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Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 343-344

Eccrine acrospiroma: A rare malignant tumor of the eyelid

Government Medical College, Amritsar, Punjab, India

Date of Submission30-Jun-2021
Date of Acceptance08-Oct-2021
Date of Web Publication13-Apr-2022

Correspondence Address:
Gaurang Sehgal
Regional Institute of Ophthalmology, Government Medical College, Amritsar, Punjab - 143 001
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1747_21

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Eccrine acrospiroma are benign skin tumors of sweat duct origin which are occurring in eyelids. They present as small solid or cystic lesions that are confused clinically with other lesions. This is a case of eccrine acrospiroma with malignant potential of a 90 year-old female with mass over the ventral aspect of the left upper eyelid with bleeding, ulceration, and discharge. This is a rare presentation of histopathological that confirmed eccrine acrospiroma so as to alert the clinician about the differential diagnosis of mass over eye lids. By careful examination, meticulous wide excision and rigorous follow-up potential vision can be restored.

Keywords: Eccrine acrospiroma, eyelid, swelling

How to cite this article:
Sehgal G, Bal P, Singh K, Chopra R, Setia S. Eccrine acrospiroma: A rare malignant tumor of the eyelid. Indian J Ophthalmol Case Rep 2022;2:343-4

How to cite this URL:
Sehgal G, Bal P, Singh K, Chopra R, Setia S. Eccrine acrospiroma: A rare malignant tumor of the eyelid. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 May 26];2:343-4. Available from: https://www.ijoreports.in/text.asp?2022/2/2/343/342898

In a general out-patient setting, masses on eye lids and adnexa are common presentations, which in turn can be eye lid tumors. Eccrine sweat glands are widely distributed in the body, and each gland consists of a single duct with a coiled deeper component. By contrast, apocrine sweat glands are limited to special regions such as axilla, nipple, external ear, external genitalia, and the eyelids.

Eccrine acrospiroma is a benign cutaneous tumor arising from the epithelial cells of eccrine sweat ducts.[1] They usually present as small solid or cystic lesions confused clinically with other solid or cystic lesions.[2] Malignant form of acrospiromas arise from the intradermal duct of eccrine sweat glands and account for approximately 6% of malignant eccrine tumors. This tumor occurs in young adults, more frequently in women than men.[3] Ulceration may occur on the lesion.[3],[4] This tumor has an aggressive behavior with more than 50% local recurrence rates. Wide surgical excision is the treatment of choice.

Occasionally, eccrine acrospiroma, a benign tumor of probable eccrine gland origin, arises in the skin of the eyelid.[5],[6],[7],[8] There is limited information about the clinical features of eccrine acrospiroma involving the eyelids.

  Case Report Top

A 90- year-old female presented to us with mass over the ventral aspect of left upper eyelid. The lesion started 10 years back as small peasized mass, which gradually increased to form a large, lemon sized mass. It was associated with onandoff foul-smelling discharge and bleeding from the surface of the mass. There were frequent episodes of fresh red bleeding and discharge from the surface of the masses which decreased on pressure and lying down, whereas it increased on sitting and after manipulation during examination.

The patient gives history of excision of the mass 6 years back, details of which are available with the patient. On first presentation in the OPD for the recurrence, the patient was advised pressure bandage with adequate antibiotic cover and nonsteroidal antiinflammatory drugs.

On examination, a pigmented mass measuring 3 cm × 1.5 cm × 3 cm was observed having necrotic margins, soft to firm in consistency, nonpulsatile, nontender, noncompressible, multiloculated, and extended from medial canthus to lateral canthus on the left eye lid margin.

The magnetic resonance imaging of the orbit showed lobulated mass lesion measuring 3.3 × 1.7 × 2.8 cm on the right eye lid with the areas of T1 hyperintensity and foci of T2 hypointensity seen within the lesion. Compression of globe superiorly was present. Cystic foci and multiple thick enhancing septations were seen within the lesion. There were no extensions to brain, extraconal space, or peripheral nervous system.

Complete blood counts, C-reactive protein levels, and kidney and liver function tests were within normal limit. There was no local lymph node swelling or palpable mass in the body.

