DOCSLIB.ORG

Angiokeratoma.Pdf

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Angiokeratoma.Pdf Classification of vascular tumors/nevi Vascular tumors mainly of infancy and childhood • Hemangioma of infancy. • Congenital hemangioma. • Miliary hemangiomatosis of infancy. • Spindle cell hemangioma. • Kaposiform hemangioendothelioma. • Tufted angioma. • Sinusoidal hemagioma. Vascular malformation - Capillary: • Salmon patch. • Potr-wine stain. • Nevus anemicus. - Mixed vascular malformation: • Reticulate vascular nevus. • Klipple ternaunay syndrome. • Venous malformation. • Blue rubber bleb nevus syndrome. • Maffucci syndrome. • Zosteriform venous malformation. • Other multiple vascular malformation syndrome. - Lymphatic malformations: • Microcystic/Macrocystic. • Rapid flow (arteriovenous malformation). Angiokeratoma: • Angiokeratoma circumscriptum naeviforme. • Angiokeratoma of Mibelli (or angiokeratoma acroasphyticum digitorum) . • Solitary papular angiokeratoma. • Angiokeratoma of fordyce (or angiokeratoma scroti). • Angiokeratoma corporis diffusum. Cutanous vascular hyperplasia: • Lobular capillary hemangioma. • Epithelioid hemangioma. • Crisoid aneurysm. • Reactive angioendotheliomatosis. • Gromeruloid hemangioma. • Hobnail hemangioma. • Microvascular hemangioma. Benign neoplasm: • Glomus tumors. Malignancy: • Kaposi sarcoma. • Angiosarcoma. • Retiform hemangioendothelioma. Modified classification of the International Society for the Study of Vascular Anomalies (Rome, Italy, 1996) : Tumors: -Hemangiomas: • Superficial (capillary or strawberry haemangioma). • Deep (cavernous haemangioma). • Combined. - Others: • Kaposiform haemangioendothelioma. • Tufted angioma. • Hemangiopericytoma. • Spindle - cell hemangioendothelioma. • Glomangioma. • Pyogenic granuloma. • Kaposi sarcoma. • Angiosarcoma. Vascular malformation: • Single capillary (C): (port-wine stain or nevus flammeus). • Venous (V). • Lymphatic (L): (lymphangioma or cystic hygroma). • Arterial (A). • combined arteriovenous fistula (AVF). • Arteriovenous malformation (AVM). • CLVM(include most cases of Klippel-Trenaunay syndrome). • CVM(include most cases of Klippel-Trenaunay syndrome). • LVM. • CAVM. • CLAVM. Difinition of angiokeratoma: The term angiokeratoma is applied to a number of quite distinct conditions that share a clinical presentation with asymptomatic hyperkeratotic vascular skin lesions and a histological combination of superficial dermal vascular ectasia and hyperkeratosis. Aetiopathogenesis The pathogenesis is still unknown. It is thought to be a telangiectatic lesion arising from local injury to capillaries in the papillary dermis, either from trauma or venous hypertension. In men, varicoceles have been implicated as a common cause. In women, increased venous pressure is noted during pregnancy and in vulval varicosity, post‐partum and post‐hysterectomy. Systemic angiokeratoma corporis diffusum (Anderson- Fabry disease) is an unusual X-linked lysosomal disorder characterized by deficiency of a-galactosidase. Varieties of angiokeratoma Angiokeratoma circumscriptum naeviforme. Angiokeratoma of Mibelli (or angiokeratoma acroasphyticum digitorum) . Solitary papular angiokeratoma. Angiokeratoma of fordyce (or angiokeratoma scroti). Angiokeratoma corporis diffusum. Angiokeratoma circumscriptum naeviforme • The condition is rare. Usually but not always present from birth and occasionally they appear to extend during adolescence. • The lesions are typically situated unilaterally on a lower leg or foot, but can occur on thigh, buttock or occasionally elsewhere. • They are deep red to blue–black warty keratotic papules or nodules and tend to take on a streaky, banded or zosteriform configuration. They may bleed on trauma. • There is no tendency to spontaneous improvement. • It may