Shahbazi Taha, Abdollahi Yeganeh Soheil, Borzouei Shiva, Arjipour Mahdi
Neurosurgery Research Group (NRG), Student Research Committee, Hamadan University of Medical Sciences, Hamadan, Iran.
Department of Endocrinology, School of Medicine, Hamedan University of Medical Sciences, Hamadan, Iran.
J Med Case Rep. 2024 Dec 22;18(1):604. doi: 10.1186/s13256-024-04967-5.
Giant prolactinoma (size > 4 cm) is a rare condition and accounts for less than 1% of pituitary adenomas. In even rarer cases, these lesions may involve craniocervical structures requiring surgical intervention. The present case is the largest reported giant prolactinoma (99 × 72 × 57 mm). It led to cranial settling and significant central herniation at the foramen magnum, causing quadriparesis.
The patient was a 39-year-old Iranian man from a village around Hamadan city who presented with quadriparesis and paresthesia as well as loss of libido and impotence. Magnetic resonance imaging and computed tomography showed a huge diffuse avid enhancing infiltrating osteolytic lesion at the skull base. It had spread predominantly extramurally into the skull base resulting in cranial deposition, significant central herniation, myelopathy, and acute exacerbation of quadriparesis. On the basis of the pathology, immunohistochemistry, and elevated serum prolactin levels, the diagnosis of giant prolactinoma was almost definite. In addition to urgent midline suboccipital craniotomy for the central herniation and quadriparesis, he was treated with cabergoline followed by occiput cervical fixation and fusion to control the progressive subsidence.
Prolactinoma that is treatable with dopamine agonists should be considered as a differential diagnosis of skull base lesions, even those that are extradural, diffuse, and infiltrative. Giant prolactinoma can lead to craniocervical settling and central herniation that requires surgery and multidisciplinary management.
巨大泌乳素瘤（直径>4 cm）是一种罕见疾病，占垂体腺瘤的比例不到1%。在更为罕见的情况下，这些病变可能累及颅颈结构，需要进行手术干预。本病例是已报道的最大的巨大泌乳素瘤（99×72×57 mm）。它导致了颅骨沉降以及枕骨大孔处明显的中央疝形成，引起四肢瘫。
患者为一名39岁的伊朗男子，来自哈马丹市周边的一个村庄，出现四肢瘫、感觉异常以及性欲减退和阳痿症状。磁共振成像和计算机断层扫描显示颅底有一个巨大的弥漫性强化浸润性溶骨性病变。它主要向颅外扩散至颅底，导致颅骨沉积、明显的中央疝形成、脊髓病以及四肢瘫急性加重。根据病理、免疫组化以及血清泌乳素水平升高，巨大泌乳素瘤的诊断几乎明确。除了因中央疝形成和四肢瘫紧急进行枕下中线开颅手术外，患者还接受了卡麦角林治疗，随后进行枕颈固定融合术以控制病情进展性沉降。
即使是硬膜外、弥漫性和浸润性的颅底病变，可使用多巴胺激动剂治疗的泌乳素瘤也应被视为鉴别诊断之一。巨大泌乳素瘤可导致颅颈沉降和中央疝形成，需要手术及多学科管理。
