DOCSLIB.ORG

Early Lesions of Kaposi's Sarcoma in Homosexual Men an Ultrastructural Comparison with Other Vascular Proliferations in Skin

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Early Lesions of Kaposi's Sarcoma in Homosexual Men an Ultrastructural Comparison with Other Vascular Proliferations in Skin Early Lesions of Kaposi's Sarcoma in Homosexual Men An Ultrastructural Comparison With Other Vascular Proliferations in Skin N. SCOTT McNUTT, MD, VAN FLETCHER, MD and From the Departments of Pathology and Dermatology and the Kaposi MARCUS A. CONANT, MD Sarcoma Study Group, Veterans Administration Medical Center, and the University of California, San Francisco, California An aggressive variant of Kaposi's sarcoma (KS) has ap- thelial lining, and had only a few small junctional peared in young homosexual men with evidence of sys- densities between endothelial cells. Some clinically temic immunosuppression. The ultrastructure in biop- aggressive cases of KS also had necrosis of individual sy specimens from 8 KS cases in young homosexual endothelial cells and had prominent cytoplasmic pro- men has been compared with that in biopsy specimens cesses entrapping individual collagen fibers. The be- from 4 KS cases in elderly heterosexuals and with that nign disorders lacked these features. These differences in biopsy specimens from 23 cases of benign vascular in the structure of the small vessels may be of diagnos- disorders of skin. In all cases of KS the small blood ves- tic value in some early cases ofKS. The loss ofdendritic sels lacked a prominent investment of pericytes and pericytes in blood capillaries in KS might relate to the their processes, had a fragmented and often absent telangiectasia which is a prominent feature of the early basal lamina, had frequent discontinuities in the endo- lesions of KS. (Am J Pathol 1983, 111:62-77) A DRAMATIC CHANGE in the epidemiology and patients the disease progresses rapidly, and they die clinical course of the vascular neoplasm called of KS within 1 year, despite standard combination Kaposi's sarcoma (KS) has occurred in the United drug chemotherapy regimens.4 States since 1981. Previous to that time KS affected Although this variant KS has definitely increased in predominantly older men (over age 50) of Jewish, incidence in these locations of the United States, Italian, Mediterranean, or black ancestry,1`3 particu- earlier studies showed that an aggressive form of KS larly when it arose in otherwise apparently healthy in- affects African children.5'6 Also, 20% of the cases re- dividuals. Also KS usually appeared in the skin of the ported by Cox and Helwig2 occurred in patients aged lower extremities, often bilaterally, and gradually 9 to 49 years, and 7 of the 11 deaths from KS in that spread centripetally, to involve lymph nodes, lung, series occurred in patients less than 45 years old at the liver, intestine, or other viscera. Since 1979 a more onset of their disease. Twenty-two percent of the aggressive variant of KS has been found in young cases reviewed by McCarthy and Pack1 also occurred homosexual men (under age 58) predominantly in in patients under age 40. New York and California, which together account The chief candidate as an explanation for the very for 77%o of the currently reported cases (Centers for aggressive behavior of KS is the finding of profound Disease Control, personal communication). In addi- tion to departing from the usual ethnic predisposi- tion, this variant KS also tends to begin in a more Supported in part by American Cancer Society Grant scattered distribution on the body surface. It may RD151 and by Veterans Administration research funds. begin in unusual sites, such as the skin of the Accepted for publication November 8, 1982. Address reprint requests to Dr. N. Scott McNutt, Chief, shoulder or trunk, and tends to spread rapidly, to in- Dermatopathology Section, Department of Pathology, The volve many other skin sites, oral mucosa, lymph New York Hospital-Cornell Medical Center, 525 East 68th nodes, and viscera. In many (15-40%o) of these young Street, New York, NY 10021. 