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Hemangiosarcoma

Angiosarcomas

Hemangiosarcoma is a rare malignant neoplasm of vascular origin. It consists of atypical endothelial cells and undifferentiated sarcoma cells, proliferates rapidly, grows invasively and is associated with a poor prognosis.


Presentation

About 75% of HS arise in the skin or soft tissues, with other organs being affected as follows: liver, 6%; heart, 5%; bones, 4%; spleen 3% [2].

  • According to a retrospective study conducted in Mexico [3], cutaneous HS most commonly develop in the following regions of the human body: scalp and neck (37%), trunk (24%), face (20%). In the same study, tumor sizes ranging from a few millimeters to more than 30 cm have been reported. HS typically manifest in form of multiple, reddish blue or purple papules. As the disease progresses, these papules augment in size, their surface may ulcerate and become increasingly prone to bleed. Most patients diagnosed with this type of HS have a medical history of cancer, often of breast cancer treated with radiation therapy or by means of mastectomy.
  • HS affecting soft tissues or internal organs may cause pain and exert local mass effects. Further symptoms may develop due to HS interfering with organ function, e.g., hepatic HS may be associated with jaundice and lead to hepatic failure [4], while cardiac HS may cause dyspnea, pericardial effusion and cardiac tamponade [5] [6]. Spontaneous tumor rupture has repeatedly been described and may lead to anemia, thrombocytopenia, and coagulopathy, or hemorrhagic shock [7] [8].

Hematogenous spread is common, and metastases most frequently develop in the lungs. Accordingly, patients may present with signs of lung cancer.

Additionally, patients may claim constitutive symptoms like fatigue, fever, night sweats and weight loss. Hemolytic anemia may be observed [9] [10].

Constitutional Symptom
  • Additionally, patients may claim constitutive symptoms like fatigue, fever, night sweats and weight loss. Hemolytic anemia may be observed.[symptoma.com]
Thyroid Nodule
  • Fine needle aspiration cytology from the thyroid nodule was suspicious. After blood transfusion, total thyroidectomy was performed. Postoperative histopathological examination of the specimen revealed hemangiosarcoma of the thyroid.[ncbi.nlm.nih.gov]
Hepatosplenomegaly
  • But hemoglobin values decreased again and hepatosplenomegaly developed at the second month of surgery. Bone marrow aspiration cytology which was performed demonstrated the same tumoral cells infiltrating bone marrow.[ncbi.nlm.nih.gov]
Vascular Disease
  • Initial clinical diagnostic impressions included pericarditis, vasculitis due to a collagen vascular disease, interstitial pneumonia, sarcoidosis, and metastatic adenocarcinoma ( n 13).[nature.com]
Night Sweats
  • Additionally, patients may claim constitutive symptoms like fatigue, fever, night sweats and weight loss. Hemolytic anemia may be observed.[symptoma.com]
Recurrent Fractures
  • An 87-year-old man with Paget's disease of the left humerus was found to have a Paget's sarcoma upon pathologic examination of the left upper limb, which was disarticulated for severe pain and recurrent fractures.[ncbi.nlm.nih.gov]
Scalp Mass
  • The patient underwent an extended resection of the frontal and occipital scalp masses, measuring 6.0 2.5 cm and 1.0 1.0 cm, respectively, and a bilateral neck nodal dissection.[spandidos-publications.com]

Workup

Fine-needle aspiration biopsy and histopathological analysis of obtained specimens will reveal characteristic features. On the one hand, there are epithelioid cells lining small cavities that distantly resemble blood vessels. On the other hand, spindle-shaped sarcoma cells are present. They are generally arranged in strands and form the backbone of vessel-like structures. With regards to nuclei, aberrant mitotic figures are frequently observed [1]. There may be necrotic foci. Antibodies directed against vascular markers like CD31, CD34, and FLI1 may be used for immunohistochemical analyses to corroborate histological findings.

While this approach is feasible in case of superficial HS, a more extensive preparation is required to obtain tumor samples from other sites. Here, imaging techniques have to be applied first in order to confirm the presence of a local mass and to obtain information regarding its accessibility [11] [6].