associated with fordyce angiokeratoma and caviar spots. Similar lesions also may be a feature of the Klippel–Trenaunay syndrome and of Cobb’s syndrome. Angiokeratoma of Mibelli • It is rare condition with a definite familial predisposition. It is transmitted as an AD trait . • Females appear to be affected predominantly. • The distribution and the regular association with acrocyanosis and chilblains appear to implicate cold injury as a provocative factor. Clinical features • Age of 10 and 15 years, but both earlier and later onset has been recorded. • A history of recurrent chilblains is usual. • The earliest lesions are minute, bright-red macules, which slowly increase in size and become elevated, warty and darker in colour. Many attain a diameter of about 5 mm, but some may be larger. • The dorsal and lateral aspects of fingers and toes are most often affected, but lesions also occur on the dorsa of hands and feet and occasionally on the knees ,elbows and buttocks . They may be associated with ulceration of the fingertips . • There is little tendency to spontaneous resolution. Solitary papular angiokeratoma • Appear usually between the age of 10 and 40 years. • Local trauma and cold injury may be predisposing factors . • They occur in both sexes and at any site, although the legs are the site of predilection. • The lesions appear as single or multiple warty papules, generally between 2 and 10 mm in diameter, and dark red to blue-black in colour. Angiokeratoma of fordyce • The most common of the angiokeratomas. • The pathogenesis of AKF may be due to injury the capillaries e.g ; trauma and chronic irritation. • It is also believed that atrophy of dartos muscle and degeneration of elastic tissue incidental to senility also plays a role in loss of support of blood vessels. So It frequently appear with increasing age and considered as a degenerative disorder. • Local venous hypertension as in varicocele plays a part in their development . Clinical features • Small, 1–6 mm, bright-red vascular papules may develop on the scrotum as early as late adolescence. With increasing age they become larger, darker and more numerous. • Patients may occasionally complain of itching, soreness or bleeding. • Identical lesions may also occur on the glans or shaft of the penis, labia majora or even on the upper thighs and in the groins. • Well circumscribed,macular, telangiectatic lesions have been described on the oral mucosa in patients with angiokeratomas of the scrotum. Angiokeratoma corporis diffusum (Fabry’s disease) • It’s a generalized form of angiokeratoma. • Males are primarily affected, females are carrriers. • Incidence about 1:40 000 to 1:60 000. Pathogenesis: • X-linked recessive inborn error of glycosphingolipid metabolism due to deficiency of lysosomal enzyme α-glycosidase A. • More than 200 mutations were identified in the long arm of chromosome X (Xq22). • Deficiency of α-glycosidase A enzyme cause storage of 2 neutral uncleaved glycosphingolipids; trihexosylceramide (α-galactosyle- lactosyle-ceramide) and digalactosyle-ceramide ( galabiosylceramide) to ccumulate within lysosomes in a variety of cell types, including capillary endothelial cells, fibroblasts, pericytes, heart, kidneys and autonomic nervous system giving rise to the symptoms and signs of Fabry’s disease. Clinical features: • Onset of the disease usually occurs during childhood or adolescence. Dermatological manifestations: • Angiokeratomas commonly seen in the lower half of the body namely lower abdomen, genitalia, buttock and lower limbs. It can also be seen in the lips. • Anhidrosis or hypohidrosis. Neurological manifestations: Two types of pain • Episodic crises ("Fabry crises”) characterized by agonizing burning pain originating in the extremities and radiating inwards to the limbs and other