0002-9440/83/0407-0062 62 Vol. 111 * No. 1 ULTRASTRUCTURE OF KAPOSI'S SARCOMA 63 immunosuppression in this young homosexual popu- easily distinguished from one another, particularly in lation, as indicated by associated opportunistic infec- large punch or scalpel biopsies. tions with Pneumocystis carinii and cytomegalovirus4 The present study has two objectives: 1) To deter- as well as by markedly depressed ratios of helper to mine whether or not there are ultrastructural features suppressor T lymphocytes7-9 in peripheral blood. KS that correlate with the aggressive behavior of KS in has been noted previously to be one of the earliest young homosexual men and 2) To define those ultra- tumors that can appear after immunosuppression structural features that can be used to distinguish therapy in transplant recipients.0," Exactly how this early patch stage or angiomatous lesions of KS from immunosuppression might act to favor a more ag- a variety of benign vascular growths. gressive form of KS is not known at present. Preliminary reports by others have not indicated The histologic criteria for making a diagnosis of that there are any obvious differences at the histo- KS have been expounded clearly.'2"3 The lesions of logic level between KS in homosexual men and the KS usually begin as flat erythematous (red-purple to more usual forms of KS.14 In this study we carry this red-brown) macular lesions which have been called comparison to the ultrastructural level and observe patch stage lesions.13 These early patch lesions have differences with regard to the presence of necrotic en- been described histologically as having an angio- dothelial cells and cellular projections into the col- matous phase'12"3 in which there is a proliferation of lagen bundles in some but not all cases. thin-walled irregular vascular channels mainly in the reticular dermis. As the clinical lesions become Materials and Methods thickened to form plaques and eventually tumor nod- ules, histologic sections show that the lesions tend Ten biopsy specimens from 8 cases of Kaposi's sar- to contain nodules of spindle-shaped cells, which coma (KS) arising in young homosexual men (Table entrap many erythrocytes. Because of the spindle 1) were taken with the use of standard biopsy shape of the cells, some authors have called this the punches or scalpels for elliptical excisions. Biopsies fibroblastic phase of KS.'2 In the late stages, biopsies were performed in all cases before any treatment was of KS usually present no diagnostic problems, but initiated except in Case 4, where several injections of there can be occasional cases in which it is difficult to intravenous vincristine had been given 9 weeks before distinguish in a small punch biopsy between the early biopsy. Anesthesia was provided by intracutaneous patch stage of KS and a variety of benign lesions such injection of lidocaine, usually with added epine- as dermatofibromas ("fibrous histiocytoma"), non- phrine to help control hemostatis. The biopsy speci- palisading forms of granuloma annulare, insect bite mens were carefully divided for light microscopy and reactions, pyogenic granuloma, or even stasis derma- electron microscopy or histochemistry. Tissues for titis. Well-developed lesions of these types usually are standard light microscopy were fixed in phosphate- Table 1 - Kaposi's Sarcoma: Clinical Data Duration Follow-up at biopsy Initial Lesion interval Case Age Race Sex HP (years) stage thickness (years) Therapy Progression 46 w M + 0.1 sv Tu 2.2 C RP; D (KS) 2 40 w M + 1.0 SLV PI,Tu 0.5 C RP; D (KS) 3 38 w M + 1.0 SL Pi 0.6 C R 4 40 w M + 0.3 S Pi 0.5 C St (KS); D (P) 5 29 w M + 0.2 SL Ma 0.5 vi St 6 35 w M + 0.2 S Pi 0.4 vi St 7 34 w M + 0.1 S Pi 0.2 R 8 34 w M + 0.2 S Ma 0.2 C RP 9 87 w M - U S PI,Tu 0.2 N St 10 62 B M - 4.0 S Ma 0.3 N St 11 72 B F - 1.5 S Pi 0.3 N SP 12 64 W M - 14.0 S Pi 0.1 N RP* Abbreviations used: B, black; C, combination drug chemotherapy; D, died of (cause); F, female; HP, homosexual preference; I, interferon (Dr. Bijan Safai, Memorial Hospital, New York, NY); KS, Kaposi's sarcoma; L, lymph node involvement; M, male; Ma, macular