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Apocrine adenocarcinoma arises from the sweat glands of the body, which are densely located in the axillae, areola and nipple of breast, ear canal, perianal area, and external genitalia.
However, these sweat glands are also present in the caruncle region of the eye. We present a rare case of rapidly progressing primary apocrine adenocarcinoma of the caruncle in an elderly male.
Case description
A 60-year-old male presented to us with complaints of rapidly increasing swelling over the medial side of the right eye of 3 months duration. On clinical examination his visual acuity in the both eyes was 6/9 with normal pupillary reflexes. Both eye anterior and posterior segment examination was normal. On examining the mass, there was a firm pinkish elevated lesion of 10 mm × 15 mm with lobulated surface seen at the left caruncular region with inferior eyelid fornix involvement (Fig. 1A). Eyelid margin and skin was free of tumour. There was limitation of adduction of the involved eye. Contrast enhanced computed tomography of the orbit showed an enhancing mass lesion in the medial orbital space extending from the medial canthus, displacing the globe laterally (Fig. 1B). Positron emission tomography scan, ultrasound abdomen, and chest tomography were normal, ruling out any metastasis or primary foci. A biopsy was done, which made a confirmatory diagnosis of apocrine adenocarcinoma of the caruncle. Due to orbital extension, an exenteration was performed followed by radiotherapy.
Fig. 1A, Caruncular mass in left eye with normal eyelid architecture. B, Axial cuts of computed tomography scan to show enhancing mass in the medial orbital space extending from medial canthus.
Gross examination of the exenterated orbital contents revealed 2.5 × 2.5 × 2.0 cm tumour medial to the eyeball (Fig. 2A). On light microscopy the tumour cells were arranged in lobules with focal tubular and glandular differentiation (Fig. 2B). The tumour cells had abundant eosinophilic cytoplasm and prominent nucleoli. Periodic acid-Schiff (PAS) positive diastase-resistant granules (Fig. 2C) and Alcian-blue PAS positivity was also seen. Immunohistochemistry on formalin fixed paraffin embedded sections revealed positivity for cytokeratin (CK-7), gross cystic fluid protein 15 (GCDFP-15) (Fig. 2C), carcinoembryonic antigen, and androgen receptors (AR), suggesting a malignant tumour of sweat gland origin. Immunostains were negative for CK-20, CDX2, thyroid transcription factor (TTF), and prostate specific antigen (PSA), ruling out primary from colon, lung, and prostate, respectively. Immunostains for estrogen (ER) and progesterone (PR) receptors as well as HER2 were negative, ruling out metastatic apocrine breast carcinoma. Based on these findings a diagnosis of primary caruncular apocrine adenocarcinoma was made.
Electron microscopy revealed intermediate filaments in bundles, secretory granules, and pleomorphic mitochondria (Fig. 3A−C). The patient was referred to radiotherapy due to orbital extension. Postexenteration no recurrence has been noted at 4-month follow-up.
Fig. 3Transmission electron micrographs showing features of apocrine adenocarcinoma. A, Cytoplasm containing bundled intermediate filaments (arrow) B, Abundant glycogen granules in the cytoplasm (arrows) C, Pleomorphic mitochondria (arrow).
Sweat gland tumours of the periocular region are rare and may be eccrine or apocrine. In the periorbital and orbital region these arise from the glands of Moll, which are modified sweat glands present in the eyelids. To date only 23 cases of apocrine adenocarcinoma of the eyelid are reported in literature. Of these, 19 cases were reviewed by Figueira et al. in 2013.
The sweat glands are also present in the caruncle of the eye. To our knowledge only 1 case of primary apocrine adenocarcinoma of the caruncle has been described in literature. Duke et al. reported a 34-year-old male presenting with slow growing medial canthal swelling of 18 months duration with orbital extension. An exenteration was done in their case with no recurrence noted at 21 months follow-up.
In comparison, our case was an elderly male of 60 years old presenting with a rapidly progressing caruncular mass of 3 months duration with orbital extension, and postexenteration no recurrence was noted at 4 months follow-up.
For diagnosis of apocrine adenocarcinoma, the following criteria were described by Kipkie and Haust for histopathological diagnosis: (i) PAS positive, (ii) strongly eosinophilic cytoplasm, (iii) decapitation secretion, (iv) iron-positive intracellular pigment, and (v) occurrence in areas where apocrine glands are normally located.
It is a marker of adnexal tumours including breast. Metastatic adenocarcinomas from breast, prostate, colon, and thyroid need to be ruled out by performing a panel of immunohistochemical stains including PSA, CDX2, TTF, ER, PR, and HER2.
The current management guidelines are ill-defined due to the paucity of data described in literature. For smaller tumours wide local excision with margin control has been performed. Those with orbital extension required exenteration and those cases with lymph node metastasis required regional lymphnode dissection. The role of chemotherapy is still not clear; however, radiotherapy has been used as an adjunct with exenteration in cases with aggressive metastatic spread. Primary determinants of survival in patients with eyelid apocrine adenocarcinomas include lymph node metastasis, local (orbital, periorbital) invasion, and systemic metastasis.
To conclude, due to presence of sweat glands, primary apocrine adenocarcinoma can arise from the caruncle and should be considered as a rare differential of tumours in this region. Clinicopathological correlation and immunohistochemical panel are invaluable in arriving at the final diagnosis.
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