Evolution of a refractory prolactin-secreting pituitary adenoma into a pituitary carcinoma: report of a challenging case and literature review.

BACKGROUND: Pituitary carcinomas (PCs), defined as distant metastases of pituitary neoplasms, are very rare malignancies. Because the clinical presentation of PCs is variable, early diagnosis and management remain challenging. PCs are always refractory to comprehensive treatments, and patients with PCs have extremely poor prognoses. CASE PRESENTATION: We describe one case of a prolactin-secreting pituitary adenoma (PA) refractory to conventional therapy that evolved into a PC with intraspinal metastasis. A 34-year-old female was diagnosed with an invasive prolactin-secreting PA in 2009 and was unresponsive to medical treatment with bromocriptine. The tumor was gross totally removed via transsphenoidal surgery (TSS). However, the patient experienced multiple tumor recurrences or regrowth despite comprehensive treatments, including medical therapy, two gamma knife radiosurgeries (GKSs), and four frontal craniotomies. In 2016, she was found to have an intradural extramedullary mass at the level of the fourth lumbar vertebra. The intraspinal lesion was completely resected and was confirmed as a metastatic PC based on histomorphology and immunohistochemical staining. The literature on the diagnosis, molecular pathogenesis, treatment, and prognosis of patients with prolactin-secreting PCs was reviewed. CONCLUSION: PCs are very rare neoplasms with variable clinical features and poor prognosis. Most PCs usually arise from aggressive PAs refractory to conventional therapy. There is no reliable marker to identify aggressive PAs with a risk for progression to PCs; thus, it is difficult to diagnose these PCs early until the presence of metastatic lesions. It is still very challenging to manage patients with PCs due to a lack of standardized protocols for diagnosis and treatment. Establishing molecular biomarkers and the pathobiology of PCs could help in the early identification of aggressive PAs most likely to evolve into PCs.

A comparative analysis of ESM-1 and vascular endothelial cell marker (CD34/CD105) expression on pituitary adenoma invasion.

OBJECTIVE: Pituitary adenomas are benign neoplasms that display invasive behavior-a characteristic traditionally associated with malignancy-through an ill-defined mechanism. The role of angiogenesis-related molecules in this pathological condition remains perplexing. Our purpose is to assess the impact of endocan (endothelial cell specific molecule-1, ESM-1), CD34 and CD105 on pituitary adenoma invasion. METHODS: In this study, immunohistochemical analyses for endocan, CD34 and CD105 were performed on paraffin-embedded samples of 66 pituitary adenomas, five normal pituitaries, and five primary hepatic carcinomas. Knosp tumor grades based on magnetic resonance imaging coronal scanning were used to assess the invasiveness of each sample. The associations between endocan expression, CD34/CD105-positive microvessel densities (MVDs), and Knosp tumor invasion grades were evaluated. RESULTS: These results showed that endocan protein expression in tumor cells (TCs) was higher than that in endothelial cells (ECs) and strongly correlated with Knosp grades (P < 0.001, Spearman's r = 0.616). Moreover, while endocan-positive TCs localized around the blood vessels in adenomas with higher Knosp grades, no significant association was found between CD34/CD105-MVDs and Knosp grades (CD34: P = 0.256, r = 0.142; CD105: P = 0.183, r = 0.166). Normal pituitary seemed to exhibit lower endocan expression and contained more CD34/CD105-MVDs than pituitary adenomas. CONCLUSION: Endocan expresses in both TCs and ECs of pituitary adenoma. Endocan overexpression in TCs more accurately reflects invasiveness compared to that of CD34/CD105-MVDs and that angiogenesis may not be the primary driver of endocan-medicated pituitary adenoma invasion.

[Expressions of aquaporin-4, matrix metallo-proteinase-2 and matrix metallo-proteinase-14 in peritumor edematous zone of glioma and clinical implications].