parts of the body. • Chronic pain characterized by burning and tingling paraesthesias. Gastrointestinal manifestation: • Abdominal pain (often after eating),diarrhea, nausea, vomiting, and anorexia. • These gastrointestinal symptoms may be related to the deposition of Gb3 in the autonomic ganglia of the bowel and mesenteric blood vessels. Cardiac manifestation: • Arrhythmias and cardiomyopathy. Renal manifestation: • Proteinuria, renal impairment and renal faileur. Cerebro vascular manifestation: • Transient ischemic attacks, ischemic strokes and more rarely vascular dementia. Auditory and vestibular manifestation : • Hearing loss, tinnitus and vertigo. Ocular manifestation: • Corneal opacities(cornea verticillata) and cataract. • ACD is no longer regarded as specific to Fabry`s disease only, but also occur in patients with several enzyme deficiencies as; α-Fucosidase (fucosidosis), Neuraminidase (sialodosis) Aspartylglucosaminidase (aspartylglucosaminuria), β-Mannosidase (β-mannosidosis), α-N acetylgalactosaminidase (Kanski disease), β-galactosidase (adult onset GM1 gangliosidosis). Differential Diagnosis I. Clinical differential diagnosis:- Melanocytic nevi. Malignant melanomas. Pigmented basal cell carcinomas. Spitz nevi. Lymphangioma circumscriptum. Seborrheic keratoses. Dermatofibromas. Other vascular lesions such as angiomas, hemangiomas or pyogenic granulomas. Wart. Molluscum contagiosum. Herpes zoster. Acral pseudolymphomatous angiokeratoma. Diagnosis 1. Clinical picture. 2. Dermoscopy. 3. Biopsy and histopathology. 4. In fabryʼs disease: Diagnosis is confirmed by: Finding low levels of α galactosidase A in the leukocytes, serum, tears and skin fibroblasts. Molecular genetic analysis of the GLA gene is the most accurate method of diagnosis in females. ECG, MRI, echocardiography, eye examination, renal function tests and kidney biopsy may also be suggestive of Fabry disease if excessive lipid buildup is noted. Prenatal diagnosis by enzyme activity or DNA testing in chorionic villi
Load more

Recommended publications
  • Hemangiosarcoma Philip J
    Ettinger & Feldman – Textbook of Veterinary Internal Medicine Client Information Sheet Hemangiosarcoma Philip J. Bergman What is hemangiosarcoma? Hemangiosarcoma (HSA; angiosarcoma or malignant hemangioendothelioma) is an extremely aggressive tumor of blood vessel origin. Because blood vessels are present throughout the body, virtually any site in the body can have HSA. HSA occurs most frequently in dogs (approximately 2% of all tumors) and the most common site is the spleen. However, additional common sites include the heart, liver, muscle, lung skin, bones, kidney, brain, abdomen, and oral cavity. In three large canine splenic disease studies encompassing approximately 2000 dogs, a “rule of two thirds” was found suggesting that approximately two thirds of dogs with a splenic mass have a cancer (therefore one third are not malignant) and two thirds of the malignant tumors of the spleen are HSA. HSA is a disease generally of older dogs and cats with an average onset of 9 to 10 years; however, there are reports of extremely young dogs and cats with this disease (5 to 6 months to a few years of age). German shepherd dogs are most commonly diagnosed with HSA; however, other large breed dogs such as golden retrievers and Labrador retrievers may also be overrepresented. In cats, the most common breed is the domestic shorthair. The cause of HSA in dogs and cats is presently unknown. Exposures to toxins such as chemicals, insecticides, and radiation have been reported in humans to be associated with HSA. Ultraviolet light exposure from the sun may be a potential cause of HSA in dogs, as HSAs of the skin are commonly seen in dogs with light hair and poor pigmentation (e.g., Salukis, Whippets, and white Bulldogs).
