Papillary intralymphatic angioendothelioma (Dabska tumor) is a rarely metastasizing lymphatic vascular neoplasm that usually affects children and young adults. The majority of these cases occur in soft tissues of extremities, and to date less than 40 cases have been described. Despite the generally indolent evolution, can be locally invasive with the potential to metastasize.
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| Dabska's tumour - Violaceous lesion, with poorly defined limits, on the sole of the right foot. |
Papillary intralymphatic angioendothelioma (PILA or Dabska tumor) is a rare vascular neoplasm, defined as low grade malignancy tumor, with high tendency of local recurrence.
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| Histopathology. Papillary projections with vascular axes lined by rounded and hyperchromatic cells – “Hobnail cells” (Hematoxylin & eosin, ×100). |
Originally considered a malignant tumor and called malignant endovascular papillary hemangioendothelioma, it was renamed in 1998, due to its borderline behavior, prominent presence of lymphatic vessels and the presence of intravascular papillary proliferation.
📖 Diagnostic Pathology: Soft Tissue Tumors 3rd Edition
To date, less than 40 cases have been reported in the literature, mostly affecting soft tissue. Affects mainly children and young adults, and may be present at birth, without predilection for sex. Its diagnosis is often a challenge for the pathologist due to its rarity, multifocality and morphological characteristics. Clinically, the lesion may present as an intradermal nodule or discrete superficial nodule, with slow growth, purplish, pink or bluish coloration and large variation in size (up to 40 cm).
In some cases, it may present with superficial ulceration and/or satellite nodules. It is most commonly located in the dermis and subcutaneous cellular tissue of the extremities, and can also affect the trunk, head and neck, with rare cases described in deeper locations – such as spleen, tongue, testis and bones.Histopathologically, the tumor is characterized by presenting, in the dermis and/or subcutaneous tissue, thin-walled intercomposite vessels lined by endothelial hobnail cells, forming the characteristic intraluminal papillary projections, which assume a focal pattern in rosettes or “match-head”. Glomeruli-like structures may be present. Mitoses are rare and necrosis is absent.
The immunohistochemical study demonstrates positivity for VEGFR-3 and podoplanin (D2-40) in the hobnail endothelial proliferations, indicative of lymphatic differentiation.8, 9, 10 The differential diagnosis is made with reactive angioendotheliomatosis, benign intravascular endothelial hyperplasia and retiform hemangioendothelioma, which present negative immunohistochemistry for podoplanin (D2-40). It has a generally indolent development, however, it may be locally invasive, with rare reports of dissemination to regional lymph nodes and even distal metastases. The gold standard treatment is wide surgical excision with free margins, which presents an excellent prognosis.Thus, its recognition by the dermatologist becomes important to determine early diagnosis and treatment. Long-term clinical follow-up of these patients is mandatory.
Maria Dabska (1920 - 2014), Polish pathologist.
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| Maria Dabska |
Maria Dabska as born Maria Suchy. She graduated from the faculty at Gdañsk about 1950 and began working as an assistant to professor Wilhelm Czarnocki (1886-1962) at the Department of Pathological Anatomy in Gdañsk. In 1953 she moved to Warsaw to the Department of Tumour Pathology in the Maria Sklodowska Institute of Oncology. Her chief was professor Józef Laskowski (1900-1970), a leading Polish oncopathologist.
The so-called "Laskowski's histo-clinic school" includes regular consultations and analyses of unusual oncologic cases from the all territory of Poland. This was the inspiring form of post-graduate education of Polish pathologists and the source of many first original descriptions of interesting and rare neoplasms, e.g. aponeurotic epithelioid sarcoma.
Unfortunately the discussions and conclusions were published mainly in the Polish language. One of such examples of rare cases was a series of six low-grade vascular tumours of childhood collected by Dabska and described by her in 1969 as "malignant endovascular papillary angioendothelioma", now often called "Dabska tumour". Thirty years later Dabska was the co-author of the retrospective analysis of this controversial entity.
After Laskowski's death in 1970, Dabska became chief of the department. She was author and co-author of many impressive histological and histoclincal analyses of tumours and their microscopical peculiarities, especially soft tissue tumours, bone tumours, thyroid cancers and skin tumours. Among her other achievements was the first description of a new oncological entity she named "parachordoma", in 1977. In her English-language papers she popularized the name of professor Laskowski in the western literature.
In 1982 Dabska moved to Austria with her son, and later to West Germany, near Hamburg. For a few year she worked as a prosector in Germany. In the end of 1980s, she moved to the United States where she worked in a university in Florida. Her son is a dermatopathologist.
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