OBJECTIVE: To distinguish the expressions of matrix metallo-poteinase and aquaporin in peritumor edematous zone and normal brain tissue for different pathological levels of glioma and explore the relationship of glioma cell invasiveness and brain edema. METHODS: The immunohistochemical method of SP was employed to detect the expressions of aquaporin-4 (AQP-4), matrix metallo-proteinase-2 (MMP-2) and matrix metallo-proteinase-14 (MMP-14) in glioma and normal brain tissue. Due to a rarity of glioma Grades I and II, grades I and II glioma were pooled into low malignancy group (LMG) and grades III and IV into high malignancy group (HMG). The software program SPSS 11.0 was used for Kolmogorov-Smirnov test of independent samplets. The differences were detected between normal brain tissue and LMG and HMG. Also the relationship of AQP4, MMP-2 and MMP-14 was analyzed. RESULTS: With the advancing pathological grades of glioma, the expression of AQP-4, MMP-2 and MMP-14 were higher in positive areas. There were significant deviations among 3 groups. CONCLUSION: The expressions of AQP-4, MMP-2 and MMP-14 in normal brain tissue and all levels of glioma edema are positively correlated. And there is a close correlation between glioma invasiveness and edema extent.

[Therapeutic strategy for intrasellar cystic lesions].

OBJECTIVE: To make a clear distinction of intrasellar cystic lesions: craniopharyngioma (CR), Rathke's cleft cyst (RCC), cystic pituitary adenoma (PA) and intrasellar arachnoid cyst (AC). METHODS: A total of 72 adult patients underwent transsphenoidal approach for the removal of intrasellar cystic lesions. The authors conducted a study to (1) investigate preoperative clinical, biochemical and radiographic features of patients with CR, RCC, PA and AC; (2) identify clinicopathological features of independently predicting recurrence in CR, PA and RCC in adults. These adult patients included CR (n = 10), RCC (n = 27), cystic PA (n = 32) and 1 patient with AC (n = 1). RESULTS: The CR patients presented with hypopituitarism in 80% of cases. According to the biochemical criteria, the percentage of patients with a slight prolactin increase happened in PA (55.6%) and RCC (44.4%). Cystic PA had post-contrasting enhancement in cyst wall because of tumor tissue. Calcification detectable on computed tomographic scanning was present in 70% of CR patients. It was a significantly greater proportion compared to other diseases. Typical RCC was located between anterior and posterior pituitary and the contents in RCC were variable. Mass effects vanished post-operatively in all kinds of lesions. Long-term hormone replacement therapy was administered more in CR patients (60%) and diabetes insipidus than other lesions. One PA patient and 2 CR patients had recurrence during a follow-up of 12 - 52 months (mean: 34). RCC and AC had no recurrence. CONCLUSION: Craniopharyngioma, Rathke's cleft cyst, cystic pituitary adenoma and intrasellar arachnoid cyst are a spectrum of diseases with different therapeutic strategies. An accurate diagnosis of these lesions is essential so as to determine the type of treatment to improve outcome.

[Preservation of pituitary stalk in the microsurgery of giant pituitary adenoma and its significance].

OBJECTIVE: To investigate and elucidate how to preserve the pituitary stalk in the microsurgery of giant pituitary adenoma (GPA) and its clinical significance. METHODS: 45 GPA patients, 23 males and 22 female; aged 40.8, including 12 cases of invasive pituitary adenoma (IPA) underwent craniotomy based on the respective preoperative neuroradiological imaging characteristics. The anatomical relationship between the pituitary stalk and tumor was recorded. The methods to protect the pituitary stalk were summarized. RESULTS: Total tumor excision was achieved in 25 patients (55.5%), near-total resection was done in 12 (26.7%), and subtotal resection in 8 (17.8%). During the surgical proceeding, the pituitary stalk was distinguished from the tumor and preserved well in all 33 cases with non-invasive giant pituitary adenoma. On the contrary, in the 12 cases of invasive giant pituitary adenoma (IPA) the pituitary stalk was visualized in only 7 cases. In the patients with visualized pituitary stalks 4 pituitary stalks were not identified very well. In most cases (91%) the pituitary stalks were located laterally (on the left or right side) or supero-posterior to the tumor, only a few were located anteriorly. In all 12 IPA patients 2 cases of postoperative hemorrhage occurred associated with remnant tumor and immediate hematoma evacuation was performed, however, one patient died due to hypothalamus injury. CONCLUSION: Pituitary stalk has various anatomical relationships to the entity of GPA; most are located lateral or supero-posterior to the tumor. However, the relationship between the stalk and tumor is not clear in IPA. Identifying and preserving the pituitary stalk well during surgical manipulation will be beneficial to get an excellent outcome.

