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Ocul Oncol Pathol 2018 Jun 12;4(4):230-235. Epub 2018 Jan 12.

Department of Ophthalmic Pathology, Thomas Jefferson University, Philadelphia, PA, USA.

Primary cutaneous angiosarcoma is a rare vasoformative malignant neoplasm that can present a diagnostic and therapeutic challenge. We describe a 76-year-old Caucasian man with right upper eyelid swelling and nodularity, initially suspected clinically to represent either ocular adnexal lymphoma or basal cell carcinoma. Incisional biopsy and wide resection of the mass with frozen section control of margins were interpreted as compatible with hobnail (Dabska-retiform) hemangioendothelioma. Read More

Dig Endosc 2018 May 25;30(3):390. Epub 2018 Mar 25.

Department of Gastroenterology, Changhai Hospital, Naval Medical University, Shanghai, China.

Clin Sarcoma Res 2018 30;8. Epub 2018 Jan 30.

1Department of Pathology, Rizzoli Institute, Bologna, Italy.

Background: Papillary intralymphatic angioendothelioma (PILA) is a locally aggressive, rarely metastasizing vascular tumor, generally occurring in the soft tissues, with less than 40 cases described in the literature and only three cases reported in bone.

Case Presentation: We describe the case of a 51-year-old male with an intraosseous PILA of the proximal edge of his left clavicle and two other lesions evident on imaging. The patient was treated with marginal resection of the clavicle lesion but was lost to follow-up 1 month after surgery. Read More

Histol Histopathol 2017 Dec 1;32(12):1239-1279. Epub 2017 Aug 1.

Department of Cell Biology and Histology, School of Medicine, Campus of International Excellence, "Campus Mare Nostrum", IMIB-Arrixaca, University of Murcia, Murcia, Spain.

We review the morpho-functional basis of the different types of angiogenesis and report our observations, including the formation of angiogenesis-related secondary structures. First of all, we consider the following issues: a) conceptual differences between angiogenesis and vasculogenesis, b) incidence of angiogenesis in pre- and postnatal life, c) regions of vascular tree with angiogenic capacity, d) cells (endothelial cells, pericytes, CD34+ adventitial stromal cells of the microvasculature and inflammatory cells) and extracellular matrix components involved in angiogenesis, e) events associated with angiogenesis, f) different types of angiogenesis, including sprouting and intussusceptive angiogenesis, and other angiogenic or vascularization forms arising from endothelial precursor cells (postnatal vasculogenesis), vasculogenesis mimicry, vessel co-option and piecemeal angiogenesis. Subsequently, we consider the specific morpho-functional characteristics of each type of angiogenesis. Read More

J Cutan Pathol 2017 Jan 2;44(1):107-109. Epub 2016 Nov 2.

Dermatek, Centro Médico Teknon, Barcelona, Spain.

J Eur Acad Dermatol Venereol 2016 Oct 31;30(10):e59-e61. Epub 2015 Aug 31.

Department of Dermatology, Paracelsus Medical University Salzburg, Muellner Hauptstraße 48, A-5020, Salzburg, Austria.

Zhonghua Bing Li Xue Za Zhi 2015 Jul;44(7):480-5

E-mail:

Objective: To further elucidate the clinical and pathologic features of retiform hemangioendothelioma (RH) and its clinical spectrum.

Methods: Eight cases of RH were reviewed. The clinicopathologic profiles, immunophenotypes and outcome data were investigated. Read More

J Cutan Pathol 2016 Mar 12;43(3):258-62. Epub 2015 Oct 12.

Department of Dermatology, Fundación Jiménez Díaz, Madrid, Spain.

Dabska tumor, also known as papillary intralymphatic angioendothelioma (PILA), is a locally aggressive hemangiendothelioma characterized by intravascular papillary proliferations of atypical endothelial cells. Besides PILA, papillary tufts lined by hobnail endothelial cells have been rarely described in vascular proliferations. We report two cases of acquired hemangiomas, which focally showed this finding. Read More

Pol J Pathol 2015 Mar;66(1):1-2

Diagn Pathol 2013 Sep 24;8:160. Epub 2013 Sep 24.

