Giant and atypical eccrine tumors of the eyelid: A clinicopathologic analysis

Abstract

Objective:

To analyse the clinicopathological features, diagnostic pitfalls, and unusual presentations of eyelid eccrine tumors.

Methods:

Retrospective case series of 25 histopathologically confirmed eyelid eccrine tumors surgically excised between 2016 and 2023. Clinical records, histopathology (H&E with selective PAS staining), and follow-up data were reviewed.

Results:

Eccrine hidrocystoma was the most common subtype (80%, n=20), followed by eccrine hidradenoma (16%, n=4) and eccrine spiradenoma (4%, n=1). Mean patient age was 43.8 years, with a 4:1 male predominance. Clinical misdiagnosis occurred in 84% of lesions, most commonly as sebaceous cysts (64%). cases demonstrated unusual features, including three giant hidrocystomas (>2 cm), one giant hidradenoma (3.0 cm) with ulceration and mechanical ptosis clinically suspicious for squamous cell carcinoma, and one spiradenoma with maggot infestation mimicking malignancy. All giant tumors required excision with reconstruction (e.g., staged Cutler-Beard procedure). No recurrences were observed during follow-up (mean 14 months; range 6–24 months).

Conclusion:

Eyelid eccrine tumors present a broad clinical spectrum, from small cysts to large masses mimicking malignancy. Giant lesions, though rare, cause diagnostic confusion and functional impairment. Histopathological examination remains the gold standard for definitive diagnosis, and heightened clinical awareness is essential to avoid misdiagnosis.

Sweat gland tumors are rare adnexal neoplasms arising from either eccrine or apocrine glands. While they most commonly involve the scalp, face, and trunk, involvement of the ocular adnexa—particularly the eyelid—is exceptionally rare. Yu et al.[1] reported that eccrine and apocrine eyelid tumors constituted only 13 of 2228 adnexal tumors (0.6%) in their cohort, underscoring their rarity. This observation aligns with broader literature, which places eccrine tumors among the least frequent adnexal neoplasms of the eyelid.[1]

The most frequently encountered eccrine tumors include eccrine hidrocystoma (EHC), eccrine hidradenoma (EHA), and eccrine spiradenoma (ES). Their diagnosis is often challenging because of nonspecific clinical presentations that mimic benign cystic lesions such as chalazion or sebaceous cysts and, at times, malignant tumors.[2] Eccrine hidradenoma—also referred to as clear cell hidradenoma or eccrine acrospiroma—arises from the distal excretory duct of eccrine glands and typically presents as a slow-growing solid-cystic lesion.[3] Although commonly reported in the head and neck region of middle-aged females, eyelid involvement is exceedingly rare. Only three eyelid cases have been reported over the past decade, none exceeding one-third of the eyelid.

Eccrine hidrocystomas are ductal retention cysts that clinically present as translucent, skin-colored to bluish papules or nodules, most often in the periorbital region of middle-aged women.[4,5] Lesions larger than 10 mm are uncommon and are designated as “giant eccrine hidrocystomas.”[6] Eccrine spiradenomas are benign intradermal tumors, characteristically painful, and usually occur on the trunk. Eyelid involvement is extremely rare, with only a handful of cases reported worldwide.[7]

This series presents a retrospective case series of 25 histopathologically confirmed eyelid eccrine tumors, emphasizing seven unusual presentations, including giant lesions and frequent clinical misdiagnosis.

Methods

This retrospective study included 25 eyelid eccrine tumors surgically excised between 2016 and 2023. Clinical records and histopathological reports were reviewed for demographic data, clinical presentation, lesion size and location, preoperative diagnosis, surgical procedure, and follow-up information. Hematoxylin and eosin (H and E) staining was performed on all specimens, with selective Periodic Acid Schiff staining. Inclusion criteria comprised all histopathologically confirmed eyelid eccrine tumors surgically excised between 2016 and 2023, with apocrine tumors excluded based on morphological criteria including the presence of decapitation secretion and abundant eosinophilic cytoplasm. The study was approved by the Institutional Ethics Committee, and informed consent was obtained from all patients for use of clinical photographs.

