To determine the occurrence and clinical characteristics of ectopic pituitary adenoma (EPA) in China.

The study are done by searching systematically and comprehensively on major Chinese and English medical literature databases and conference papers before 2015, which are only limited to collected, summarized, sorted and analyzed EPA cases that reported by Chinese authors in English or Chinese occurred in China.

① Among the 86 Chinese articles and 27 English articles related to ectopic pituitary adenoma (EPA) gathered by the author, except for cases that have been confirmed as repeated reports, a total of 73 EPA cases were found. Of 70 cases with complete data, 31 were male cases, accounting for 44.29%; 39 were female cases, accounting for 55.71%; the ratio is 1:1.3. The frequency of EPA occurrence according to the location of the lesion, from high to low in turn, was sphenoid sinus (31 cases, 42.47%) , nasopharynx (7 cases, 9.59%) , suprasellar region (7 cases, 9.59%) , clivus (5 cases, 6.85%) , followed by the third ventricle, sphenoid sinus/clivus, nasal cavity, and the temporal lobe of the brain, with the same incidence of 4.11%. According to the functional properties of EPA, the frequency of different secreting hormones adenoma was PRL-ma (21 cases, 28.77%) , NF-ma (21 cases, 28.77%) , ACTH-ma (15 cases, 20.55%) , GH-ma (10 cases, 13.70%) , TSH-ma (2 cases, 2.74%) and FSH-ma (1 cases. 1.37%) . Three cases of EPA were uncertain in their property due to lack of information. The incidence of PRL and nonfunctional tumors was the highest, which was different from what was reported in other countries. Among them, one case of EPA was in pineal region and one in parapharyngeal space, which was even more rare and were never reported abroad. ② Except for 3 cases with incomplete medical records, 15 out of 70 cases of EPA were accompanied by empty sella, accounting for 21.43%, among which 11 (73.33%) cases involved the sphenoid sinus, and 3 (20%) cases involved clivus. The sphenoid sinus and clivus cases together accounted for 93.33%. ③ 29 out of the 69 cases of EPA with complete record were invasive pituitary adenomas, accounting for 42.03% and including 1 case of pituitary adenocarcinoma, which accounted for 1.45%. ④ All cases were treated with surgery as the first choice, and some of them were treated with radiotherapy or drug therapy.

Ectopic pituitary adenoma is extremely rare. By the end of 2015, the total number of cases reported in China is only 73, which are mostly located in the sphenoid sinus, suprasellar region and nasopharynx. In the functional classification, the most common types are still PRL adenoma and nonfunctional adenoma as in situ pituitary adenoma. 42.03% of EPA adenomas are invasive. Surgical resection of EPA is the first choice and some cases can be treated with radiotherapy and drug therapy.

Ectopic pituitary adenoma (EPA); Ectopic pituitary adenocarcinoma; Invasive ectopic pituitary adenomas; Empty sella