Department of Pathology, The First Affiliated Hospital, Sun Yat-sen university, 58, Zhongshan Road II, Guangzhou 510080, China.

Unlabelled: Papillary intralymphatic angioendothelioma (PILA) or Dabska tumor is extremely rare, and often affects the skin and subcutaneous tissues of children. Since its first description by Dabska, only a few intraosseous cases have been described in the literature and none of them presents with multifocal osteolytic lesion of bones. We present a case of unusual multifocal intraosseous PILA in facial bones occurring in a 1 year 3 month old male child. Read More

Eplasty 2013 10;13:e46. Epub 2013 Sep 10.

Divisions of General Surgery, Rutgers-Robert Wood Johnson Medical School, New Brunswick.

Objective: The Dabska tumor is a rare low-grade angiosarcoma first described in 1969 by Maria Dabska. Approximately 30 cases of varying presentations have been reported since its initial description.

Methods: We describe a case of a 23-year-old woman presenting with a massive recurrent left flank hemangioendothelioma, at final resection diagnosed to be an endovascular papillary angioendothelioma (Dabska tumor). Read More

Case Rep Pathol 2013 31;2013:531479. Epub 2013 Mar 31.

Institute of Pathology, University of Cologne Medical Center, 50937 Cologne, Germany.

Papillary intralymphatic angioendothelioma is a rare, low-grade neoplasm of lymphatic channels that usually presents intradermally or subcutaneously. We report the case of a 24-year-old male presenting with an isolated intratesticular palpable mass and symptoms of testicular pain. Preoperative ultrasound examination showed an irregular, heterogeneous mass. Read More

Coll Antropol 2012 Nov;36 Suppl 2:171-2

Dr. Ivo Pedisić, General Hospital, Department of ENT Surgery, Sisak, Croatia.

In 1969 Dabska and her colleagues described for the first time this rare malignant tumour, also later known as a malignant endovascular papillary angioendothelioma of childhood. Overall, depending amongst other factors on its location, it is thought to have a generally favourable prognosis and a wide local excision seems to be the treatment of choice. We here present a very rare and unusual case of a 63 year old woman with a 20 year history of slow-growing right buccal dermatological lesion which resembled a common mole. Read More

Semin Diagn Pathol 2013 Feb;30(1):29-44

Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain.

Hemangioendothelioma is the term used to name those vascular neoplasms that show a borderline biological behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas. Although originally spindle cell hemangioendothelioma was proposed as a specific clinicopathologic variant of hemangioendothelioma, currently, it is considered as an entirely benign lesion, and thus, the name spindle cell hemangioma seems to be the most accurate for this lesion. Authentic hemangioendotheliomas involving the skin and soft tissues include papillary intralymphatic angioendothelioma (also known as Dabska tumor), retiform hemangioendothelioma, kaposiform hemangioendothelioma, epithelioid hemangioendothelioma, pseudomyogenic hemangioendothelioma (also known as epithelioid sarcoma-like hemangioendothelioma), and composite hemangioendothelioma. Read More

Pediatr Neurosurg 2012 21;48(1):48-50. Epub 2012 Jul 21.

Department of Pathology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, India.

Encephaloceles arise from developmental defects in neural tube formation. These lesions contain brain and meninges which herniate through a defect in the skull. These may present as isolated malformations or rarely be associated with brain tumors. Read More

Histopathology 2011 Nov 18;59(5):1027-9. Epub 2011 Oct 18.

Am J Dermatopathol 2011 Oct;33(7):e84-7

Department of Pathology, Necker-Enfants Malades Hospital, APHP, Université Paris Descartes, Paris, France.

Retiform hemangioendothelioma (RH) is a rare low-grade malignancy angiosarcoma, with a high rate of local recurrence and a low metastatic risk. A 6 year-old girl with a large cervical cystic lymphangioma diagnosed by ultrasound and Doppler ultrasound, which showed a large multiloculated anechoic cyst with no flow. The lymphangioma was treated with injections of Picibanil (OK-432). Read More

Diagn Cytopathol 2011 Jul 2;39(7):514-6. Epub 2010 Nov 2.