lesions "patch" stage; N, none; P, Pneumocystis carinii pneumonia; Pi, plaque lesions; R, remission; RP, rapidly progressive; S, skin involvement; St, stable; SP, slow- ly progressive; Tu, tumor lesions; U, unknown; V, visceral involvement; Vi, vincristine (Velban); W, white. * This patient had experienced recent rapid progression of lesions at the time of biopsy, although more indolent disease had been present for a long time. 64 McNUTT ET AL AJP * April 1983 Table 2-Benign Disorders: Clinical Data Lesion Follow-up duration interval Pro- Case Age Race Sex Diagnosis (years) (years) Therapy gression 13 63 w M Dermatofibroma U 4.3 S No 14 51 w M Dermatofi broma 2.0 4.3 S No 15 58 w M Dermatofi broma 2.5 3.8 S No 16 79 0 M Dermatofi broma U 2.7 S No 17 59 w M Dermatofibroma 30.0 0.8 S No 18 54 w M Dermatofi broma 0.3 1.3 S No 19 U w M Fibrous histiocytoma, atypical 4.0 0.0 S U 20 74 B M Dermatofi broma U 0.8 S No 21 58 w M Dermatofi broma U 1.8 S No 22 37 B M Fibrous histiocytoma 20.0 1.8 S No 23 64 w M Dermatofibroma U 0.1 S No 24 62 w M Dermatofi broma 1.0 0.0 S U 25 62 w M Pyogenic granuloma 0.3 1.6 S No 26 61 B M Pyogenic granuloma 0.2 2.0 S No 27 71 w M Pyogenic granuloma 0.3 0.0 S U 28 27 w F Pyogenic granuloma 0.2 0.1 S R (1) 29 53 w M Capillary hemangioma 2.0 0.0 S U 30 74 w M Hemangioendothelioma, benign U 3.0 S No 31 70 w M Stasis dermatitis U 2.0 Bx p 32 55 w M Stasis dermatitis (hypertrophic) U 1.0 Bx p 33 55 w M Stasis dermatitis U 1.0 Bx p 34 42 B M Angiokeratoma U 0.3 S U 35 65 w M Telangiectasia, post BCNU 0.1 1.0 Bx St Abbreviations used: B, black; Bx, biopsy; BCNU, 1,3-bis(2-chloroethyl) 1-nitrosourea; F, female; M, male; No, no recurrence or progression; 0, oriental; P, persistant; R, recurrent for (number of times); S, surgical excision; St, stable; U, unknown; W, white.
Load more

Recommended publications
  • 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]
  • 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]
  • 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]
  • 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]
  • 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
    [Show full text]
  • Angiokeratoma Corporis Diffusum - a Case Report
    IOSR Journal of Dental and Medical Sciences (IOSR-JDMS) e-ISSN: 2279-0853, p-ISSN: 2279-0861.Volume 14, Issue 1 Ver. IV (Jan. 2015), PP 11-13 www.iosrjournals.org Angiokeratoma Corporis Diffusum - A Case Report Dr.S.Nageswaramma1 , Dr. G. Swarna Kumari2, Dr. P. Rajashekar3, Dr. S. Sowmya4, Dr. G. Sirisha5. Department of DVL, Guntur Medical College, DRNTRUHS, Andhra pradesh , India Abstract: Angiokeratoma corporis diffusum, a rare clinical type of angiokeratoma, reported in association with various diseases of which Fabry disease is most common. Fabry disease, an X-linked recessive inborn error of glycosphingolipid metabolism due to deficiency of lysosomal enzyme α- galactosidase A . Clinically the disease is characterized by acroparesthesias, multiple cherry red coloured raised angiomatous hyperkeratotic lesions over trunk, abdomen, sides of buttocks and genitilia. A 27-year-old male born to a consanguineous marriage presents with acroparaesthesias and multiple cherry red and hyperkeratotic lesions over trunk, abdomen, sides of buttocks and genitilia. Histopathological examination is consistent with angiokeratoma and our case was diagnosed as angiokeratoma corporis diffusum. This case is being reported because of its rarity. Keywords: Angiokeratoma corporis difffusum, fabry disease, X-linked recessive disease, α- galactosidase A, acroparaesthesias I. Introduction Fabry disease is an X – linked inborn error of glycosphingolipid metabolism resulting from deficient or absent activity of lysosomal enzyme α-galactosidase A. The enzyme defect leads to accumulation of globotriaosyl ceramide(GL-3) and related glycosphingolipids in plasma and tissue lysosomes. Males are primarily affected, females are carrriers. In classically affected males symptoms begin in childhood with acroparesthesias, burning and tingling pain in upper and lower extremities, hypohidrosis, even anhidrosis.
