Malignant epitheloid hemangioendothelioma

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Classification according to ICD-10
C49 Malignant growth of other connective and soft tissues
- blood vessels
ICD-10 online (WHO version 2019)

The malignant epithelioid hemangioendothelioma ( EHE ) is a very rare malignant vascular tumor , d. H. a malignant sarcoma of the supporting and connective tissues that originates from the endothelium of the blood vessels. It was only defined as an independent tumor entity in 1982 .

Localizations and Epidemiology

As a sarcoma, MARRIAGE can in principle arise in almost any place in the human body. It occurs mainly in the liver , lungs , heart , bones , spleen , lymph nodes , connective tissue , brain and meninges . Initial multifocal involvement within an organ is the rule - with the exception of tumors located in the connective tissue, which are usually solitary at diagnosis. Due to the rare occurrence and the difficult differential diagnosis, it is difficult to estimate the total incidence of this neoplasm . In analogy to the available data for the localization in the liver, an incidence of <1 / 1,000,000 without a clear age preference can be assumed, whereby women seem to be statistically slightly overrepresented , especially during treatment with hormonal contraceptives or during pregnancy. Exposure to asbestos , thorotrast , vinyl chloride , viral hepatitis and alcohol consumption are to be assumed as further risk factors .

Pathology and pathological differential diagnosis

As already described, EHE as a primary vascular tumor can in principle occur in any vascular location of the human body. In the liver, it usually shows a multifocal, invasive and displacing growth with several foci, some of which merge with one another, but - in contrast to hepatic angiosarcoma - typically no diffuse infiltration of the entire organ. EHE is a malignant tumor and, depending on its location, metastasizes to the blood ( hematogenic ) and lymphatic ( lymphogenic ) routes , including in the lungs, local lymph nodes and bones. Histologically , the hematoxylin-eosin stain cannot or can hardly be distinguished from an angiosarcoma, a sclerosing hemangioma , or the much more common bile duct carcinoma . Special histological features of EHE are primary atypia of the cells of the vascular endothelium and the formation of intracellular vacuoles , some of which are filled with erythrocytes . The degenerate endothelial cells show a certain morphological resemblance to cells of the multilayered uncornified squamous epithelium and form irregularly configured dysfunctional vascular structures that should not be confused with the secondary angiogenesis in other malignant tumors. The name "epitheloid hemangioendothelioma" results from the combination of these properties. The origin of the tumor cells from vascular structures can be proven in special stains (e.g. with antibodies against specific features of vascular cells). In this way the much more common carcinomas , which i. d. Usually do not wear, as a differential diagnosis to be excluded. A differentiation between the primary vascular tumors hemangioma (benign), hemangioendothelioma, and angiosarcoma (both malignant) can ultimately only be achieved by looking at the histology, the macroscopic tumor growth pattern and the growth rate (via clinical monitoring and / or with the help of histological growth markers).

Clinical picture

The clinical presentation of the patient is very variable and depends primarily on the tumor location. In the clinic, everything from asymptomatic incidental findings to fully developed systemic tumor disease occurs. For the comparatively frequent (hepatic) EHE, which is located in the liver, either no symptoms or rather unspecific symptoms such as tenderness on the right upper abdomen, hepatomegaly and B symptoms are reported.

Diagnostics and differential diagnoses

The suspicion of a malignant neoplasm is often based on the above. unspecific symptoms or an incidental finding during an examination for another cause. A targeted tumor search with imaging methods ( magnetic resonance tomography (MRT), computed tomography (CT), sonography ) can confirm this initial suspicion. Laboratory tests on the functions of the affected organs provide further information on tumor behavior. There are no established tumor markers that would allow a positive diagnosis of sarcoma of any differentiation. Thus, the ultimate diagnosis of EHE can only be made through invasive measures ( biopsy with sampling) and pathological assessment. Since EHE is a very rare entity , so there is little diagnostic experience in most clinics and the diagnosis can only be made by looking at all factors (histology, immunohistochemistry , imaging , clinical course), approx. 60–80% of the affected patients initially misdiagnosed. The most common misdiagnoses are sclerosing hemangioma and angiosarcoma, less often metastases from other tumors and primary carcinomas. In the case of masses in the liver, in addition to other benign or malignant neoplasms, cystic echinococcosis must be excluded.

Classification and Therapy

Body trunk sarcomas - the most common site of manifestation of EHE - are not classified according to the TNM classification.