Department of Pathology, Rajendra Institute of Medical Sciences, Ranchi, India.

Here we described a rare case of malignant endovascular papillary angioendothelioma (Dabska tumor) in an adult female. On fine needle aspiration, the smear showed many small clusters of tumor cells with rosettoid arrangement along with papillary fragments with fibrovascular core and hobnail like arrangement of the cells. Histopathological examination revealed a vascular tumor in the form of papillary projection into the vascular lumina, lined by atypical endothelial cells. Read More

Hum Pathol 2011 Jul;42(7):913-7

Dermatology and Pathology, New Jersey Medical School, Newark, NJ 07103, USA.

There is only one cancer to our knowledge named in honor of its female describer, the Dąbska tumor. Maria Dąbska was a brave hero of the Warsaw Resurrection of 1944 who survived deportation to Germany to graduate from medical school in Gdańsk and pursue a career in pathology, for which her research on the Dąbska tumor, breast cancer, sweat gland tumors, keratoacanthoma, soft tissue sarcomas, bone pathology, parachordoma, melanoma, and other entities remains salient. Working behind the Iron Curtain, she nevertheless achieved international recognition, yet few today are cognizant of her and her accomplishments. Read More

Pathol Res Pract 2011 May 11;207(5):327-31. Epub 2011 Mar 11.

Division of Diagnostic Pathology, National Hospital Organization Hokkaido Cancer Center, Sapporo, Japan.

Extra-axial soft tissue chordoma is rare. We report a case of extra-axial soft tissue chordoma of the right wrist in an 87-year-old man. The tumor was large, and the cut surface showed multinodular and myxoid appearance. Read More

J Pediatr Surg 2011 Jan;46(1):e25-8

Division of Plastic Surgery, Department of Surgery, College of Medicine, Milton S. Hershey Medical Center, The Pennsylvania State University, Hershey, PA 17033, USA.

Endovascular papillary angioendothelioma, or Dabska tumor, was first presented in 1969 by Maria Dabska where she presented cases occurring in 6 children. The tumor was described as being a low-grade angiosarcoma characterized by papillary endovascular proliferations of atypical endothelial cells and anastomosing vascular channels within the dermis. Since its original description, only 30 case reports have been written describing the varying presentation, clinical course, and histopathology of this tumor. Read More

Neuro Endocrinol Lett 2010 ;31(4):454-6

Department of Pathology District Specialist Hospital, and Department of Surgery, Medical University of Silesia, Tychy, Poland.

We report an angiosarcoma arising within a malignant endovascular papillary angioendothelioma (Dabska tumor) in soft tissue of the upper thigh/buttock of a 42-year-old woman. Although neoplastic progression within a vascular tumor of an existing low-grade lesion into DT has been described so far, we seem to be the first to report transformation of DT into an angiosarcoma. Read More

Dermatol Online J 2010 Apr 15;16(4). Epub 2010 Apr 15.

University of Minnesota, USA.

The term angiosarcoma, encompasses several neoplasms, all of which exhibit a malignant process derived from endothelial cells of the vessels. The most common form of angiosarcoma is highly aggressive, often fatal, and usually affects the head and neck region of elderly white men. Other low-grade forms of angiosarcoma, including papillary intralymphatic angioendothelioma, also known as Dabska tumor, are less invasive, affect a wider age range, and offer a better prognosis. Read More

Cesk Patol 2009 Jul;45(3):72-4

Ustav patologickej anatómie Jesseniovej lekárskej fakulty a Martinskej fakultnej nemocnice, Martin.

Retiform hemangioendothelioma (RHE) is a rare vascular tumoriform lesion characterized by rete testis--like vascular structures. RHE belongs to a group of vascular tumors of intermediate malignancy and together with Dabska tumor form a category of so-called hobnail hemangioendotheliomas. Authors present a case of a 8-year-old girl with RHE which was located in right calf and measured 12 x 8 mm. Read More

Afr J Paediatr Surg 2009 Jan-Jun;6(1):19-23

Department of Pathology, Ahmadu Bello University Teaching Hospital, Shika, Zaria-810 001, Kaduna State, Nigeria.

