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Hemangiosarcoma can cause a wide variety of hematologic and hemostatic abnormalities, including anemia, thrombocytopenia (low platelet count), disseminated intravascular coagulation (DIC); presence of nRBC, schistocytes, and acanthocytes in the blood smear; and leukocytosis with neutrophilia, left shift, and monocytosis.
A definitive diagnosis requires biopsy and histopathology. Cytologic aspirates are usually not recommended, as the accuracy rate for a positive diagnosis of malignant splenic disease is approximately 50%. This is because of frequent blood contamination and poor exfoliation. Surgical biopsy is the typical approach in veterinary medicine.
Treatment is varied and depends on the site and extent of tumor involvement, site(s) of metastasis, and specific individual factors. Surgical resection, radiotherapy, and chemotherapy have all been used to treat these masses, although studies on survival have yet to be conducted to delineate various treatment regimens.
The management of PASH is controversial. Excision may be indicated in enlarging masses or lesions with atypical features.
The diagnosis of PASH is by biopsy.
The important differential diagnosis is angiosarcoma, from which it was first differentiated in 1986.
The most successful treatment for angiosarcoma is amputation of the affected limb if possible. Chemotherapy may be administered if there is metastatic disease. If there is no evidence of metastasis beyond the lymphedematous limb, adjuvant chemotherapy may be given anyway due to the possibility of micrometastatic disease. Evidence supporting the effectiveness of chemotherapy is, in many cases, unclear due to a wide variety of prognostic factors and small sample size. However, there is some evidence to suggest that drugs such as paclitaxel, doxorubicin, ifosfamide, and gemcitabine exhibit antitumor activity.
Treatment includes chemotherapy and, where practical, removal of the tumor with the affected organ, such as with a splenectomy. Splenectomy alone gives an average survival time of 1–3 months. The addition of chemotherapy, primarily comprising the drug doxorubicin, alone or in combination with other drugs, can increase the average survival time to 2-4 months, or more.
A more favorable outcome has been demonstrated in recent research conducted at University of Pennsylvania Veterinary School, in dogs treated with a compound derived from the Coriolus versicolor (commonly known as "Turkey Tail") mushroom:
“We were shocked,” Cimino Brown said. “Prior to this, the longest reported median survival time of dogs with hemangiosarcoma of the spleen that underwent no further treatment was 86 days. We had dogs that lived beyond a year with nothing other than this mushroom as treatment.”There were not statistically significant differences in survival between the three dosage groups, though the longest survival time was highest in the 100 mg group, at 199 days, eclipsing the previously reported survival time.
The results were so surprising, in fact, that the researchers asked Penn Vet pathologists to recheck the dogs’ tissue biopsies to make sure that the dogs really had the disease.
“They reread the samples and said, yes, it’s really hemangiosarcoma,” Cimino Brown said.
Chemotherapy is available for treating hemangiosarcoma, but many owners opt not to pursue that treatment once their dog is diagnosed.
“It doesn’t hugely increase survival, it’s expensive and it means a lot of back and forth to the vet for the dog,” Cimino Brown said. “So you have to figure in quality of life.”
This treatment does not always work. So, one should always be prepared for their pet to have the same survival time as a dog who is untreated.
Visceral hemangiosarcoma is usually fatal even with treatment, and usually within weeks or, at best, months. In the skin, it can be cured in most cases with complete surgical removal as long as there is not visceral involvement.
Intravascular papillary endothelial hyperplasia (also known as "Masson's hemangio-endotheliome vegetant intravasculaire," "Masson's lesion," "Masson's pseudoangiosarcoma," "Masson's tumor," and "Papillary endothelial hyperplasia") is a rare, benign tumor. It may mimic an angiosarcoma, with lesions that are red or purplish 5-mm to 5-cm papules and deep nodules on the head, neck, or upper extremities.
Early detection is key. Untreated patients usually live 5 to 8 months after diagnosis.
Eccrine carcinoma (also known as a syringoid carcinoma) is a rare cutaneous condition characterized by a plaque or nodule on the scalp, trunk, or extremities.
Hemangioendotheliomas may be classified as:
- "Epithelioid sarcoma-like hemangioendothelioma" is an exceedingly rare vascular tumor of intermediate grade that was first described by Steven Billings, Andrew Folpe, and Sharon Weiss in 2003. These tumors are so named because their histologic appearance is very similar to that of epithelioid sarcoma, a more malignant tumor with which they are commonly mistaken.