Due to the rarity of EHE, there are no therapy guidelines in Germany. Therefore, there are and have been many approaches to treating this tumor, depending on its stage and location. In the case of hepatic EHE, these include surgical ( partial liver resection , liver transplantation ), chemotherapy and radiotherapeutic as well as local procedures ( radio frequency ablation ) or simply watch and wait. When choosing a therapy, the localization, extent and clinical condition of the patient must be taken into account. In particular, in contrast to angiosarcoma, cure is possible in principle even in the metastatic state (see “prognosis”) and that EHE is not a contraindication for liver transplantation. As with most solid tumors, permanent cure without surgery is unlikely.

forecast

The prognosis of the patient depends on the primary location of the tumor, the metastasis at the time of diagnosis, and the general clinical condition of the patient - the latter in particular with regard to the possible therapy options. In contrast to most carcinomas, an initial metastasis of the tumor does not in itself result in a verifiable deterioration in the prognosis. A significant deterioration in prognosis can only be assumed if more than two bones are involved, if the pleura is involved or if there is an associated ascites or coughing up blood ( hemoptysis ) as a sign of an advanced systemic disease. In men and patients over 55 years of age, the prognosis is worsened regardless of this. In general, the prognosis for EHE is much better than for angiosarcoma. Depending on the therapeutic approach and initial stage, patients with hepatic EHE achieve 5-year survival rates of 60% or more - even with initial multiple organ involvement.

Individual evidence

  1. ^ SW Weiss, FM Enzinger: Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. In: Cancer . Volume 50, Number 5, September 1982, pp. 970-981, ISSN  0008-543X . PMID 7093931 .
  2. ↑ a b c d e A. Mehrabi, A. Kashfi, H. Fonouni, P. Schemmer, BM Schmied, P. Hallscheidt, P. Schirmacher, J. Weitz, H. Friess, MW Buchler, J. Schmidt: Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. In: Cancer. Volume 107, Number 9, November 2006, pp. 2108-2121, ISSN  0008-543X . doi : 10.1002 / cncr.22225 . PMID 17019735 . (Review).
  3. ↑ PJ Dean, RC Haggitt, CJ O'Hara: Malignant epithelioid hemangioendothelioma of the liver in young women. Relationship to oral contraceptive use. In: The American Journal of Surgical Pathology . Volume 9, Number 10, October 1985, pp. 695-704, ISSN  0147-5185 . PMID 3904492 .
  4. ^ RA de Man, DJ Bac, M. van Blankenstein, PE Zondervan: Sterile necrosis of the liver due to primary epithelioid haemangio-endothelioma presenting as fever of undetermined origin. In: The Netherlands journal of medicine. Volume 45, Number 1, July 1994, pp. 25-29, ISSN  0300-2977 . PMID 8065481 .
  5. ↑ RA Soslow, P. Yin, CR Steinberg, GC Yang: Cytopathologic features of hepatic epithelioid hemangioendothelioma. In: Diagnostic cytopathology. Volume 17, Number 1, July 1997, pp. 50-53, ISSN  8755-1039 . PMID 9218904 .
  6. ↑ T. Darras, R. Moisse, JM Colette: Epithelioid hemangioendothelioma of the liver. In: Journal belge de radiologie. Volume 71, Number 6, 1988, pp. 722-723, ISSN  0021-7646 . PMID 3243752 .
  7. ↑ HR Makhlouf, KG Ishak, ZD Goodman: Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. In: Cancer. Volume 85, Number 3, February 1999, pp. 562-582, ISSN  0008-543X . PMID 10091730 .
  8. ^ SW Weiss, FM Enzinger: Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. In: Cancer. Volume 50, Number 5, September 1982, pp. 970-981, ISSN  0008-543X . PMID 7093931 .
  9. ↑ AJ Demetris, M. Minervini, RB Raikow, RG Lee: Hepatic epithelioid hemangioendothelioma: biological questions based on pattern of recurrence in an allograft and tumor immunophenotype. In: The American journal of surgical pathology. Volume 21, Number 3, March 1997, pp. 263-270, ISSN  0147-5185 . PMID 9060595 .
  10. ↑ K. Lau, M. Massad, C. Pollak, C. Rubin, J. Yeh, J. Wang, G. Edelman, J. Yeh, S. Prasad, G. Weinberg: Clinical Patterns and Outcome in Epithelioid Hemangioendothelioma With or Without Pulmonary Involvement: Insights From an Internet Registry in the Study of a Rare Cancer. In: Chest. 140, 2011, pp. 1312-1318, doi : 10.1378 / chest.11-0039 .