Background: The increased prevalence of hitherto uncommon tumours in children in our geographic setting formed the basis for this study. This study aimed to determine the current histopathologic distribution pattern of paediatric malignancies in Zaria.

Materials And Methods: An eight year (2000-2007) consecutive analysis of malignant tumours in children ages 0 to 15 years in a referral University laboratory. Read More

Eur J Pediatr Surg 2009 Oct;19(5):337-9

Department of Histopathology, Shaheed Sadoughi Hospital, Yazd, Islamic Republic of Iran.

J Foot Ankle Surg 2008 Sep-Oct;47(5):487-93. Epub 2008 Jul 31.

Foot and Ankle Surgical Program, Penn Presbyterian Medical Center, Philadelphia, PA 19104, USA.

Unlabelled: An 8-year-old female presented with a recurrent hobnail (Dabska-retiform) hemangioendothelioma of her right foot. After initial tumor excision and subsequent recurrence, the patient underwent a successful tumor resection and forefoot reconstruction using a fillet of second digit flap. In an attempt to create a plantigrade foot, while sufficiently removing this rare pedal tumor, the authors employed the use of a fillet of second digit flap. Read More

Fetal Pediatr Pathol 2008 ;27(3):175-83

Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.

We describe a pediatric case of primary angiosarcoma of the brain displaying striking intravascular papillary pattern, consistent with the "Dabska tumor," often in continuity with a massive, multifocal intravascular papillary endothelial hyperplasia. The tumor contained small hemangioma and obliterated dysplastic arteries as well as very large thin-walled veins. The surrounding brain tissue showed scattered telangiectasias, conglomerates of calcified dysplastic arteries, old hemorrhages and gliosis. Read More

Am J Dermatopathol 2008 Feb;30(1):31-3

Department of Pathology, Medical Center, Medical Center Boulevard, Wake Forest University Baptist, Winston-Salem, NC, USA.

Retiform hemangioendothelioma (RH) is a rare vascular neoplasm most often occurring in the limbs of middle-aged females. This entity is characterized by infiltrative vascular spaces arranged in a pattern similar to the rete testis. RH differs from angiosarcoma by lacking cytologic atypia and high mitotic rates. Read More

J Cutan Pathol 2008 Jan;35(1):65-9

Department of Dermatopathology, Mount Sinai Medical Center, New York, NY 10128, USA.

We describe a case of Dabska tumor (DT) occurring within a large congenital lymphangioma circumscriptum on the thigh of a 14-year-old female. Diagnostic biopsy showed numerous intravascular papillary projections lined by atypical endothelial cells within the anastomosing vascular channels of a lymphangioma circumscriptum. DT is regarded as a vascular tumor of intermediate malignant potential, most probably of lymphatic origin. Read More

J Pediatr Hematol Oncol 2007 Dec;29(12):808-10

Department of Radiology , Tulane School of Medicine, New Orleans, LA, USA.

Papillary angioendothelioma is a rare, low-grade neoplasm of lymphatic channels that usually presents intradermally. We report the case of a 6-year-old girl with isolated splenomegaly and symptoms of early satiety and weight loss, whom was found to have a splenic papillary angioendothelioma. Preoperative abdominal computed tomography scan showed an irregular, heterogeneous mass; a tagged red cell scan ruled out a hemangioma, whereas a positron emission tomography scan showed mildly increased uptake. Read More

Diagn Pathol 2006 Jul 22;1:12. Epub 2006 Jul 22.

Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

The Dabska tumor also known as Endovascular papillary angioendothelioma is a rare type of hemangioendothelioma characterized by intraluminal papillary endothelial structures. Most of these are superficial in location but occurrence in deeper tissues is also known. We describe case report of testicular Dabska tumor in a child presenting as inguinal hernia. Read More

Histopathology 2005 Apr;46(4):396-402

Department of Pathology, the Jikei University School of Medicine, Tokyo, Japan.