[Anatomic research of the subtemporal transpetrosalridge approach].

OBJECTIVE: Subtemporal transpetrosal ridge approach (STA) was introduced to remove the petroclival meningiomas with the simple, safe and minimal invasive character. It is suggested to replace the combined approach to reduce the morbidity and mortality. METHODS: Surgical anatomic study was done on 10 adult cadaver heads fixed in 10% formalin and 10 dry skulls. Ten cadaver heads were examined by bone-window CT scan pre and post-operation. The relationships of important anatomic structures and positions were measured and photographed. RESULTS: For the STA, the important structures include Vein of Labbe, petrous bone and brain stem ventral space. The important data include the drilling space of the petrous ridge. CONCLUSIONS: STA is an original approach to remove petroclival meningiomas. By drilling the petrous ridge, it allows the resecion of the tumor simple, safe and minimal invasive. It is suggested to replace the combined approach with STA to diminish the morbidity and mortality.

[The diagnosis and microsurgical treatment of solid cerebellar hemangioreticuloma].

OBJECTIVE: To study the clinical features, neuroimaging presentations, diagnosis and differential diagnosis, and operative warning events of the solid cerebellar hemangioblastoma. METHODS: The clinical data of 48 patients with solid cerebellar haemangioreticuloma were retrospectively analysed. Preoperative examinations included CT and MRI in all patients, DSA in 20, preoperative embolization of feeding arteries of tumor in 16, a preoperative ventriculoperitoneal shunt was placed in 5 cases. The lesion distribution of 48 patients was as follows: 14 lesions were situated in the left hemisphere of cerebellum with an extent into the cerebellopontine angle in 2 cases, 12 in right hemisphere of cerebellum, 8 in superior vermis, 7 in inferior vermis, 6 in left subtentorium, 1 in left cerebellar tonsil. RESULTS: The tumors of 48 patients were totally removed. One patient occurred normal perfusion pressure breakthrough during surgery, and gastrointestinal hemorrhage on the second day after operation. Other patients all were intra- and postoperatively uneventful. Except for autoinfusion, no patients transfused heterogenous blood. There were no operative mortality and serious complications in this series. CONCLUSIONS: The solid cerebellar hemangioblastoma was benign neoplasm. Abiding by properly operative techniques, the optimal results can be obtained.

[Microsurgical treatment of cranial-cervical type giant ependymoma within the fourth ventricle in adult patients].

OBJECTIVE: To study the neuroimaging characteristics, microsurgical treatment and prognosis of cranial-cervical type giant ependymoma within the fourth ventricle in adult patients. METHODS: The clinical data of 22 patients with cranial-cervical type giant ependymoma within the fourth ventricle were retrospectively analyzed. The suboccipital and C(1-3) posterior midline approach were performed in all patients. RESULTS: The tumor of all patients not only occupied the cavity of the fourth ventricle but also protruded into the cervical canal through the foramen of Magendie and extended to the cervical 2 or cervical 3 level. The tumor was totally removed in 20 patients, and subtotally removed in 2. Six patients received radiotherapy postoperatively. The time of mean follow-up of all patients was 5.5 years. One patient underwent a second operation 5 years after the first surgical procedure in other hospital due to tumor recurrence. The 5-year survival rate was 100%. All patients recovered their normal life or work. CONCLUSION: Cranial-cervical type giant ependymoma within the fourth ventricle in adult patients has a good prognosis. The surgery with postoperative adjuvant radiotherapy can obtain a optimal results.