Pericardial Effusion
  • ., hepatic HS may be associated with jaundice and lead to hepatic failure, while cardiac HS may cause dyspnea, pericardial effusion and cardiac tamponade.[symptoma.com]
  • All four patients with a known pericardial angiosarcoma also exhibited a pericardial effusion radiologically (4/4). No pericardial effusion was identified in the four patients with a known cardiac parenchymal angiosarcoma (0/4).[nature.com]
Bicarbonate Increased
  • Serum bicarbonate increased within 24 h of pregabalin administration in mice and rats. Rats compensated appropriately, but mice developed metabolic alkalosis and increased blood pH.[ncbi.nlm.nih.gov]
Pseudomonas
  • Here, we hypothesized that EGFuPA-toxin, a bispecific ligand-targeted toxin (BLT) consisting of a deimmunized Pseudomonas exotoxin (PE) conjugated to epidermal growth factor and urokinase, would efficiently target and kill cells derived from canine hemangiosarcoma[ncbi.nlm.nih.gov]

Treatment

  • Angiosarcoma is a rare neoplasm of endothelial origin that has limited treatment options and poor five-year survival.[ncbi.nlm.nih.gov]
  • A review of the literature shows that treatment modalities range from wide excision alone to surgical excision in conjunction with preoperative and postoperative chemotherapy and radiotherapy.[ncbi.nlm.nih.gov]
  • Radical excision followed by post-operative radiotherapy, especially in cases where the tumor has been partially excised, is the treatment of choice.[ncbi.nlm.nih.gov]
  • Despite treatment with doxorubicin and radiation therapy there was recurrence in lymph nodes and scan evidence of liver and bone metastases. The patient died 38 months after diagnosis.[ncbi.nlm.nih.gov]
  • Given the poor prognosis and the predominance in the third and fourth decades of life when fertility tends to decrease, reports of term pregnancies after treatment of this disease are rare.[ncbi.nlm.nih.gov]

Prognosis

  • Early splenectomy will improve prognosis and survival.[ncbi.nlm.nih.gov]
  • Primary angiosarcoma of the breast is an unusual neoplasm which is generally associated with a dismal prognosis.[ncbi.nlm.nih.gov]
  • Pathological aspects of splenic hemangiosarcoma and differences of prognosis between splenectomy performed before and after rupture are described.[ncbi.nlm.nih.gov]
  • The prognosis of the tumor is uniformly poor and most of the patients surviving laparotomy have followed a uniformly fatal clinical course. In a few cases treated with chemotherapy there has been no evidence of clinical benefit.[ncbi.nlm.nih.gov]
  • Pediatric hepatic angiosarcoma is a rare condition in children with poor prognosis. Microscopically this neoplasm has a particular 'Kaposi-form' arrangement. Hemangiosarcoma in non-human primates is a rare finding.[ncbi.nlm.nih.gov]

Etiology

  • The etiology of hemangiosarcoma remains incompletely understood. Its common occurrence in dogs suggests predisposing factors favor its development in this species.[ncbi.nlm.nih.gov]
  • We also discuss their etiology, incidence, and differential diagnosis, particularly in relation to more frequent primary adrenal lesions, such as adrenal adenomas and carcinomas.[ncbi.nlm.nih.gov]
  • Thus, we cannot be certain that radiation was the true or sole etiologic factor. Whether the addition of systemic chemotherapy was a contributory agent is also speculative.[ncbi.nlm.nih.gov]
  • Little is known about the disease' etiology, but patients suffering from chronic lymphedema as well as those who received radiation therapy are known to be predisposed for HS.[symptoma.com]
  • Disease Primary malignant vascular tumours of bone Etiology Although there is in a very small percentage an association between angiosarcoma and radiation, the etiology of the majority of angiosarcomas remains unknown.[atlasgeneticsoncology.org]

Epidemiology

  • Angiosarcomas also can originate in the liver, breast , spleen, bone, or heart. [1, 2 , 3, 4 ] Epidemiology Frequency United States Angiosarcomas are rare neoplasms.[emedicine.medscape.com]
  • Epidemiology Angiosarcomas are relatively rare. They comprise 1-2% of sarcomas in the surgical database from 1982-present from Memorial Hospital. Like other sarcomas, the risk of recurrence depends on the stage of disease.[curesarcoma.org]
  • Definition / general Angiosarcomas are malignant tumors that recapitulate functional and morphological features of normal endothelium Primary pleural angiosarcomas are morphologically very similar to their counterpart in soft tissue Epidemiology Extremely[pathologyoutlines.com]
  • *GROW, School for Oncology and Developmental Biology, Departments of †Obstetrics and Gynecology, ‡Internal Medicine, §Epidemiology, and Pathology, Maastricht University Medical Center, Maastricht; and ¶Department of Pathology, Atrium Medisch Centrum Parkstad[journals.lww.com]
  • Epidemiology Angiosarcoma is extremely rare and represent less than 1% of primary malignant bone tumours . Clinics Angiosarcomas of bone arise in adults and have a wide, nearly equal age distribution from the second till eighth decade.[atlasgeneticsoncology.org]
Sex distribution
Age distribution

Pathophysiology

  • Moreover, the growth-factor environment of HSA is very similar to that of pathophysiological angiogenesis, which some homeobox genes regulate in the transcription of angiogenic molecules.[ncbi.nlm.nih.gov]
  • Pathophysiology Angiosarcomas arising at different sites and in different organs have some distinct features. Angiosarcomas may occur in any region of the body but are more frequent in skin and soft tissue.[emedicine.medscape.com]