    [Show full text]
  • Angiokeratoma of the Scrotum (Fordyce Type) Associated with Angiokeratoma of the Oral Cavity
    208 Letters to the Editor anti-thyroperoxidas e antibody in addition to, or, less Yamada A. Antineutrophil cytoplasmic autoantibody- likely, instead of MPO-ANCA cannot be excluded. positive crescentric glomerulonephritis associated with thi- amazole therapy. Nephron 1996; 74: 734–735. Vesiculo-bullous SLE has been reported to respond 6. Cooper D. Antithyroid drugs. N Engl J Med 1984; 311: to dapsone (15). However, in our patient, an early 1353–1362. aggressive treatment with steroid pulse therapy and 7. Yung RL, Richardson BC. Drug-induced lupus. Rheum plasmapheresis was mandatory because of her life- Dis Clin North Am 1994; 20: 61–86. threatening clinical condition. The contributory factors, 8. Hess E. Drug-related lupus. N Engl J Med 1988; 318: 1460–1462. such as an environmental trigger or an immunological 9. Sato-Matsumura KC, Koizumi H, Matsumura T, factor, for the presence of a serious illness in this patient Takahashi T, Adachi K, Ohkawara A. Lupus eryth- remain to be elucidated. The mechanism by which ematosus-like syndrome induced by thiamazole and methimazole induces SLE-like reactions is unclear. propylthiouracil. J Dermatol 1994; 21: 501–507. 10. Wing SS, Fantus IG. Adverse immunologic eVects of antithyroid drugs. Can Med Assoc J 1987; 136: 121–127. 11. Condon C, Phelan M, Lyons JF. Penicillamine-induced REFERENCES type II bullous systemic lupus erythematosus. Br J Dermatol 1997; 136: 474–475. 1. Alarcon-Segovia D. Drug induced lupus syndromes. Mayo 12. Stankus S, Johnson N. Propylthiouracil-induced hyper- Clin Proc 1969; 44: 664–681.2. sensitivity vasculitis presenting as respiratory failure. Chest 2. Cush JJ, Goldings EA.
    [Show full text]
  • The Health-Related Quality of Life of Sarcoma Patients and Survivors In
    Cancers 2020, 12 S1 of S7 Supplementary Materials The Health-Related Quality of Life of Sarcoma Patients and Survivors in Germany—Cross-Sectional Results of A Nationwide Observational Study (PROSa) Martin Eichler, Leopold Hentschel, Stephan Richter, Peter Hohenberger, Bernd Kasper, Dimosthenis Andreou, Daniel Pink, Jens Jakob, Susanne Singer, Robert Grützmann, Stephen Fung, Eva Wardelmann, Karin Arndt, Vitali Heidt, Christine Hofbauer, Marius Fried, Verena I. Gaidzik, Karl Verpoort, Marit Ahrens, Jürgen Weitz, Klaus-Dieter Schaser, Martin Bornhäuser, Jochen Schmitt, Markus K. Schuler and the PROSa study group Includes Entities We included sarcomas according to the following WHO classification. - Fletcher CDM, World Health Organization, International Agency for Research on Cancer, editors. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon: IARC Press; 2013. 468 p. (World Health Organization classification of tumours). - Kurman RJ, International Agency for Research on Cancer, World Health Organization, editors. WHO classification of tumours of female reproductive organs. 4th ed. Lyon: International Agency for Research on Cancer; 2014. 307 p. (World Health Organization classification of tumours). - Humphrey PA, Moch H, Cubilla AL, Ulbright TM, Reuter VE. The 2016 WHO Classification of Tumours of the Urinary System and Male Genital Organs—Part B: Prostate and Bladder Tumours. Eur Urol. 2016 Jul;70(1):106–19. - World Health Organization, Swerdlow SH, International Agency for Research on Cancer, editors. WHO classification of tumours of haematopoietic and lymphoid tissues: [... reflects the views of a working group that convened for an Editorial and Consensus Conference at the International Agency for Research on Cancer (IARC), Lyon, October 25 - 27, 2007]. 4. ed.