Results

Among 25 histopathologically confirmed eyelid eccrine tumors, eccrine hidrocystoma was the predominant subtype, accounting for 80% of cases (n = 20). Eccrine hidradenoma comprised 16% (n = 4), while eccrine spiradenoma represented 4% (n = 1) of cases. The patients’ ages ranged from 16 to 84 years, with a mean age of 43.8 years. A notable male predominance was observed, with a male to female ratio of 4:1. The left upper eyelid was the most frequent site of involvement (52%), followed by the lower eyelid (32%) and medial canthus (16%).

Clinical misdiagnosis occurred in the majority of lesions (84%), most commonly as sebaceous cysts (64%), followed by chalazion, meibomian cyst, dermoid cyst, or nodular squamous cell carcinoma. The lesion size ranged from 0.2 to 3.0 cm. Seven cases demonstrated unusual features including giant size (>2 cm), atypical location, or unusual clinical presentation mimicking malignancy. In all cases, definitive diagnosis was achieved through a structured workflow of histopathological confirmation following surgical excision or incisional biopsy, underscoring the limitations of clinical assessment alone.

Category 1: Eccrine hidrocystomas – unusual presentations

Among the 20 eccrine hidrocystomas, five cases (25%) demonstrated unusual features warranting detailed description.

Giant Eccrine Hidrocystoma (Case 2): A 33-year-old male presented with a progressively enlarging left upper eyelid mass causing mechanical ptosis [Fig. 1a-c]. Clinical examination revealed a large cystic mass spanning the entire upper eyelid [Fig. 1d]. Magnetic resonance imaging demonstrated a well-defined T2 hyperintense cystic lesion measuring 25 × 25 × 15 mm [Fig. 1e]. Surgical excision yielded a 2 × 2 × 1 cm cyst with 3 ml yellow serous fluid. Histopathological examination revealed a fibrocollagenous cyst wall lined by a double layer of cuboidal epithelium without apocrine features [Fig. 1f]. Periodic Acid Schiff staining was negative. The patient showed no recurrence at 1 year follow-up.

Figure 1.

Eccrine hidrocystomas – Spectrum of unusual presentations. (a-c) Clinical photographs of Case 2 (33-year-old male) showing enlarging left upper eyelid mass causing mechanical ptosis. (d) Excised specimen and (e) MRI (axial and coronal) demonstrating a well-defined T2 hyperintense cystic lesion (25 × 25 × 15 mm). (f) Histopathology showing the cyst wall lined by a double layer of cuboidal epithelium (H and E, 100×). (g) Histopathology of Case 4 (55-year-old male) showing large cysts lined by 2–3 layers of cuboidal epithelium (H and E, 400×). (h) Histopathology of Case 5 (65-year-old male) showing multiple cystic spaces lined by cuboidal cells (H and E, 200×). (i) Clinical photograph of Case 3 (28-year-old female) showing diffuse conjunctival cyst on the inner surface of the right lower eyelid. (j) Histopathology showing cyst lined by cuboidal epithelium over fibrocollagenous stroma (H and E, 200×)

Additional Giant Hidrocystomas (Cases 4 and 5): Two additional cases presented with unusually large hidrocystomas. A 55-year-old male had a medial canthus cystic mass measuring 5 × 4 × 1.5 cm (Case 4), while a 65-year-old male presented with a left lower eyelid swelling of 4 × 3 × 1 cm (Case 5). Both lesions were painless and had been present for 7–12 months. Histopathology in both cases demonstrated multiple large cystic spaces lined by 2–3 layers of cuboidal epithelium resting on fibrocollagenous stroma, with adjacent unremarkable adnexal glands [Fig. 1g and h]. These represented giant eccrine hidrocystomas.