- "Composite hemangioendothelioma" is a low-grade angiosarcoma typically occurring in adults, although it has been described in infancy.
- "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 angiosarcoma, first described in 1994, presenting as a slow-growing exophytic mass, dermal plaque, or subcutaneous nodule.
- "Kaposiform hemangioendothelioma" (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 angiosarcoma of lymphatic channels. Approximately 30 such tumors have been described in the medical literature. Although included in the World Health Organization 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 tumor of the skin and subcutaneous tissues of the head, neck, or extremity, of infants or young children. However, EPA has involved the testicle, deep muscle tissue as a neoplastic transformation of a larger existing benign cavernous hemangioma, bone and spleen, and has been found in adults. Some reports indicate a good prognosis but metastasis is occasionally seen.
- "Infantile hemangioendothelioma" is a rare benign vascular tumour arising from mesenchymal tissue and is usually located in the liver. It often presents in infancy with cardiac failure 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. 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.
Angiosarcoma is a cancer of the cells that line the walls of blood vessels or lymphatic vessels. The lining of the vessel walls is called the endothelium. Cancers from the walls of blood vessels are called hemangiosarcomas, and cancers from the walls of lymphatic vessels are called lymphangiosarcomas. However, they should not be confused with cherry hemangiomas.
Most tumors of visceral blood and lymphatic vessel walls are cancerous (malignant). Because these cancers are carried by the blood flow or lymphatic flow, they can more easily metastasize to distant sites, particularly the liver and lungs.
Angiosarcomas will show signs of hemorrhage and necrosis. Pathologically, tumor cells will show increased nuclear to cytoplasm ratio, nuclear hyperchromasia, nuclear pleomorphism and high mitotic activity.
In dogs, hemangiosarcoma is relatively common, especially in larger breeds such as golden retrievers and Labrador retrievers. In humans, hemangiosarcomas and lymphangiosarcomas of the skin are uncommon. Angiosarcoma of the liver, a rare fatal tumor, has been seen in workers intensively exposed to the gas vinyl chloride monomer (VCM) for prolonged periods while working in polyvinyl chloride (PVC) polymerization plants. It has also been associated with individuals exposed to arsenic-containing insecticides and Thorotrast.
Lymphangiosarcoma is a rare malignant tumor which occurs in long-standing cases of primary or secondary lymphedema. It involves either the upper or lower lymphedematous extremities but is most common in upper extremities. Although its name implies lymphatic origin, it is believed to arise from endothelial cells and may be more accurately referred to as angiosarcoma.
Tumor alopecia is the hair loss in the immediate vicinity of either benign or malignant tumors of the scalp.
Angiolymphoid hyperplasia with eosinophilia (also known as: "Epithelioid hemangioma," "Histiocytoid hemangioma," "Inflammatory angiomatous nodule," "Intravenous atypical vascular proliferation," "Papular angioplasia," "Inflammatory arteriovenous hemangioma," and "Pseudopyogenic granuloma") usually presents with pink to red-brown, dome-shaped, dermal papules or nodules of the head or neck, especially about the ears and on the scalp.
It, or a similar lesion, has been suggested as a feature of IgG4-related skin disease, which is the name used for skin manifestations of IgG4-related disease.
In the 1960s, the incidence 5 years after a radical mastectomy varied from 0.07% to 0.45%.
Today, it occurs in 0.03% of patients surviving 10 or more years after radical mastectomy.
A variant of eccrine spiradenoma which can be multiple on the scalp and can coalesce to form a 'Turban' tumour. In pathology, a cylindroma is a tumour with nests of cells that resemble a cylinder in cross section.
Types include:
- Dermal eccrine cylindroma, a benign tumour of the skin
- Adenoid cystic carcinoma, a malignant tumour of the salivary gland
Folliculosebaceous cystic hamartoma abbreviated as (FSCH) is a rare cutaneous hamartoma consisting of dilated folliculosebaceous units invested in mesenchymal elements. it typically affects adults, have a predilection for the central face or scalp, with less than 1.5 cm dimension. Clinically, the lesions are asymptomatic, rubbery to firm in consistency, and usually occur on or above the neck in (> 90%) of cases, Histopathologically, FSCH shares several similar features to sebaceous trichofolliculoma, but it is usually possible to differentiate these two tumors.