    [Show full text]
  • Angiokeratoma of the Scrotum (Fordyce)
    Keio Journal of Medicine Vol. 1, No. 1, January, 1952 ANGIOKERATOMA OF THE SCROTUM (FORDYCE) MASAKATSU IZAKI Department of Dermatology, School of Medicine, Keio University Since Fordyce, in 1896, first described a case of angiokeratoma of the scrotum, many authors have reported and discussed about this dermatosis. However the classification and the nomenclature of this skin disease still remain in a state of confusion. Recently I had a chance to see the report of Robinson and Tasker (1946)(14), discussing the nomenclature of this condition, which held my attention considerably. In this paper I wish to report statistical observation concerning the incidences of this dermatosis among Japanese males, and histopathological studies made in 5 cases of this condition. STATISTICALOBSERVATION It must be first pointed out that this study was made along with the statistical study on angioma senile and same persons were examined in both dermatosis (ref. Studies on Senile Changes in the Skin I. Statistical Observation; Journal of the Keio Medical Society Vol. 28, No. 2, p. 59, 1951). The statistics was handled by the small sampling method. Totals of persons examined were 1552 males. Their ages varied from 16 to 84 years, divided into seven groups: i.e. the late teen-agers (16-20), persons of the third decade (21-30), of the fourth decade (31-40), of the fifth decade (41-50), of the sixth decade (51-60), of the seventh decade (61-70) and a group of persons over 71 years of age. The number of persons and the incidence of this condition in each group are summarized briefly in Table 1.
    [Show full text]
  • Vascular Tumors and Malformations of the Orbit
    14 Vascular Tumors Kaan Gündüz and Zeynel A. Karcioglu ascular tumors and malformations of the orbit VIII related antigen (v,w,f), CV141 (endothelium, comprise an important group of orbital space- mesothelium, and squamous cells), and VEGFR-3 Voccupying lesions. Reviews indicate that vas- (channels, neovascular endothelium). None of the cell cular lesions account for 6.2 to 12.0% of all histopatho- markers is absolutely specific in its application; a com- logically documented orbital space-occupying lesions bination is recommended in difficult cases. CD31 is (Table 14.1).1–5 There is ultrastructural and immuno- the most often used endothelial cell marker, with pos- histochemical evidence that capillary and cavernous itive membrane staining pattern in over 90% of cap- hemangiomas, lymphangioma, and other vascular le- illary hemangiomas, cavernous hemangiomas, and an- sions are of different nosologic origins, yet in many giosarcomas; CD34 is expressed only in about 50% of patients these entities coexist. Hence, some prefer to endothelial cell tumors. Lymphangioma pattern, on use a single umbrella term, “vascular hamartomatous the other hand, is negative with CD31 and CD34, lesions” to identify these masses, with the qualifica- but, it is positive with VEGFR-3. VEGFR-3 expression tion that, in a given case, one tissue element may pre- is also seen in Kaposi sarcoma and in neovascular dominate.6 For example, an “infantile hemangioma” endothelium. In hemangiopericytomas, the tumor may contain a few caverns or intertwined abnormal cells are typically positive for vimentin and CD34 and blood vessels, but its predominating component is negative for markers of endothelia (factor VIII, CD31, usually capillary hemangioma.
    [Show full text]
  • Angiofibroma of Soft Tissue: a Newly Described Entity; a Case Report and Review of Literature
    Open Access Case Report DOI: 10.7759/cureus.6225 Angiofibroma of Soft Tissue: A Newly Described Entity; A Case Report and Review of Literature Zafar Ali 1, 2 , Fatima Anwar 1 1. Histopathology, Shifa International Hospital, Islamabad, PAK 2. Pathology, Shifa College of Medicine, Islamabad, PAK Corresponding author: Zafar Ali, [email protected] Abstract Soft tissue angiofibroma is a relatively recent addition to the ever growing list of benign soft tissue tumors. It usually presents as soft tissue mass in the lower extremities in relation to joints and tendons. The tumor is composed of spindle-shaped fibroblastic cells with arborizing capillaries. We report a case of a 55-year-old female with a lump at the dorsum of left foot. Grossly the tumor was well circumscribed with yellow white cut surface. Microscopically the tumor showed typical features of angiofibroma with myxoid areas near the periphery of the lesion. Prominent vasculature is the integral part of the tumor with numerous small, branching, thin-walled blood vessels, accompanied by medium-sized ectatic vessels. Immunohistochemically the tumor cells are positive for epithelial membrane antigen (EMA). The location of the tumor, lack of cytological atypia, mitosis, and infiltrative margins help differentiate it from a sarcoma. Categories: Pathology Keywords: angiofibroma, ema, soft tissue Introduction Angiofibroma of soft tissue is a recently described entity; it was first described in 2012 by Mariño-Enríquez and Fletcher as a benign fibrovascular tumor that resembles a low grade sarcoma [1]. These tumors typically present in the extremities as a slow growing painless lump. There is a slight female predilection.
