Hemangioendotheliomas are a family of vascular neoplasms of intermediate malignancy.

Signs and symptoms

They have been described as masses that fall between a

hemangioma A hemangioma or haemangioma is a usually benign vascular tumor derived from blood vessel cell types. The most common form, seen in infants, is an infantile hemangioma, known colloquially as a "strawberry mark", most commonly presenting on the ski ...

and

angiosarcoma Angiosarcoma is a rare and aggressive cancer that starts in the endothelial cells that line the walls of blood vessels or lymphatic vessels. Since they are made from vascular lining, they can appear anywhere and at any age, but older people are ...

. They are

vascular tumor A vascular tumor is a tumor of vascular origin; a soft tissue growth that can be either benign or malignant, formed from blood vessels or lymph vessels. Examples of vascular tumors include hemangiomas, lymphangiomas, hemangioendotheliomas, Ka ...

s that commonly present with an enlarging mass and most commonly involve the

lungs The lungs are the primary organs of the respiratory system in humans and most other animals, including some snails and a small number of fish. In mammals and most other vertebrates, two lungs are located near the backbone on either side ...

liver The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it ...

, and

musculoskeletal system The human musculoskeletal system (also known as the human locomotor system, and previously the activity system) is an organ system that gives humans the ability to move using their muscular and skeletal systems. The musculoskeletal system provid ...

, although many other body sites have been reported, including the head and neck,

intestines The gastrointestinal tract (GI tract, digestive tract, alimentary canal) is the tract or passageway of the digestive system that leads from the mouth to the anus. The GI tract contains all the major organs of the digestive system, in humans and ...

lymph nodes A lymph node, or lymph gland, is a kidney-shaped organ of the lymphatic system and the adaptive immune system. A large number of lymph nodes are linked throughout the body by the lymphatic vessels. They are major sites of lymphocytes that inclu ...

pleura The pulmonary pleurae (''sing.'' pleura) are the two opposing layers of serous membrane overlying the lungs and the inside of the surrounding chest walls. The inner pleura, called the visceral pleura, covers the surface of each lung and dips b ...

retroperitoneum The retroperitoneal space (retroperitoneum) is the anatomical space (sometimes a potential space) behind (''retro'') the peritoneum. It has no specific delineating anatomical structures. Organs are retroperitoneal if they have peritoneum on their ...

, heel,

stomach The stomach is a muscular, hollow organ in the gastrointestinal tract of humans and many other animals, including several invertebrates. The stomach has a dilated structure and functions as a vital organ in the digestive system. The stomach i ...

Cause

Possibly Bartonella spp bacteraemia

Diagnosis

Classification

Hemangioendotheliomas may be classified as: * '' Epithelioid hemangioendothelioma'' is an uncommon

of intermediate malignancy that was first described by Steven Billings, Andrew Folpe, and Sharon Weiss in 2003. These tumors are so named because their histologic appearance resembles a proliferation of epithelioid cells, with polygonal shape and eosinophilic cytoplasm. * ''Composite hemangioendothelioma'' is a low-grade

typically occurring in adults, although it has been described in infancy.James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. . * ''Spindle-cell hemangioendothelioma'') is a vascular tumor that was first described in 1986 by Sharon Weiss, M.D., and commonly presents in a child or young adult who develops blue nodules of firm consistency on a distal extremity. These tumors were reclassified by Dr. Weiss in 1996 as "spindle cell hemangioma", rather than hemangioendothelioma, due to the excellent prognosis observed in a group of 78 patients. * ''Retiform hemangioendothelioma'' (also known as a "Hobnail hemangioendothelioma") is a low-grade

, first described in 1994, presenting as a slow-growing exophytic mass,

dermal The dermis or corium is a layer of skin between the epidermis (with which it makes up the cutis) and subcutaneous tissues, that primarily consists of dense irregular connective tissue and cushions the body from stress and strain. It is divided ...

plaque, or subcutaneous nodule. * ''

Kaposiform hemangioendothelioma A vascular tumor is a tumor of vascular system, vascular origin; a soft tissue growth that can be either Benign tumor, benign or malignant, formed from blood vessels or lymph vessels. Examples of vascular tumors include hemangiomas, lymphangiomas, ...

'' (also known as "Infantile kaposiform hemangioendothelioma") is an uncommon vascular tumor, first described by Niedt, Greco, et al. (Hemangioma with Kaposi's sarcoma-like features: report of two cases.(Niedt GW, Greco MA, Wieczorek R, Blanc WA, Knowles DM 2nd. that affects infants and young children, with rare cases having also been reported in adults. Pediatr Pathol. 1989;9(5):567-75.) * ''Endovascular papillary angioendothelioma'', also known as "Dabska tumor", "papillary intralymphatic angioendothelioma" (PILA), "Dabska-type hemangioendothelioma", "hobnail hemangioendothelioma", and "malignant endovascular papillary angioendothelioma", is a rare low-grade

lymphatic channel The lymphatic system, or lymphoid system, is an organ system in vertebrates that is part of the immune system, and complementary to the circulatory system. It consists of a large network of lymphatic vessels, lymph nodes, lymphatic or lymphoid ...

s. Approximately 30 such

tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

s have been described in the

medical literature Medical literature is the scientific literature of medicine: articles in journals and texts in books devoted to the field of medicine. Many references to the medical literature include the health care literature generally, including that of denti ...