Surgical excision is required to treat a trichilemmal cyst. The method of treatment varies depending on the physician's training. Most physicians perform the procedure under local anesthetic. Others prefer a more conservative approach. This involves the use of a small punch biopsy about 1/4 the diameter of the cyst. The punch biopsy is used to enter the cyst cavity. The content of the cyst is emptied, leaving an empty sac. As the pilar cyst wall is the thickest and most durable of the many varieties of cysts, it can be grabbed with forceps and pulled out of the small incision. This method is best performed on cysts larger than a pea which have formed a thick enough wall to be easily identified after the sac is emptied. Small cysts have walls that are thin, and easily fragmented on traction. This increases the likelihood of cyst recurrence. This method often results in only a small scar, and very little if any bleeding.
Most heart tumors begin with myxomas, fibromas, rhabdomyomas, and hamartomas, although malignant sarcomas (such as angiosarcoma or cardiac sarcoma) have been known to occur. In a study of 12,487 autopsies performed in Hong Kong seven cardiac tumors were found, most of which were benign. According to Mayo Clinic: "At Mayo Clinic, on average only one case of heart cancer is seen each year." In a study conducted in the Hospital of the Medical University of Vienna 113 primary cardiac tumour cases were identified in a time period of 15 years with 11 being malignant. The mean survival in the latter group of patients was found to be .
Primary malignant cardiac tumors (PMCTs) are even more rare. A study using the Surveillance, Epidemiology and End-Results (SEER) Cancer Registry from 1973–2011 found 551 cases of PMCTs, with an incidence of 34 cases per million persons. The study also found that the incidence has doubled over the past four decades. The associated mortality was very high, with only 46% of patients alive after one year. Sarcomas and mesotheliomas had the worst survival, while lymphomas had better survival. When compared with extracardiac tumors, PMCTs had worse survival.
A trichilemmal cyst, also known as a wen, pilar cyst or isthmus-catagen cyst, is a common cyst that forms from a hair follicle. They are most often found on the scalp. The cysts are smooth, mobile and filled with keratin, a protein component found in hair, nails, skin, and horns. They are, however, clinically and histologically distinct from Trichilemmal Horns, which are much more rare and not limited to the scalp. Trichilemmal cysts may run in families and they may or may not be inflamed and tender, often depending on whether they have ruptured. Rarely, these cysts may grow more extensively and form rapidly multiplying trichilemmal tumors, also called proliferating trichilemmal cysts, which are benign but may grow aggressively at the cyst site. Very rarely, trichilemmal cysts can become cancerous.
Actinic granuloma (also known as "O'Brien granuloma") is a cutaneous condition characterized histologically by a dermal infiltrate of macrophages.
Actinic granuloma is an asymptomatic granulomatous reaction that affects sun-exposed skin, most commonly on the face, neck, and scalp.
It is characterized by annular or polycyclic lesions that slowly expand centrifugally and have an erythematous elevated edge and a hypopigmented, atrophic center.
Advise to reduce exposure to the sun and to use sunscreen.
Treatment with topical halometasone cream, pimecrolimus cream.
Alopecia mucinosa (also known as "Follicular mucinosis," "Mucinosis follicularis,", "Pinkus’ follicular mucinosis," and "Pinkus’ follicular mucinosis–benign primary form") is a skin disorder that generally presents, but not exclusively, as erythematous plaques or flat patches without hair primarily on the scalp and face. This can also present on the body as a follicular mucinosis and may represent a systemic disease.
Heart cancer is an extremely rare form of cancer that is divided into primary tumors of the heart and secondary tumors of the heart.
There is no diagnosis as yet for Cutis verticis gyrata (CVH), but it can generally be found out by self, when the person is applying oil to the scalp or getting the hair fully shaven. The ripples are present either in identical form, mostly in the posterior direction, sometimes horizontally also, but it looks more like the ripples of the brain.
There is no clinical diagnosis for CVG as these cases are rarely seen and is often comorbid with other conditions.
Management consists of vigilant observation over days to detect progression. The subgaleal space is capable of holding up to 50% of a newborn baby's blood and can therefore result in acute shock and death. Fluid bolus may be required if blood loss is significant and patient becomes tachycardic. Transfusion and phototherapy may be necessary. Investigation for coagulopathy may be indicated.