    [Show full text]
  • Benign Hemangiomas
    TUMORS OF BLOOD VESSELS CHARLES F. GESCHICKTER, M.D. (From tke Surgical Palkological Laboratory, Department of Surgery, Johns Hopkins Hospital and University) AND LOUISA E. KEASBEY, M.D. (Lancaster Gcaeral Hospital, Lancuster, Pennsylvania) Tumors of the blood vessels are perhaps as common as any form of neoplasm occurring in the human body. The greatest number of these lesions are benign angiomas of the body surfaces, small elevated red areas which remain without symptoms throughout life and are not subjected to treatment. Larger tumors of this type which undergb active growth after birth or which are situated about the face or oral cavity, where they constitute cosmetic defects, are more often the object of surgical removal. The majority of the vascular tumors clinically or pathologically studied fall into this latter group. Benign angiomas of similar pathologic nature occur in all of the internal viscera but are most common in the liver, where they are disclosed usually at autopsy. Angiomas of the bone, muscle, and the central nervous system are of less common occurrence, but, because of the symptoms produced, a higher percentage are available for study. Malignant lesions of the blood vessels are far more rare than was formerly supposed. An occasional angioma may metastasize following trauma or after repeated recurrences, but less than 1per cent of benign angiomas subjected to treatment fall into this group. I Primarily ma- lignant tumors of the vascular system-angiosarcomas-are equally rare. The pathological criteria for these growths have never been ade- quately established, and there is no general agreement as to this par- ticular form of tumor.
    [Show full text]
  • Infantile Hemangioendothelioma of the Parotid Gland
    Elmer ress Case Report Int J Clin Pediatr. 2015;4(4):184-185 Infantile Hemangioendothelioma of the Parotid Gland Veeranna A. Kotrashettia, Vijay Baburao Sonawanea, b, Kapil Bainadea, Reshu Agarwala Abstract parotid region, 4 × 3 cm in size, smooth in surface, no pulsa- tions with no signs of inflammation (Fig. 1). Tumors of salivary glands are uncommon in children (less than 5%). Complete blood count was normal. USG revealed en- Hemangioma is the commonest tumor of salivary gland tumors (more larged parotid gland measuring 3.1 × 2.2 × 2.5 cm with in- than 50%). We report a rare case of infantile hemangioma (IH) of creased vascularity. parotid gland. MRI showed well-defined, lobulated homogenously en- hancing leison diffusely involving the superficial and deep Keywords: Hemangioma; Hemangioendothelioma; Parotid gland layers of parotid gland (Fig. 2). Findings are suggestive of in- fantile hemangioendothelioma. Introduction Discussion Infantile hemangiomas (IHs) are the most common tumor of IH of parotid gland is the most common tumor. Weiss and oth- infancy and have a well-described natural history of rapid growth during early infancy followed by gradual involution [1, 2]. Congenital capillary hemangioma is classified as true hemangioma of infancy [3]. It is usually not noticed in new- born period but becomes prominent in first months of life. Most common age of presentation is about 4 months. Parotid hemangioma demonstrates rapid growth in first months of life but usually regresses after 18 months [4]. MRI is the best im- aging technique to demonstrate parotid hemangioma. MRI shows hyperintense parotid gland hemangioma con- taining vascular flow voids.
