CASE REPORT
A RARE AND UNIQUE CASE OF ECCRINE SPIRADENOMA OF THE EYELID
H. R. Padmini, Dinesh P.
1.
2.
Professor & HOD. Department of Ophthalmology, Adichunchanagiri Institute of Medical Sciences.
Assistant Professor. Department of Ophthalmology, Adichunchanagiri Institute of Medical Sciences.
CORRESPONDING AUTHOR:
Dr. H.R. Padmini,
Professor & HOD,
Department of Opthalmology,
Adichunchanagiri Institute of Medical Sciences,
Mandya, Karnataka.
E-mail: drpdinesh@gmail.com
Ph: 0091 8951522929
ABSTRACT: Rare group of tumors with potential for local destruction and metastasis,
Taxonomically segregated into 2 main groups, as follows: those that are histologically similar to
certain benign appendage tumors (e.g., sclerosing sweat duct carcinoma, porocarcinoma,
malignant chondroid syringoma, malignant nodular hidradenoma, and malignant eccrine
spiradenoma) and those that show a diverse array of histologic features, not recapitulating to
any degree aspects of a benign counterpart derived de novo from any portion of the normal
eccrine apparatus or result from the transformation of an existing benign eccrine tumor. An
etiologic role for UVR has been suggested; Abbate et al reported that 5 out of 10 patients with
MAC gave a prior history of radiation therapy. Chiller et al reported a potential etiologic role for
UVR as they describe MAC predominantly affecting the left side of the face, corresponding to
higher UVR exposures while driving. Immunosuppression has been known to increase the risk
of nonmelanoma skin cancers, particularly squamous cell carcinomas, Harwood et al suggested
that patients who are immunosuppressed have a propensity to also develop cutaneous
appendageal tumors over their immunocompetent counterparts, with increased rates of both
benign eccrine tumors and malignant eccrine tumors.
REPORT OF A CASE: It is exceedingly rare, accounting for roughly 1 of 13,000 specimens
submitted to a dermatopathology laboratory. The more common subtypes include microcystic
adnexal carcinoma, eccrine porocarcinoma, and hidradenocarcinoma. The less common
subtypes include eccrine mucinous carcinoma, malignant eccrine spiradenoma, malignant
mixed tumor, malignant cylindroma, and papillary eccrine adenoma. Only several hundred cases
of eccrine carcinoma have been reported in the literature worldwide. Many of these tumors
metastasize (up to 60%), with a fatal outcome. MAC was previously only described in white
patients; however, Peterson et al and Gardner et al reported the first and second cases of MAC
affecting African Americans, respectively. Sex incidence would appear to be equal for eccrine
carcinoma, although this has not been definitively stated. Exceptions to this are the malignant
chondroid syringoma and primary cutaneous adenoid cystic eccrine carcinoma, both of which
occur more commonly in females than in males. Eccrine carcinomas most commonly are
diagnosed in patients in their fifth through eighth decades of life. Here we describe a unique and
a rare case of malignant eccrine spiradenoma which mimicked like a basal cell carcinoma,
squamous cell carcinoma or a malignant melanoma.
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CASE REPORT
A 65 year old female presented with a single, asymptomatic, nondescript cutaneous
lesion at the right lower lid margin, growing slowly over years, reaching a size of several
centimeters over a few months. There was slight pain and pruritus but no spontaneous
bleeding. There was no history of local trauma. Examination revealed a 2 x 1.8x1 cm blue grey
firm, lobulated mass at the right lower lid margin near the lateral canthus. There were satellite
papules surrounding the tumor. No significant lymphadenopathy was noted. Her past medical
history was unremarkable. She denied any family members with similar skin findings or any
history of skin cancers. Local examination revealed multiple, well-circumscribed, subcutaneous,
blue-grey nodules near the right lower lid margin near the lateral canthus. The nodules were
tender, firm, and fixed to the overlying skin. MRI demonstrated low-signal intensity on T1weighted images and high-signal intensity on short tau inversion recovery images. An
excisional biopsy with lid reconstruction and cheek rotation flap was performed to obtain a
representative sample of the lesion; this sample was sent for histopathologic evaluation to make
the diagnosis. On histopathological examination the nodule consisted of sharply demarcated
lobules located in the dermis without connection to the epidermis, there were groups of cells in
cords, islands and sheets. Two types of cells were present; there were small, dark, basaloid cells
with hyperchromatic nuclei and cells with large, pale, vesicular, and ovoid nuclei. The lesions
were filled with lymphocytes. The Cells were arranged around small lumina, the Lumina
contained small amounts of granular eosinophilic material that was PAS- positive, dilated
vessels were seen. Excellent cosmetic and functional results were seen and as there were no
metastases seen in the MRI, the patient was advised to return for future excisions in the case of
increasingly painful or rapidly growing lesions.
COMMENT: Spiradenomas seem to be caused by a defective tumor suppressor gene. In BrookeSpiegler syndrome, a defect exists in the CYLD gene located on chromosome 9. The actual cause
of a solitary spiradenoma has yet to be defined. Radiation, hyperthermic limb perfusion
chemotherapy, and chemotherapy are used in the treatment of malignant spiradenomas, the
mainstay of treatment of spiradenomas and malignant spiradenomas is surgical removal, Mohs
micrographic surgery offers the most conservative treatment choice Multiple spiradenomas,
such as those found in Brooke-Spiegler syndrome can be treated with a high-energy continuous
wave carbon dioxide laser after debulking with bipolar scissors. Debulking with bipolar scissors
prior to laser therapy can be a beneficial technique in the surgical removal of large tumors.
Management during the course of metastatic spiradenoma includes surgery, radiation therapy,
hyperthermic limb perfusion chemotherapy, and chemotherapy, in particular tamoxifen.
Surgery is usually curative; therefore, further care is not needed. If malignant spiradenomas
occur, the patient will need to follow up with oncologists, radiation oncologists, and radiologists.
Most spiradenomas are benign and stable and do not require treatment. Malignant
spiradenomas require treatment, and it is metastatic in about 50% of cases. If metastatic and
untreated, malignant spiradenomas are fatal.
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