Prevention

  • The formation of vessel-like structures was blocked by nicotine, and restored by FK506, suggesting that 'nuclear factor of activated T cells' activity prevents differentiation of these cells.[ncbi.nlm.nih.gov]
  • Hence, surgical resection is preceded or followed by brachytherapy or external beam radiotherapy to prevent local recurrence.[omicsonline.org]
  • Epidemiology and Prevention . 3rd ed. New York: Oxford University Press; 2006. 763-786/959-974. E. Palmerini, R.G. Maki, E.L. Staals, M. Alberghini, et. al. Primary angiosarcoma of bone: A retrospective analysis of 60 patients from two institutions.[emedicine.medscape.com]
  • Small, frequent treatments by radiation therapy have been shown to have some effectiveness in preventing local recurrence of breast angiosarcomas.[breast-cancer.ca]
  • When indicated, surgery should be completed by adjuvant radiotherapy to prevent local recurrence [ 13 , 14 ]. Inoperable, locally advanced or metastatic AS are treated by cytotoxic chemotherapy.[clinicalsarcomaresearch.biomedcentral.com]

Summary

Hemangiosarcoma (HS) is a rare malignant neoplasm originating from mesenchymal tissue, namely from endothelial cells. This neoplasm is often described as a spongy tumor composed of dysfunctional blood vessel cells that are positioned together by undifferentiated sarcoma cells arranged in strands. The tumor is well supplied with blood. It differs largely from its benign counterpart, the hemangioma; HS generally affect the elderly, grow rapidly and invasively, and metastasize early. Hemangioma is typically diagnosed in neonates, grows slowly and resolves spontaneously.

HS primarily develop in the skin, in superficial or deep soft tissues, but they may also affect the liver, spleen, heart, or any other organ [1]. Little is known about the disease' etiology, but patients suffering from chronic lymphedema as well as those who received radiation therapy are known to be predisposed for HS [2]. It has been suggested that the growing number of patients exposed to therapeutic radiation accounts for the observed increase in HS incidence rates.

Of note, HS is a rather common diagnosis in veterinary medicine. Dogs are most frequently affected, but this tumor is also diagnosed in other species of domestic and wild animals. In order to avoid confusion between human and non-human HS, the term angiosarcoma is sometimes preferred for the disease of men. In any case, research concerning human HS is complicated by the low incidence of the disease. This does not apply to veterinary medicine and research; efforts made in this discipline may eventually allow the design of target-oriented studies in humans.

References

Article

  1. Geller RL, Hookim K, Sullivan HC, Stuart LN, Edgar MA, Reid MD. Cytologic features of angiosarcoma: A review of 26 cases diagnosed on FNA. Cancer Cytopathol. 2016;124(9):659-668.
  2. Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma. Lancet Oncol. 2010;11(10):983-991.
  3. Albores-Saavedra J, Schwartz AM, Henson DE, et al. Cutaneous angiosarcoma. Analysis of 434 cases from the Surveillance, Epidemiology, and End Results Program, 1973-2007. Ann Diagn Pathol. 2011;15(2):93-97.
  4. Abegunde AT, Aisien E, Mba B, Chennuri R, Sekosan M. Fulminant hepatic failure secondary to primary hepatic angiosarcoma. Case Rep Gastrointest Med. 2015;2015:869746.
  5. Ge Y, Ro JY, Kim D, et al. Clinicopathologic and immunohistochemical characteristics of adult primary cardiac angiosarcomas: analysis of 10 cases. Ann Diagn Pathol. 2011;15(4):262-267.
  6. Patel SD, Peterson A, Bartczak A, et al. Primary cardiac angiosarcoma - a review. Med Sci Monit. 2014;20:103-109.
  7. Xu B, Xie X, Zhou X, Zhai M, Yang W. Spontaneous rupture of primary splenic angiosarcoma: A case report. Oncol Lett. 2015;10(5):3271-3273.
  8. Tang K, Shang QL, Zhou QC, Zhou JW, She XL, Zhang M. Primary cardiac angiosarcoma with spontaneous ruptures of the right atrium and right coronary artery. Echocardiography. 2013;30(6):E156-160.
  9. Dalal B, Al Riyami A, Yenson P. Microangiopathic haemolytic anaemia and hyposplenism in angiosarcoma. Br J Haematol. 2010;151(5):413.
  10. Fong CY, Low M. An unusual case of microangiopathic hemolytic anemia resulting from metastatic angiosarcoma. Blood. 2013;122(1):7.
  11. Koyama T, Fletcher JG, Johnson CD, Kuo MS, Notohara K, Burgart LJ. Primary hepatic angiosarcoma: findings at CT and MR imaging. Radiology. 2002;222(3):667-673.

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Last updated: 2018-06-22 09:49