    [Show full text]
  • Angiosarcomas and Other Sarcomas of Endothelial Origin
    Angiosarcomas and Other Sarcomas of Endothelial Origin a,b a Angela Cioffi, MD , Sonia Reichert, MD , c a,b,d, Cristina R. Antonescu, MD , Robert G. Maki, MD, PhD, FACP * KEYWORDS Angiosarcoma Epithelioid hemangioendothelioma Vascular sarcoma Kaposi sarcoma VEGF KDR FLT4 Translocation Organ transplant KEY POINTS Vascular sarcomas are rare and collectively affect fewer than 600 people a year in the United States (incidence approximately 2/million). Because angiosarcomas, hemangioendotheliomas, and other vascular tumors have unique embryonal derivation, it is not surprising that they have a unique sensitivity pattern to chemotherapy agents. Surgery, when possible, remains the primary treatment for angiosarcomas. Adjuvant radiation for primary disease seems prudent for at least some angiosarcoma, given the high local-regional recurrence rate of these tumors. Angiosarcomas also have a high rate of metastasis, but it is not clear that adjuvant chemotherapy improves survival. Epithelioid hemangioendothelioma is a unique form of sarcoma often presenting as multi- focal disease. Most patients can do well with observation alone, although a fraction of pa- tients have more aggressive disease and have difficulties in both local control and metastatic disease. Continued Disclosures: R.G. Maki receives clinical research support from Morphotek/Eisai, Ziopharm, and Imclone/Lilly. He has also consulted for Eisai, Morphotek/Eisai, Imclone/Lilly, Taiho, Glaxo- SmithKline, Merck, Champions Biotechnology, and Pfizer. He has received speaker’s fees from Novartis. He is an unpaid consultant for the Sarcoma Foundation of America, SARC: Sarcoma Alliance for Research through Collaboration, n-of-one, and 23 & me. C.R. Antonescu, A. Cioffi, and S. Reichert report no conflicts.
    [Show full text]
  • View Open Access Histological Variants of Cutaneous Kaposi Sarcoma Wayne Grayson1 and Liron Pantanowitz*2
    Diagnostic Pathology BioMed Central Review Open Access Histological variants of cutaneous Kaposi sarcoma Wayne Grayson1 and Liron Pantanowitz*2 Address: 1Histopathology Department, Ampath National Laboratory Support Services, Johannesburg, South Africa and 2Department of Pathology, Baystate Medical Center, Tufts University School of Medicine, Springfield, Massachusetts, USA Email: Wayne Grayson - [email protected]; Liron Pantanowitz* - [email protected] * Corresponding author Published: 25 July 2008 Received: 23 July 2008 Accepted: 25 July 2008 Diagnostic Pathology 2008, 3:31 doi:10.1186/1746-1596-3-31 This article is available from: http://www.diagnosticpathology.org/content/3/1/31 © 2008 Grayson and Pantanowitz; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract This review provides a comprehensive overview of the broad clinicopathologic spectrum of cutaneous Kaposi sarcoma (KS) lesions. Variants discussed include: usual KS lesions associated with disease progression (i.e. patch, plaque and nodular stage); morphologic subtypes alluded to in the older literature such as anaplastic and telangiectatic KS, as well as several lymphedematous variants; and numerous recently described variants including hyperkeratotic, keloidal, micronodular, pyogenic granuloma-like, ecchymotic, and intravascular KS. Involuting lesions as a result of treatment related regression are also presented. Introduction progression, (2) KS variants alluded to in the older litera- Kaposi sarcoma (KS) is a vascular lesion of low-grade ture, (3) more recently described KS variants, and (4) KS malignant potential that presents most frequently with lesions as a consequence of therapy.