Aims: To evaluate the expression of D2-40 in normal lymphatic endothelium and vascular tumours or tumour-like lesions of the skin and soft tissue. D2-40 is a novel monoclonal antibody to a Mr 40 000 O-linked sialoglycoprotein that reacts with a fixation-resistant epitope in lymphatic endothelium.

Methods And Results: Formalin-fixed paraffin-embedded sections from 30 normal tissue samples, including skin, soft tissue, stomach, and colon, and 84 vascular tumours or vascular tumour-like lesions were immunostained with monoclonal antibodies to D2-40 and CD31. Read More

J Oral Pathol Med 2004 Apr;33(4):249; author reply 250-1

Orthopedics 2004 Mar;27(3):327-8

Department of Orthopedic Surgery, Kyoto City Hospital, Kyoto, Japan.

J Oral Pathol Med 2003 Sep;32(8):492-5

Department of Oral and Maxillofacial Surgery, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan.

Endovascular papillary angioendothelioma (EPA), known as Dabska tumor, is a very rare vascular neoplasm that usually occurs in the skin or subcutis of infants and young children. There is no previous report of EPA appearing in the oral cavity. Here, we present an exceptionally rare case of EPA of the tongue in a 67-year-old man. Read More

J Am Acad Dermatol 2001 Feb;44(2 Suppl):395-8

Deparment of Pathology, Hospital General de Móstoles, Madrid, Spain.

Spiradenoma is a benign skin adnexal neoplasm that usually appears as a solitary nodule in any area of the body. There have been only 33 reports on malignant transformation of a benign eccrine spiradenoma since the first description by Dabska in 1972. Most cases have originated on a long-standing cutaneous lesion. Read More

Dermatology 2000 ;201(1):1-5

Dermatology and Pathology, New Jersey Medical School, Newark, N.J., and Pathology, State University of New York at Buffalo School of Medicine, Buffalo, N.Y., USA.

The Dabska tumor is a rare low-grade angiosarcoma also known as malignant endovascular papillary angioendothelioma of childhood. It primarily affects the skin of children and has a distinctive histologic pattern of anastomosing vascular channels with intravascular papillary outpouchings projecting, sometimes, in a glomerulus-like pattern, into a lumen lined by atypical columnar endothelial cells. Since its original description 30 years ago by one of us, knowledge in this area has expanded. Read More

Mod Pathol 2000 Feb;13(2):180-5

Department of Pathology, Emory University, Atlanta, Georgia, USA.

Recently, a novel monoclonal antibody to vascular endothelial growth factor receptor 3 (VEGFR-3), a tyrosine kinase receptor expressed almost exclusively by lymphatic endothelium in the adult, has been shown to react with a small number of cases of Kaposi's sarcoma (KS) and cutaneous lymphangiomas. We sought to extend these studies to a large number of well-characterized vascular neoplasms to evaluate diagnostic uses of this antibody and to determine whether it defines them in a thematic fashion. Formalin-fixed, paraffin-embedded sections from 70 vascular tumors were immunostained with antibodies to VEGFR-3 von Willebrand factor (vWF), and CD31. Read More

Am J Surg Pathol 1999 Sep;23(9):1004-10

Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.

Six childhood vascular tumors were designated as "malignant endovascular papillary angioendothelioma" by Dabska in 1969. Since then, a few reports of similar cases were published, often called "Dabska tumors." Twelve similar cases were identified in review of vascular tumors from the authors' institutions. Read More

Skeletal Radiol 1999 Feb;28(2):100-3

The Johns Hopkins Hospital, Department of Pathology, Baltimore, MD 21287, USA.

