Paget's carcinoma
| Classification according to ICD-10 | |
|---|---|
| C50- | Malignant neoplasm of the mammary gland |
| C21.0 | Malignant neoplasm of the anus and anal canal; Anus, unspecified |
| C44.5 | Other malignant neoplasms of the skin; Skin of the trunk (anus: skin, edge (area); skin of the mammary gland, perianal skin) |
| C44.6 | Other malignant neoplasms of the skin; Skin of the upper extremity, including the shoulder |
| ICD-10 online (WHO version 2019) | |
The Paget's disease of the breast , and Paget's disease , Paget's disease , Paget's disease of the nipple or rare Paget's disease of the vulva , is a very rare form of cancer in the nipple and rarely on other locations. Women are almost always affected. The disease is mostly based on a deeper ductal carcinoma in situ (2/3 of the cases) or a ductal breast carcinoma . If there is no other underlying carcinoma , it is referred to as an isolated Paget carcinoma.
Occasionally, Paget's carcinoma also occurs in men and as a blistered tumor ( bowenoid papulosis ) outside the breast, mostly in the genital area , anus or armpits (possibly involving the apocrine sweat glands ).
The Paget's disease of bone , also known as Paget's disease , Paget's disease , or Paget's disease referred to, is a skeletal disorder and has in common with Paget's disease of the breast, only the first person to describe and name.
Paget's carcinoma was described in the scientific literature by James Paget in 1874 .
Symptoms
The mostly one-sided tumor lying in the skin initially resembles an inflammatory change, with a crusty, scaly, brown-red skin surface, sometimes oozing, not painful or not very painful. At this stage there is a risk of confusion with simple eczema of the nipples, which, however, usually occurs on both sides. In Paget's carcinoma, over time, a very typical sign of breast cancer, the nipple pulls in.
Etiology and histology
It is assumed that in mammary Paget's carcinoma the cells originate from in-situ or invasive ductal breast carcinoma cells , in extramammary manifestations there is uncertainty about the origin of the cells, since here often no CIS or other carcinoma could be detected, but some authors assume indicates that these are also degenerate gland cells. This thesis is supported by the cell shapes, the intracellular mucin , and the existing glandular cytokeratins ( e.g. KRT7 + KRT19 ). In addition, epithelial membrane antigens and carcinoembryonic antigens are often detected.
In the tissue section under the microscope, moderate acanthosis and penetration of the stratum basale (often also the milk ducts) with paga cells can be seen . These are large cells without tonofibrils with a light, glycogen-rich cytoplasm and a large, oval, hyperchromic nucleus .
Therapy and prognosis
If the disease is detected at an early stage, surgical removal of the tumor is usually sufficient, otherwise chemotherapy or radiation is also carried out . If the disease is recognized early and there are no complications, the chances of recovery are good. In Paget's disease of the vulva, a generous excision in healthy tissue or vulvectomy is recommended as well as treatment with imiquimod as an immunomodulation therapy and, if the breast is affected, with chemotherapy, radiotherapy or radiochemotherapy. Therapy attempts with local chemotherapy , laser treatment and photodynamic therapy were also tried ; in addition, some cells show androgen receptors, which suggests anti-androgen therapy as a treatment option.
The prognosis depends on the treatment chosen and is difficult to determine based on the rarity of the disease and its association with other invasive tumors.
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- ↑ Paget J. On disease of the mammary areola preceding cancer of the mammary gland. St Bartholemew Hospital Research London 1874; 10: 87-9.
- ↑ a b J. Lloyd, AM Flanagan: Mammary and extramammary Paget's disease . In: Journal of Clinical Pathology . 53, No. 10, Jan 10, 2000, ISSN 1472-4146 , pp. 742-749. doi : 10.1136 / jcp.53.10.742 . PMID 11064666 . Retrieved February 17, 2014.
- ↑ a b Bernhard Schüssler, Joachim Diebold: Paget's disease of the vulva . In: Frauenheilkunde Aktuell . January 18, 2009. Retrieved February 17, 2014.
- ↑ Serena Bertozzi, Ambrogio P. Londero, Arrigo Fruscalzo, Diego Marchesoni, RJ Lellé: Paget's disease of the vulva: remission through local treatment with imiquimod - case report and literature review . In: Gynecological-obstetric review . 49, No. 4, 2009, ISSN 1423-0011 , pp. 326-330. doi : 10.1159 / 000301110 . Retrieved February 17, 2014.