Under peribulbar anesthesia, the mass was excised with wide and adequate margins and was sent for histopathological examination. On gross examination, a skin attached globular gray black grey brown soft tissue piece measuring 3 × 2.4 × 1.5 cm. On cutting, the inner surface is gray white gray brown [Figure 1].
Figure 1: Preoperative picture showing mass extending from lateral canthus to medial canthus on upper eyelid, measuring about 30 mm × 15 mm

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On microscopic examination: hemotoxylin & eosin stained sections showed normal stratified squamous epithelium, beneath which is a well of circumscribed dermal nodule showing sheets and whorls of round to spindly cells with vesicular nuclei and pale pink to clear cell cytoplasm. Nuclear rooving seen was seen. Increased mitosis seen, increased vascular with areas of hemorrhages, and pigment containing macrophages were also seen. On final impression, findings were those of appendigeal tumor eccrine acrospiroma [Figure 2].
Figure 2: (a) H and E section showing normal stratified squamous epithelium, with circumscribed dermal nodule showing sheets and whorls of round to spindly cells with vesicular nuclei. (b) Nuclear rooving (circled with black), increased mitosis, increased vascular with areas of hemorrhages, and pigment containing macrophages also seen

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  Discussion Top

Clinically, eccrine acrospirioma presents as nodular and cystic lesions. The characteristic feature of this tumor is that the skin over the tumor is smooth and either flesh colored, red, or blue at times. It can be thickened and papillary.

Typical microscopy of malignant eccrine acrospiroma shows cellular atypia, increased mitoses, local areas of infiltration and necrosis, and perineural and angiolymphatic invasion.[9] In this case, a dermal nodule with sheets and whorls of round to spindly cells was seen with nuclear rooving, increased vascularity, and increased mitosis. Malignant transformation of benign eccrine acrospiromas has also been reported[9] earlier as in our case.

On rigorous follow-up, till date the there are no complaints of recurrence of mass or bleeding episodes. The visual acuity has not declined below 6/60 (partial). The differential diagnosis for a mass like this includes hemangioma, squamous cell carcinoma, melanoma, metastatic tumors, and other adnexal tumors.[10]

What once we thought to enucleate the eye, on clinical examination, precise and wide excision, and ocular findings after removal of the mass such as slightly edematous cornea, vertically dilated pupil with subconjunctiva bleed with finger counting at bedside [Figure 3]; the vision was restored up to 6/60 (partial). Dilated pupil can be attributed to compression of optic atrophy due to constant increased pressure on eye ball due to mass.
Figure 3: Postoperative picture after complete removal of the mass showing the margins of mass on the upper eye lid. Edematous cornea, subconjunctival bleed, and a sluggishly reacting pupil can also be appreciated

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Though eccrine acrospiroma are benign skin tumors of sweat duct origin, they usually present as small solid or cystic lesions that are confused clinically with other solid or cystic lesions, but this case report is to bring into consideration the rare presentation of eccrine acrospiroma so as to alert the clinician about the differential diagnosis of bleeding, ulcerating mass over eye lids, which could in turn be a presentation for malignant eccrine acrospiroma of the eyelid. Also, it was thought that eye behind the mass would have been affected and could lead to enucleation, but with meticulous wide excision and preservation of eyeball potential vision can be restored. To best of our knowledge and after exhaustive reviewing of medical literature, this is the first case of malignant Eccrine acrospirioma of eyelid in the subcontinent.

Limitations: Frozen section and immunohistochemistry could not been done as facilities were not available.

  Conclusion Top

When evaluating lesions on eyelid with bleeding and ulceration, eccrine acrospiroma should be kept as a differential diagnosis. If suspected, proper local and systemic examination should be done to look for malignant transformation of this tumor.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Johnson BL Jr, Helwig EB. Eccrine acrospiroma: A clinicopathologic study. Cancer 1969;23:641-57.  Back to cited text no. 1
Mahipathy SRRV, Durairaj AR, Sundaramurthy N, Rajamanohar VC, Sivagnanam PS. Large eccrine acrospiroma of the hand. J Clin Diagn Res 2017;11:PD15-6.  Back to cited text no. 2
Kersting OW. Clear cell hidradenoma and hidradenocarcinoma. Arch Dermatol 1963;87:323-33.  Back to cited text no. 3
Winkelmann RK, Wolff K. Solid-cystic hidradenoma of the skin. Clinical and histopathologic study. Arch Dermatol 1968;97:651-61.  Back to cited text no. 4
Liu Y. The histogenesis of clear cell papillary carcinoma of the skin. Am J Pathol 1949;25:93-103.  Back to cited text no. 5
Boniuk M, Halpert B. Clear cell hidradenoma or myoepithelioma of the eyelid. Arch Ophthalmol 1964;72:59-63.  Back to cited text no. 6
Font RL. Eyelids and lacrimal drainage system. In: Spencer WH, editor. Ophthalmic Pathology: An Atlas and Textbook. 3rd ed, vol 3. Philadelphia: WB Saunders; 1986. p. 2219.  Back to cited text no. 7
Ferry AP, Haddad HM. Eccrine acrospiroma (porosyrongoma) of the eyelid. Arch Ophthalmol 1970;83:591-3.  Back to cited text no. 8
Laws RA, English JC 3rd, Elston DM. Acrospiroma: A case report and review. Cutis 1996;58:349-51.  Back to cited text no. 9
Hunt SJ, Santa Cruz DJ, Kerl H. Giant eccrine acrospiroma. J Am Acad Dermatol 1990;23:663-8.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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