Pigmented Papillary Epithelial Neoplasm of the Pituitary Fossa,
A Rare Disease - A Case Report
Dr. Sravana Kumari Chintam *1, Dr Rantna Devi Ramadas 1, Dr Subathira Balasundaram 1
*Correspondence to: Dr. Sravana Kumari Chintam, Apollo Speciality Hospital, Chennai, Tamilnadu-600035, India.
Copyright
© 2026 Dr. Sravana Kumari Chintam is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Received: 29 September 2026
Published: 05 October 2026
DOI: https://doi.org/10.5281/zenodo.23208490
Abstract
Pigmented papillary epithelial neoplasm of the pituitary region is a very rare benign entity. This rare tumour of little known natural history and behavior of the disease was described in only 3 cases till date. The optimal treatment for this rare tumour is not established yet. We report a case of Pigmented papillary epithelial neoplasm of the pituitary fossa in a 33-year-old woman. She was treated with surgical excision followed by local radiotherapy. This case is being reported due to its rarity.
Key words: Pigmented papillary epithelial neoplasm, Pituitary fossa, Radiotherapy.
Introduction
Primary pigmented intracranial neoplasms are very uncommon.[1] The differential diagnosis includes epithelial and non-epithelial tumors. This group includes melanoma, melanocytoma, melanotic ependymoma, sub-ependymoma;[2] pigmented choroid plexus papilloma or carcinoma and melanotic astrocytoma.[3-6] Only few cases have been reported till date to the best of our knowledge.[7-8] The present case is a pigmented papillary epithelial neoplasm of pituitary fossa in a 33yr old lady.
Case Report
This 33-year-old lady presented with the complaints of headache, giddiness, defective vision on right side, galactorrhea and amenorrhea for 3 months duration. Magnetic resonance imaging [MRI] of brain revealed well defined dumbell shaped enhancing sellar and suprasellar mass lesion causing mass effect over the optic chiasm. The lesion was hyperintense on T1 weighted images and hypointense on T2 weighted images.[Fig.1.] She was treated with transnasal trans-sphenoidal excision of the lesion. Histopathological examination was consistent with pigmented papillary epithelial neoplasm. Post-operative MRI brain revealed residual disease. She was given a course of external beam radiotherapy to a dose of 54Gy in 30 fractions over 6 weeks duration while respecting surrounding normal tissue tolerance levels. Patient was asymptomatic with complete regression of the tumour after 3 years of treatment.
Discussion
Pigmented papillary epithelial neoplasm is a distinctive central nervous system tumour. The differential diagnosis of pigmented intracranial neoplasms is limited. In our case histopathological examination showed a cellular neoplasm composed of cords and anastomosing trabeculae lined by columnar to polygonal cells having abundant eosinophilic cytoplasm, round nucleus with prominent nucleoli. Few intranuclear inclusions are noted. Atypia was mild to moderate. There was extensive intracytoplasmic blackish pigmentation. [Fig.2.] The tumour showed weak and focal positivity for CK, CAM5.2 and EMA which was suggestive of epithelial origin. Vimentin and S100 showed strong and diffuse positivity and negative for HMB45 which rules out melanoma. The tumour was negative for Synaptophysin and Chromogranin which was not in favour of pituitary adenoma. Although this entity is very rare, it should be included in the differential diagnosis of tumors arising from pituitary region. Additional experience will be needed to further characterize this unusual tumour.
Conclusion
This case is being reported due to rarity of pigmented papillary epithelial neoplasm of the pituitary fossa. PPEN is a rare pituitary fossa tumour with little knowledge about tumour behavior and and management. Further experience with a large number of cases is needed to establish optimal treatment protocols.
References