Endovascular papillary angioendothelioma, also known as Dabska tumor, is a rare vascular neoplasm that usually involves the skin or subcutaneous tissue of children. There have been no reported cases of this lesion occurring in bone. We report a Dabska tumor in the distal femur of a 45-year-old woman who, clinically and radiologically, was felt to have an osteoid osteoma. Read More

Pathol Int 1998 Oct;48(10):840-1

The histological diagnosis of endovascular papillary angioendothelioma (EPA) (Dabska tumor) is controversial although the tumor is included in the World Health Organization classification. Papillary endothelial proliferation with a central hyaline core is one of the most characteristic features of EPA; however, this type of proliferation has been observed in other vascular tumors, such as angiosarcoma, retiform hemangioendothelioma and glomeruloid hemangioma. Several vascular tumors have EPA-like foci and EPA is not well defined generally. Read More

Am J Surg Pathol 1998 Jun;22(6):683-97

Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC, USA.

The clinicopathologic, immunohistochemical, and ultrastructural features of soft tissue angiosarcomas are not well defined. Eighty cases of angiosarcoma that involved the deep subcutis, skeletal muscle, retroperitoneum, mesentery, and mediastinum are reported. The lesions occurred in 50 male and 30 female patients who were 5-97 years of age; the peak incidence was in the seventh decade of life. Read More

Pathol Int 1998 Feb;48(2):164-7

Department of Pathology (Hospital), Hyogo College of Medicine, Nishinomiya, Japan.

A patient with endovascular papillary angioendothelioma with a low grade of malignancy showing papillary proliferation of endothelioid cells is presented. The patient, an 83-year-old woman, underwent resection of a tumor of the neck. At operation a 9 x 7 cm cystic tumor containing yellow transparent liquid with clots was found in the subcutaneous tissue. Read More

Arch Pathol Lab Med 1997 Sep;121(9):992-5

Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.

We report a rare malignant endovascular papillary angioendothelioma (Dabska tumor) arising within the deep periosteal soft tissue of the ulna of an 8-year-old girl. To our knowledge, this is the first report of a Dabska tumor of deep soft tissue that did not extend down from the dermis. This Dabska tumor appears to be a focal change within a larger, well-defined, cavernous hemangioma of deep muscle. Read More

Am J Dermatopathol 1996 Jun;18(3):302-7

Department of Dermatology, Hospital General Universitario, Valencia, Spain.

Malignant endovascular papillary angioendothelioma, first described by Dabska in 1969, is a rare vascular tumor that primarily affects children and is characterized by papillary proliferations of endothelial cells into vessel lumens. We report a case of this rare neoplasm in a boy with angiomatosis who developed Kasabach-Merritt syndrome. The tumor evolved as an ulcerated lesion superficially within a previous vascular malformation on his buttock. Read More

Ann Pathol 1994 ;14(3):168-73

Laboratoire d'Anatomie Pathologique, Institut Pasteur de Nouméa.

Malignant change in a benign eccrine spiradenoma is a rare but definite phenomenon. Since it was first reported by Dabska, 23 cases have been reported. All cases present recent changes in a long standing cutaneous nodule which, when excised and examined microscopically, disclosed carcinoma associated and arising from benign eccrine spiradenoma. Read More

Am J Dermatopathol 1989 Feb;11(1):64-8

Department of Pathology, Mount Sinai Medical Center of Greater Miami, Florida 33140.

Malignant endovascular papillary angioendothelioma is a rare vascular tumor of childhood that was first defined by Dabska in 1969. Microscopically, this tumor is composed of anastomosing vascular channels, some of which contain papillary projections or tuft-like structures sometimes resembling renal glomeruli. Although cases have been reported with metastases to regional lymph nodes, the long term prognosis for patients with these tumors is generally good. Read More

Rev Laryngol Otol Rhinol (Bord) 1984 ;105(2):121-3

Cancer 1983 Apr;51(7):1230-7

Almost a quarter of a century has passed since mesenchymal chondrosarcoma, a rare, insufficiently studied and still poorly understood cartilage neoplasm has been described. Based on 35 cases diagnosed and treated at this Cancer Center, this study found 20 males and 15 females with an average age of 26 years (range, 6-70 years). All but five of the tumors arose in the skeleton in the femur, humerus, and ilium in five cases each, while the os calcis (a rare site for any other osseous tumor) gave rise to this tumor in four instances. Read More