lesions. Semin Diagn Pathol 1986; 3:259-287

(6) Kitaichi M, Nagai S, Nishimura K, et al. Pulmonary epithelioid haemangioendothelioma in 21 patients, including three with partial spontaneous regression. Eur Respir J 1998: 12:89-96

(7) Luburich P, Carmen Ayuso M, Picado C, et al. CT of pulmonary epithelioid hemangioendothelioma. J Comp Assist Tomogr 1994; 18:562-565

(8) Boudousquie AC, Lawce HJ, Sherman R, et al. Complex translocation [7;22] identified in an epithelioid hemangioendothelioma. Cancer Genet Cytogenet 1996; 92:116-121

(9) Ross GJ, Violi L, Friedman AC, et al. Intravascular bronchioloalveolar tumor: CT and pathologic correlation. J Comp Assist Tomogr 1989; 13:240-243

(10) Pinet C, Magnau A, Garbe C, et al. Aggressive form of pleural epithelioid haemanginendothelioma: complete response after chemotherapy. Eur Respir J 1999; 14:237-238

(11) Kayler LK, Merion RM, Arenas JD, et al. Epithelioid hemangioendothelioma of the liver disseminated to the peritoneum treated with liver transplantation and interferon [alpha]-2B. Transplantation 2002; 74:128-130

(12) Rosenthal DI, Treat ME, Mankin HJ, et al. Treatment of epithelioid hemangioendothelioma of bone using a novel combined approach. Skeletal Radiol 2001; 30:219-222

(13) Vignon-Pennamen MD, Rybojad M, Verola O, et al. Epithelioid hemangioendothelioma: disease course in 3 cases. Ann Dermatol Venereol 1997; 124:165-166

(14) Roudier-Pujol C, Enjolras O, Lacronique J, et al. Multifocal epithelioid hemangioendothelioma with partial remission after interferon [alpha]-2a treatment. Ann Dermatol Venereol 1994; 121:898-904

* From the Department of Radiology, Division of Thoracic Radiology (Dr. Cronin), and Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine (Dr. Arenberg), University of Michigan Medical School, Ann Arbor, MI. Manuscript received January 31, 2003; revision accepted September 12, 2003.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail: permissions@chestnet.org).

Correspondence to: Douglas Arenberg, MD, FCCP, Department of Internal Medicine, Pulmonary and Critical Care Medicine, University of Michigan Medical School, 1150 West Medical Center Dr, 6301 MSRB III Box 0642, Ann Arbor, MI 48109; e-mail: darenber@umich.edu

COPYRIGHT 2004 American College of Chest Physicians
COPYRIGHT 2004 Gale Group

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