Spiradenomas (SA) are rare, benign cutaneous adnexal tumors that may progress to become their malignant counterparts, i.e. spiradenocarcinomas (SAC).
SA tumors usually occur as slow-growing,[5] single, small, nodular lesions located in the skin of the head, neck, or trunk.
[9] Currently, SA[3] and SAC[5] are usually treated by complete excision making sure that no tumor cells are left behind at the surgical site.
[5] In addition to surgical resection, some cases of SAC tumors have been treated with adjuvant therapy that includes radiotherapy and/or chemotherapy.
[10] These tumors usually present as a solitary, sometimes painful,[11] slowly growing, 1–2 cm (i.e. centimeters), gray-to-pink nodules that lie underneath the skin's epidermis in the head, neck, trunk, arms, or legs.
[14] Areas of the skin that do not have hair follicles (e.g. palms of the hands and soles of feet) do not develop these nodules.
[5] In earlier studies, SAC tumors were reported to varied in size with 40% being larger than 5 cm and the majority not having metastasized; the sites to which these lesions metastasized were most commonly to lymph nodes near to the SAC or, rarely, distant tissues such as the lung, liver, brain, spinal cord, bone, skin, and breast.
[6][15][16] As determined by the microscopic histopathological appearances of their hematoxylin and eosin-stained samples, spiradenomas are non-encapsulated nodular skin lesions that extend into the dermis.
The SAC component of these lesions consists of cells that, along with their nuclei and cytoplasm, vary greatly in size and shape.
Individuals with CCS commonly develop an increasingly large number of skin tumors, including SA and SAC, over time.
Usually, these individuals are readily distinguished from sporadic SA and SAC by their family history of the disease and the presence of large numbers of tumors.
Each check-up should incorporate the examination of the patients' regional lymph nodes and on a case-by-case basis annual chest X-rays and liver function tests.
[5] Many studies recommend wide local surgical excision of these tumors with surgical margins of ≥1 cm in order to ensure that all tumor cells are removed: local lymph node resections are usually reserved for those cases suspected of having lymph node metastases.
[6] Since SA tumors may contain unidentified areas of malignancy and therefore have high recurrence rates, may metastasize to local lymph nodes and/or distant tissues, and can result in overall poor survival times,[16] adjuvant radiation therapy has been used to minimize these aggressive events in SA tumors with equivocal signs of malignancy.
[5] Chemotherapeutic agents such as methotrexate, cisplatin, etoposide, and 5-fluorouracil have been used to treat SAC with distant tissue metastases but the success of these drug treatments has been limited.