    [Show full text]
  • Coexistence of Lymphangioma Circumscriptum and Angiokeratoma
    C logy ase to R a e m p r o e Kemeriz et al., Dermatol Case Rep 2019, 4:2 r t D Dermatology Case Reports Case Report Open Access Coexistence of Lymphangioma Circumscriptum and Angiokeratoma in the Same Area: A Case Report Funda Kemeriz1*, Muzeyyen Gonul2, Aysun Gokce3 and Murat Alper3 1Department of Dermatology, Aksaray University, Aksaray, Turkey 2Department of Dermatology, Dıskapı Research and Training Hospital, Ankara, Turkey 3Department of Pathology, Dıskapı Research and Training Hospital, Ankara, Turkey *Corresponding author: Funda Kemeriz, Department of Dermatology, Aksaray University, Faculty of Medicine, Aksaray, Turkey, Tel: +905377104130; E-mail: [email protected] Received date: May 24, 2019; Accepted date: May 31, 2019; Published date: June 10, 2019 Copyright: ©2019 Kemeriz F, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Abstract Lymphangioma is a rare benign malformation of lymphatic vessels. Lymphangioma Circumscriptum (LC) is the most common type. Angiokeratoma is a vascular lesion which is considered to be caused primarily by vascular ectasia in the papillary dermis and epidermal changes developing secondarily. Here we presented a 51-year-old patient developing angiokeratoma and LC in the same area with literature’s data. Previously, one case pointing out to the coexistence of LC and angiokeratoma in cutaneous tissue has been reported in the literature. In our case, the lesions’ general clinical appearance was consistent with angiokeratoma and dermoscopic examination revealed the findings of angiokeratoma in some areas, histopathologically one of the patients’ biopsies specimens revealed angiokeratoma, the other was consistent with lymphangioma.
    [Show full text]
  • Vascular Anomalies
    Vascular Anomalies Dr Kurosh Parsi MBBS, Msc (MED), FACD, FACP Vascular Birthmark Clinic Sydney Children’s Hospital Vascular Anomalies Can be divided into two types: Tumours Malformations Vascular Tumours Can be divided into two groups: Congenital- present at birth Non-congenital- appear later Vascular Tumours Classification Mulliken 1982 Congenital vascular tumours include: Haemangioma Kaposiform Haemangioendothelioma (KHE) Tufted angioma Haemangiopericytoma Vascular Tumours Classification Mulliken 1982 Acquired vascular tumours include: Pyogenic granuloma Kaposi’s Sarcoma Angiosarcoma Unilateral nevoid telangiectasias Angiolymphoid hyperplasia with eosinophilia Haemangioendotheliomas Vascular Tumours Haemangiomas: Proliferative vascular tumour Not present at birth Grow disproportionate to the child’s growth Evolution involution (when involuting develops areas of grey discoloration) Many sub-types: RICH, NICH, Segmental, etc 1 Rapidly Involuting Congenital Hemangioma (RICH) Vascular tumor Fully developed at birth Undergoes rapid involution and regression Rare entity Has positive glucose transporter (Glut-1) Non-Involuting Congenital Hemangioma (NICH) Present before birth Grows in proportion with patient Does not involute If removed early (2-4 years) is similar to RICH Has negative glucose transporter (Glut-1) Haemangiomas Can be life-threatening Treatment with oral steroids, supportive therapy, excision Managed by Pediatric Dermatologists, pediatric plastic surgeons ‘Strawberry haemangioma’ should not
    [Show full text]
  • There's Blood in My Underwear
    CLINICAL There’s blood in my underwear Christopher Dalby QUESTION 4 ANSWER 4 What is the pathogenesis of this condition? While the exact pathogenesis of this is unclear, angiokeratomas result from CASE QUESTION 5 vessel dilation and damaged vascular A man aged 75 years presented with What are the different variants? How are endothelial cells,2 and there appears to concerns after finding bright red blood the different variants distinguished? be an association with increased venous stains in his underwear. This had been pressure.4 occurring intermittently for the past few QUESTION 6 months, but had intensified over the last What are the management options? ANSWER 5 week. He reported no clots. He stated There are generally considered to be four he otherwise felt well and specifically QUESTION 7 other forms of angiokeratoma. These can reported no pain. He passed motions What differentials could be considered if be subdivided into localised and systemic regularly without difficulty, denied any there was uncertainty about the diagnosis? forms.2,3 blood in his stools and stated he had In addition to angiokeratoma of never had haemorrhoids. He had seen ANSWER 1 Fordyce, the localised forms are: no blood in his urine. He denied epistaxis The scrotal changes were consistent with • angiokeratoma circumscriptum – or bleeding from other areas. There was angiokeratoma of Fordyce.1 unilateral changes affecting one of the no unexplained bruising. He was on no lower limbs or trunk; found at birth, antiplatelet or anticoagulant therapy. ANSWER 2 they can become quite large A rectal exam was performed in Angiokeratomas are keratotic, vascular order to exclude a possible rectal origin, skin changes of dark violet to black and the results were unremarkable.