    [Show full text]
  • Vascular Malformations, Skeletal Deformities Including Macrodactyly, Embryonic Veins
    1.) Give a general classification and nomenclature to think about when evaluating these patients 2.) Share some helpful tips to narrow the differential in a minute or less of interaction 3.) Discuss some helpful imaging recommendations focusing on ultrasound Vascular Anomalies Tumors: Malformations: Hemangiomas: Low flow: Infantile Hemangioma (IH) Capillary malformation (CM) Rapidly involuting congenital hemangioma (RICH) Non-involuting congenital hemangioma (NICH) Venous malformation (VM) Kaposiform Hemangioendothelioma Lymphatic malformation (LM) (KHE) High flow: Arteriovenous malformation (AVM) Tufted Angioma (TA) Combined including syndromic VA. Other rare tumors www.issva.org • 2014 ISSVA classification is now 20 pages long • The key is that the imaging characteristics have not changed • Rapidly growing field • Traditionally, options were always the same – Surgery – Do nothing • With the increase in awareness and research as well as the development of the specialty of vascular anomalies: New Treatment Options Available – Treatment directly linked to diagnosis • Today, we have: – Interventional catheter based therapies – Laser surgery – Ablation technologies: Cryo, RFA, Microwave, etc. – Direct image-guided medications to administer – Infusion medicines – Oral medicines – Surgery- although much less common – Do Nothing- a VERY important alternative • Survey sample of 100 Referred patients – 47% wrong Dx – 35% wrong Tx • 14% wrong Tx with correct Dx • VAC – 14% indeterminate or wrong Dx – only 4% leave with no Tx plan • Important because
    [Show full text]
  • Mesenchymal) Tissues E
    Bull. Org. mond. San 11974,) 50, 101-110 Bull. Wid Hith Org.j VIII. Tumours of the soft (mesenchymal) tissues E. WEISS 1 This is a classification oftumours offibrous tissue, fat, muscle, blood and lymph vessels, and mast cells, irrespective of the region of the body in which they arise. Tumours offibrous tissue are divided into fibroma, fibrosarcoma (including " canine haemangiopericytoma "), other sarcomas, equine sarcoid, and various tumour-like lesions. The histological appearance of the tamours is described and illustrated with photographs. For the purpose of this classification " soft tis- autonomic nervous system, the paraganglionic struc- sues" are defined as including all nonepithelial tures, and the mesothelial and synovial tissues. extraskeletal tissues of the body with the exception of This classification was developed together with the haematopoietic and lymphoid tissues, the glia, that of the skin (Part VII, page 79), and in describing the neuroectodermal tissues of the peripheral and some of the tumours reference is made to the skin. HISTOLOGICAL CLASSIFICATION AND NOMENCLATURE OF TUMOURS OF THE SOFT (MESENCHYMAL) TISSUES I. TUMOURS OF FIBROUS TISSUE C. RHABDOMYOMA A. FIBROMA D. RHABDOMYOSARCOMA 1. Fibroma durum IV. TUMOURS OF BLOOD AND 2. Fibroma molle LYMPH VESSELS 3. Myxoma (myxofibroma) A. CAVERNOUS HAEMANGIOMA B. FIBROSARCOMA B. MALIGNANT HAEMANGIOENDOTHELIOMA (ANGIO- 1. Fibrosarcoma SARCOMA) 2. " Canine haemangiopericytoma" C. GLOMUS TUMOUR C. OTHER SARCOMAS D. LYMPHANGIOMA D. EQUINE SARCOID E. LYMPHANGIOSARCOMA (MALIGNANT LYMPH- E. TUMOUR-LIKE LESIONS ANGIOMA) 1. Cutaneous fibrous polyp F. TUMOUR-LIKE LESIONS 2. Keloid and hyperplastic scar V. MESENCHYMAL TUMOURS OF 3. Calcinosis circumscripta PERIPHERAL NERVES II. TUMOURS OF FAT TISSUE VI.
    [Show full text]