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Introduction <p>Primary apocrine adenocarcinoma (PAA) is a rare malignant neoplasm that typically arises in apocrine-rich sites such as the axilla, anogenital area, and scalp, whereas orbital involvement without periocular lesions is exceptionally uncommon.</p> Case report <p>We report a 61-year-old male with right eye pain, swelling, proptosis, and limited abduction. MRI showed a medial orbital mass with well-defined but infiltrative margins, invading extraocular muscles, encasing the superior ophthalmic vein, and extending into the ethmoidal air cells. PET/CT excluded systemic malignancy, and histopathology confirmed apocrine adenocarcinoma with immunohistochemical positivity for CK7, AR, GCDFP-15, and GATA3. Including this case, only nine orbital PAAs have been reported, all in males localized to the medial orbit.</p> Conclusion <p>The absence of native apocrine structures suggests an origin from ectopic or embryologic remnants. PAA should be considered in the differential diagnosis of medial orbital tumors, and management requires histological confirmation, surgical excision, and close follow-up due to risk of recurrence.</p>

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Primary apocrine adenocarcinoma of the orbit: case report and literature review

  • 期刊论文

  • 发表日期：

    2026年4月24日

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  • Murat Iren,
  • Ipek Balaban,
  • Fergan Bol,
  • Ece Iren,
  • Maide Mureva,
  • Berkan Cetin,
  • Bahar Atasoy

摘要

Introduction

Primary apocrine adenocarcinoma (PAA) is a rare malignant neoplasm that typically arises in apocrine-rich sites such as the axilla, anogenital area, and scalp, whereas orbital involvement without periocular lesions is exceptionally uncommon.

Case report

We report a 61-year-old male with right eye pain, swelling, proptosis, and limited abduction. MRI showed a medial orbital mass with well-defined but infiltrative margins, invading extraocular muscles, encasing the superior ophthalmic vein, and extending into the ethmoidal air cells. PET/CT excluded systemic malignancy, and histopathology confirmed apocrine adenocarcinoma with immunohistochemical positivity for CK7, AR, GCDFP-15, and GATA3. Including this case, only nine orbital PAAs have been reported, all in males localized to the medial orbit.

Conclusion

The absence of native apocrine structures suggests an origin from ectopic or embryologic remnants. PAA should be considered in the differential diagnosis of medial orbital tumors, and management requires histological confirmation, surgical excision, and close follow-up due to risk of recurrence.