    [Show full text]
  • Histopathological Study of Vascular Lesions
    IOSR Journal of Dental and Medical Sciences (IOSR-JDMS) e-ISSN: 2279-0853, p-ISSN: 2279-0861.Volume 16, Issue 11 Ver. I (Nov. 2017), PP 47-52 www.iosrjournals.org Histopathological Study of Vascular Lesions 1Dr. D. Kalyani, 2*Dr. N.Bharatarao, 1Associate Professor,Department of Pathology, Siddhartha Medical College,Vijayawada 2Professor & HOD,Department of Pathology, Siddhartha Medical College,Vijayawada Corresponding Author: *Dr. N. Bharatarao Abstract Background: Vascular lesions show a broad variety of morphological appearances and cilinical behavior, the lesions are ranging from inflammatory, benign hemangiomas to intermediate lesion, which are locally aggressive, to highly malignant angiosarcoma. There is also the grey zone between true neoplasia and hamartoma, which makes difficulty in histopathological assessment. It is also important to decide the degree of malignancy as it can strongly influence the choice of treatment and prognosis. Materials and Methods: 98 cases of vascular lesions at the department of pathology, Siddharda medical college, Vijayawada,for a period of three years(2014 -2016) have been studied retrospectivelyand histopathological features were analyzed. Results:A total of 98 vascular lesions were identified. Thsese were classified into inflammatory and neoplastic. The neoplastic tumors are subclassified into benign, intermediate and malignant according to WHO classification. The various vascular lesions identified were Leucocytoclastic vasculitis, Small vessel vasculitis, Granulomapyogenicum,Capillary hemangiomas, Cavernous hemangiomas,AV malformation, Sclerosing hemangiomas, Verrucous hemangioma, Intramuscular hemangioma, Hemangioblastoma, Angiokeratoma, Hemangioendothelioma, Epithelioidhemangioendotheliomama, Angiosarcoma,Epithelioid Angiosarcoma and Glomus tumor (Perivascular tumour). Majority of vascular tumors were benign, more common in children and young adults, most common sites were head and neck, which required only local surgical excision.
    [Show full text]
  • Provisionally Unclassified Vascular Anomalies
    Paediatric and Perinatal Pathology Update on vascular anomalies of childhood ISSVA classification for vascular anomalies Provisionally unclassified vascular anomalies Michel Wassef M.D. Dept of pathology and vascular anomalies clinics Lariboisière Hospital Paris, France Back to Type Alt overview for previous ISSVA classification for vascular anomalies view Provisionally unclassified vascular anomalies Intramuscular hemangioma * Angiokeratoma Sinusoidal hemangioma Acral arteriovenous "tumour" Multifocal lymphangioendotheliomatosis with thrombocytopenia / cutaneovisceral angiomatosis with thrombocytopenia (MLT/CAT) PTEN (type) hamartoma of soft tissue / "angiomatosis" of soft tissue (PHOST) PTEN Fibro adipose vascular anomaly (FAVA) PIK3CA * Distinct from infantile hemangioma, from intramuscular common VM, PHOST/AST, FAVA and AVM some lesions may be associated with thrombocytopenia and/or consumptive coagulopathy see details Causal genes in blue Intramuscular hemangioma Correspond to capillary type and part of mixed types of WHO "intramuscular hemangioma" T1 T1 G T1 T1 G fs T2 Intramuscular (capillary) hemangioma vs. arteriovenous malformation • Well delimited tumor-like lesion on imaging – with capillary blush on arteriograms – different from the nidus of AVM – without early venous drainage • Do not present the possible severe worsening of AVM • May resemble AVM on histological small specimens • Differences or identity with AVM need to be clarified Pediatr Radiol 2014 Back to Type Alt overview for previous ISSVA classification for vascular
    [Show full text]