.Schwartz, RA. Dabska Tumor. Medscape, updated: Apr 5, 2013
/ref> Although included in the

World Health Organization The World Health Organization (WHO) is a specialized agency of the United Nations responsible for international public health. The WHO Constitution states its main objective as "the attainment by all peoples of the highest possible level of ...

tumor classification, there is uncertainty as to whether EPA is a distinct entity or a heterogenous group of tumours. The lesion usually presents as a slow-growing

of the

skin Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation. Other animal coverings, such as the arthropod exoskeleton, have different de ...

and subcutaneous tissues of the head, neck, or extremity, of infants or young children. However, EPA has involved the

testicle A testicle or testis (plural testes) is the male reproductive gland or gonad in all bilaterians, including humans. It is homologous to the female ovary. The functions of the testes are to produce both sperm and androgens, primarily testoste ...

, deep muscle tissue as a neoplastic transformation of a larger existing

benign Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

cavernous hemangioma Cavernous hemangioma, also called cavernous angioma, venous malformation, or cavernoma, is a type of venous malformation due to endothelial dysmorphogenesis from a lesion which is present at birth. A cavernoma in the brain is called a cerebral ca ...

bone A bone is a rigid organ that constitutes part of the skeleton in most vertebrate animals. Bones protect the various other organs of the body, produce red and white blood cells, store minerals, provide structure and support for the body, ...

and

spleen The spleen is an organ found in almost all vertebrates. Similar in structure to a large lymph node, it acts primarily as a blood filter. The word spleen comes .

, and has been found in adults. Some reports indicate a good prognosis but

metastasis Metastasis is a pathogenic agent's spread from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, then ...

is occasionally seen. * ''Infantile hemangioendothelioma'' is a rare benign vascular tumour arising from mesenchymal tissue and is usually located in the

. It often presents in infancy with

cardiac failure Heart failure (HF), also known as congestive heart failure (CHF), is a syndrome, a group of signs and symptoms caused by an impairment of the heart's blood pumping function. Symptoms typically include shortness of breath, excessive fatigue, a ...

because of extensive arteriovenous shunting within the lesion. It is the third most common liver tumor in children, the most common benign vascular tumor of the liver in infancy, and the most common symptomatic liver tumor during the first 6 months of life. These hemangioendotheliomas have 2 growth phases: an initial rapid growth phase, which is followed by a period of spontaneous involution (usually within the first 12 to 18 months of life). Detection of the hemangioendothelioma within the first 6 months of life is attributed to the initial rapid growth during this time; however, the tumor has been detected with fetal ultrasonography.Banks T, Podraza J
What's Your Diagnosis? Newborn with abdominal mass and distention
. Consultant for Pediatricians. 2010;9:168-173. Histopathologically, there are 2 types of hepatic hemangioendotheliomas: ** Type I: Hemagioendotheliomas of this type have multiple vascular channels that are formed by an immature endothelial lining with stromal separation from bile ductules. ** Type II: These hemangioendotheliomas have an appearance that is more disorganized and hypercellular, and there are no bile ductules. : In children, distinguishing between a primary malignant liver tumor (hepatoblastoma) and a benign primary hepatic lesion (hemangioendothelioma) is crucial. The absence of urinary catecholamines supports the diagnosis of hemangioendothelioma. In patients with hemangioendotheliomas, elevations in α₁-fetoprotein levels are milder than those found in patients with hepatoblastomas. Infantile hepatic hemangioendothelioma is strongly suggested by the presence of a vascular lesion on imaging studies. A complex, heterogeneous mass is often seen on ultrasonograms; a complex tumor that lacks central enhancement can be see on CT scans; and the vascular nature of the lesion along with dilation of the aorta proximal to the origin of the celiac artery and a decrease in the diameter distally, indicating significant shunting, is seen on angiograms. Because most hemangioendotheliomas in infants sponanteously involute and regress within the first 12 to 18 months of life, asymptomatic lesions are generally managed conservatively. Infants who have severe anemia and/or thrombocytopenia can be given blood products; for those who have cardiac failure, diuretics and digoxin are often given. To stop further growth and to speed regression of lesions in infants with more significant clinical sequelae, treatment with corticosteroids or interferon-α-2a is administered. To slow the growth of tumors that are rapidly enlarging, chemotherapy and radiation therapy have been used. Surgical resection, partial hepatectomy, and embolization of afferent vessels should be considered for severe cases. File:Dabska histo.jpg, Low power photomicrograph of an endovascular papillary angioendothelioma showing

papilla Papilla (Latin, 'nipple') or papillae may refer to: In animals * Papilla (fish anatomy), in the mouth of fish * Basilar papilla, a sensory organ of lizards, amphibians and fish * Dental papilla, in a developing tooth * Dermal papillae, part of ...

e with hyalinized cores File:Dabska histo2.jpg, High power view showing a vascular tumor with cuboidal

endothelium The endothelium is a single layer of squamous endothelial cells that line the interior surface of blood vessels and lymphatic vessels. The endothelium forms an interface between circulating blood or lymph in the lumen and the rest of the vesse ...

lining the vessels. Few entrapped

seminiferous tubule Seminiferous tubules are located within the testes, and are the specific location of meiosis, and the subsequent creation of male gametes, namely spermatozoa. Structure The epithelium of the tubule consists of a type of sustentacular cells known ...

s are also noted (arrow). File:SkinTumors-PB061072.JPG, Characteristic budding, hobnail-like endothelial cells visible.

Treatment

Treatment is varied and depends on the site and extent of tumor involvement, sites of metastasis, and specific individual factors. Surgical resection, radiotherapy, and

chemotherapy Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs ( chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemothe ...

have all been used to treat these masses, although studies on survival have yet to be conducted to delineate various treatment regimens. Kaposiform hemangioendothelioma might respond to chemotherapy or antiangiogenic therapies. Recently propanolol and steroids have been shown to be very effective in kaposiform hemangioendothelioma.

References

External links

{{Vascular tumors Vascular neoplasia