Unusual Conjunctival Presentation (Case 3): A 28-year-old female presented with a right lower eyelid cystic mass for 4 months that increased with perspiration. Clinical examination revealed a diffuse translucent conjunctival cyst on the inner surface of the lower eyelid [Fig. 1i]. Partial excision showed a cyst lined by cuboidal epithelium over fibrocollagenous stroma [Fig. 1j], consistent with eccrine hidrocystoma in an unusual conjunctival location.

Medial Canthus Hidrocystoma (Case 7): A 55-year-old male presented with a medial canthus cystic swelling measuring 6.5 × 8 mm, clinically diagnosed as a sebaceous cyst. Excision revealed a cystic lesion lined by cuboidal epithelium on fibrocollagenous stroma, confirming eccrine hidrocystoma.

Category 2: Eccrine hidradenoma – unusual giant presentation

Among the four eccrine hidradenomas, one case (25%) demonstrated truly exceptional features.

Giant Eccrine Hidradenoma (Case 1): An 84-year-old male presented with a 3-year history of an enlarging left upper eyelid mass that exhibited rapid growth over the preceding 3 months. Clinical examination revealed a large pigmented, lobulated mass measuring 3.0 × 2.0 × 1.8 cm involving the left upper eyelid, associated with mechanical ptosis, surface ulceration, madarosis, and focal pigmentary changes [Fig. 2a-c]. The lesion was clinically suspicious for nodular squamous cell carcinoma.

Figure 2.

Giant eccrine hidradenoma with atypical features. (a-c) Clinical photographs of Case 1 (84-year-old male) showing large pigmented, lobulated mass over the left upper eyelid with ulceration, madarosis, and mechanical ptosis. (d) Post Cutler–Beard Stage I reconstruction. (e) Stage II surgery at 3 months. (f) Histopathology showing well-circumscribed solid-cystic dermal tumor (H and E, 100×). (g) High power view showing polyhedral tumor cells with eosinophilic to clear cytoplasm (H and E, 400×). (h) High power view focal sebaceous differentiation (H and E, 400×)

Surgical management involved excision with 4 mm margins followed by staged reconstruction. Stage I Cutler–Beard procedure was performed for lid reconstruction [Fig. 2d], followed by stage II lid separation at 3 months [Fig. 2e].

Gross examination revealed a gray-white solid tumor with cystic areas. Histopathological examination showed a well-circumscribed dermal tumor arranged in sheets and nodules of polyhedral cells with eosinophilic to clear cytoplasm and oval nuclei [Fig. 2f and g]. Focal sebaceous differentiation was noted [Fig. 2h]. No cytologic atypia or increased mitotic activity was observed. The features were diagnostic of nodular eccrine hidradenoma. The patient showed no recurrence at 9 months follow-up.

Category 3: Eccrine spiradenoma – unusual presentation with infestation

Eccrine Spiradenoma (Case 6): A 72-year-old male presented with an extensive swelling involving the left upper and lower eyelids, associated with ulceration and maggot infestation [Fig. 3a]. The clinical presentation was highly suspicious for squamous cell carcinoma with secondary infestation. An incisional biopsy was performed, yielding multiple gray-brown tissue bits measuring 1.2 × 1.0 × 0.3 cm.

Figure 3.

Eccrine spiradenoma with unusual ulceration and infestation. (a) Clinical photograph of Case 6 (72-year-old male) showing ulcerated swelling involving left upper and lower eyelids with maggot infestation, clinically mimicking squamous cell carcinoma. (b) Histopathology showing well-circumscribed dermal tumor arranged in solid sheets and trabeculae (H&E, 100×)

Histopathological examination revealed a well-circumscribed dermal tumor arranged in solid sheets and trabeculae [Fig. 3b]. The tumor demonstrated a characteristic dual cell population: large pale cells with vesicular nuclei centrally and peripheral basaloid cells with hyperchromatic nuclei. No necrosis, significant mitotic activity, or cytologic atypia was observed. These features confirmed the diagnosis of eccrine spiradenoma.

