Prompt management is essential in the treatment of PCA in order to reduce the possibility of developing metastatic disease. Due to its rarity, the best treatment(s) for PCA have not been formally studied and remain unclear. Tumor excision using wide local excision margins or Mohs micrographic surgery to ensure that all malignant tissue is removed have been the most often recommended treatments for localized PCA. Removal of all tumor cells is necessary for curative therapy. Metastases are more likely to be present with PCA lesions that: a) develop spontaneous bleeding, ulceration, sudden itching, pain, or rapid growth; b) are accompanied by enlarged sentinel lymph nodes, i.e. lymph nodes draining the tumor site; c) consist of poorly differentiated cells as detected on biopsy; d) penetrate 7 mm or more into the skin; e) contain rapidly proliferating cells as defined by the presence of 14 or more cells undergoing mitoses per high power microscopic field; or f) occur in the perineum, trunk, or lower limb. In these cases, studies recommend that patients be more fully examined for metastases before surgery using lymphadenectomy (i.e. lymph node dissection) in order to check for metastasis in, and remove, lymph nodes draining the site of the skin tumor and by magnetic resonance, positron emission tomography–computed tomography, X-ray, ultrasound imaging methods to detect metastases. PCA cases in which surgical resection of the primary tumor does not remove all malignant cells or the tumor recurs at its site of removal (~20% recur at the primary site within a year of excision) have been treated with repeated resections and/or local radiation therapy. Adjuvant radiation therapy may also be useful to treat localized APC tumors that are at high-risk of metastasizing such as tumors that are >5 cm in maximal diameter, have invaded lymphatic vessels, or contain poorly differentiated cells. Patients that failed to have their disease completely removed with surgical/radiation/lymphadenectomy procedures or have surgically inaccessible metastases are usually treated with various drug regimens. These regimens have included 5-fluorouracil (i.e. 5-FU) alone, 5-FU & cisplatin, 5-FU & Adriamycin & paclitaxel, docetaxel & carboplatin, paclitaxel & carboplatin & intralesional (i.e. injected directly into the lesion) interleukin 2, topical diphenylcyclopropenone (a drug which is primarily used to treat warts), intralesional interleukin 2 alone, interferon alfa & isotretinoin, topical 5-FU, intra-arterial (i.e. injection of a drug into a small artery that supplies blood to a tumor) docetaxel, or the immunotherapy drug, pembrolizumab. The two patients treated with pembrolizumab obtained a complete and excellent responses that continued at follow-up examinations done respectively 24 and 18 months after treatment. In general, however, there has been no highly and repeatedly successful drug treatments specified for metastatic PCA: the cited drug treatment regimens have been used in too few cases to be properly evaluated and in general produced no, minimal, or only very short-term benefits.
Porocarcinoma
Poroma
Choriocarcinoma
Embryonal carcinoma
Embryoma
Polyembryoma
Seminoma
Leiomyosarcoma