This case represents only the fifth reported case of eyelid eccrine spiradenoma in the literature and is unique due to the advanced patient age, extensive ulceration, and secondary infestation contributing to clinical misinterpretation as malignancy.

All lesions in this series were managed surgically, with the approach tailored to lesion type and size. Small hidrocystomas underwent simple excision with primary closure. Giant tumors required more extensive resection: The giant hidradenoma (Case 1) underwent excision with 4 mm margins and staged Cutler–Beard reconstruction, while the giant hidrocystoma (Case 2) was managed by gentle excision with preservation of lid architecture. The spiradenoma case (Case 6), clinically suspicious for malignancy, underwent incisional biopsy for diagnosis followed by definitive excision. All patients had uneventful postoperative recovery with good functional and cosmetic outcomes, assessed through clinical evaluation of lid position, vision, and ocular surface status at regular follow-up examinations. Follow-up periods ranged from 6 months to 2 years (mean 14 months), with no recurrences documented in any case.

Discussion

Eccrine tumors of the eyelid are uncommon and frequently under-recognized because of their varied and deceptive clinical presentation. In the present series, eccrine hidrocystoma was the predominant subtype, consistent with earlier reports.[1,2,3] Most lesions were clinically misdiagnosed, reinforcing prior observations that adnexal tumors of the eyelid cannot be reliably identified on clinical grounds alone. Deprez and Uffer highlighted the diagnostic difficulty posed by eyelid adnexal tumors, noting that hidrocystomas may clinically resemble melanocytic lesions because of their bluish hue.[5]

The most striking findings in this series were the two giant eccrine tumors, both of which were clinically suspected to be malignant. Giant eccrine tumors are rarely reported, particularly on the eyelid, and are more commonly described in the head and neck region.[6,7] Their size, ulceration, and associated functional impairment often necessitate wide excision and reconstructive procedures. Although eccrine hidradenomas are benign, rare malignant transformation has been documented, underscoring the importance of complete excision and histopathological evaluation.[8,9]

Eccrine spiradenoma of the eyelid remains exceedingly rare. Only four cases have been reported previously, with only one documented over the past two decades.[10] The present case was unusual due to the advanced age of the patient, extensive ulceration, and secondary infestation, all of which contributed to clinical misinterpretation as malignancy.

Conclusion

Eyelid eccrine tumors are rare adnexal neoplasms with a wide spectrum of clinical appearances, ranging from small translucent cysts to large solid or cystic masses mimicking malignancy. Eccrine hidrocystoma was the most common subtype, followed by eccrine hidradenoma and eccrine spiradenoma. Giant lesions, though uncommon, can cause significant diagnostic confusion and functional impairment. Histopathological examination remains the gold standard for definitive diagnosis, and heightened clinical awareness is essential to ensure accurate diagnosis and appropriate management.

Authors’ contributions

Dr. Savita Agarwal: Conceptualization, Formal histopathological analysis and diagnosis, Data curation, Provision of pathology images, Writing – original draft preparation, Writing – review and editing. Dr. Reena Sharma: Conceptualization, Patient diagnosis and management, Surgical intervention, Data curation, Writing – original draft preparation, Writing – review and editing. Dr. Beant Kaur: Conceptualization, Formal histopathological analysis and diagnosis, Data curation, Provision of pathology images, Writing – original draft preparation, Writing – review and editing. Dr. Pinki Pandey: Histopathological analysis, Data validation, Writing – review and editing. Dr. Surendra Kumar: Histopathological analysis, Data validation, Writing – review and editing. Dr. Anuradha Gaur: Patient management, Surgical intervention, Data collection, Writing – review and editing.Dr. Alka Yadav: Histopathological analysis, Data validation, Writing – review and editing.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.