Shih-Ming Jung

Immunohistochemical expression of OCT4 in primary central nervous system germ cell tumours.

OCT4 is a POU-domain transcription factor that is expressed in embryonic stem cells and germ cells. OCT4 has been detected in specific types of testicular germ cell tumour (GCT), including seminoma and embryonal carcinoma. The aim of this study was to evaluate the role of OCT4 expression in the diagnosis of primary central nervous system (CNS) pure and mixed GCT. Seventeen formalin-fixed, paraffin-embedded tissues of primary CNS GCT were immunohistochemically studied. The 12 pure GCT samples comprised germinoma (5), yolk sac tumour (3), mature teratoma (2), and immature teratoma (2). The five cases of mixed GCT contained various components as follows: yolk sac tumour (4), embryonal carcinoma (3), mature teratoma (1), germinoma (2), polyembryoma (1) and immature teratoma (1). Diffuse and strong nuclear staining indicating OCT4 expression was detected in all cases of pure germinoma (5), and in all cases of mixed GCT containing embryonal carcinoma (3) and/or germinoma (2). There was no corresponding staining in pure GCT of yolk sac tumour, mature teratoma, or immature teratoma except in a primitive neuroectodermal component, or in mixed GCT containing components of yolk sac tumour, mature teratoma or immature teratoma. In conclusion, we found that OCT4 immunostaining is a useful diagnostic tool to assist in the identification of primary CNS embryonal carcinoma and germinoma. In CNS mixed GCT, OCT4 expression can be detected provided that the components include embryonal carcinoma and/or germinoma.

Intrasellar cavernous hemangioma

Reports of intrasellar cavernous hemangioma are rare. They are usually incidental findings at autopsy, or initially mistaken for pituitary adenoma and treated accordingly. There are no specific symptoms. Cranial nerve palsy has occasionally been reported in patients with cavernous hemangioma, with or without sellar extension. However, intrasellar cavernous hemangioma with extension into the cavernous sinus resulting in oculomotor palsy has not been reported. We present a rare case of intrasellar cavernous hemangioma and discuss the diagnosis and management. Total surgical removal is recommended. However, attempts to resect the parasellar component of the lesion may be associated with high morbidity. Therefore, surgical cranial nerve decompression in the acute stage followed by stereotactic radiosurgery for the residual lesion may be an alternative.

A giant retroclival Rathke’s cleft cyst

Rathkes cleft cyst (RCC) is a rare sellar tumor that can extend to the retroclival region, resulting in brain stem compression. We report a male with short stature who experienced repeated syncope due to compression of the brain stem by a giant retroclival RCC. MRI revealed a large homogeneous sellar tumor with anterior displacement of the pituitary gland that extended to the left retroclival space. The tumor differed from ordinary RCCs because of its unusual clinical presentation, the direction of extension, and its imaging characteristics. The retromastoid surgical approach was used for cyst marsupialization. Intraoperative appearance and histological examination confirmed the diagnosis. A follow-up MRI, 2years after resection of the cyst, showed no recurrence.

Sellar lymphoma mimicking sphenoid infection presenting with cavernous sinus syndrome

Lymphomas can occur throughout the body, but are relatively rare in the sphenoid region. The clinical presentations are atypical, ranging from nasal symptoms, pituitary hormone dysfunction and neurological impairment. We report a 63-year-old female patient who suffered nasal discharge, retro-ocular pain and left lateral gaze palsy for 3 months. Cranial MRI revealed sellar and suprasellar lesions involving the clivus, cavernous sinus and sphenoid sinus. The patient was treated for chronic sphenoid sinusitis. Histopathological review for possible misdiagnosis was arranged owing to treatment failure 1 month later. The final diagnosis was primary large B-cell lymphoma, which was confirmed by immunohistochemical study and systemic survey. The symptoms of sellar lymphoma resemble those of chronic sphenoid sinusitis. MRI and hematoxylin and eosin histological examination can accurately diagnose most cases. Immunohistochemistry assay is recommended in equivocal cases and provides a more detailed tumor classification.

Expression of thyroid transcription factor-1 in brain metastases : A useful indicator of pulmonary origin

Determining the primary site of a brain metastasis can be difficult, particularly if there is no organ-specific tumor histology. Thyroid transcription factor-1 (TTF-1) is a marker commonly expressed in lung and thyroid carcinomas. We studied the value of TTF-1 staining to identify brain metastases from a lung primary. Forty-three consecutive patients metastatic carcinomas of the brain were reviewed from a 4 year period. Twenty-one (75%) of the 28 metastatic pulmonary carcinomas were TTF-1 positive (+), whereas only one metastatic papillary thyroid carcinoma (7%) out of 15 metastatic non-pulmonary carcinomas was TTF-1 (+). We also studied the cytokeratin profile of these tumors. However, cytokeratin profile, either alone or combined with TTF-1 staining, was not better than TTF-1 staining alone in identifying brain metastases of pulmonary origin. Overall, TTF-1 positivity is a useful indicator of pulmonary origin in brain metastases, with a very high positive predictive value (95%) and a moderate negative predictive value (67%).

Correlations between clinical hormone change and pathological features of pituicytoma

Abstract Purpose: Pituicytoma is a rare low-grade glioma arising from the pituicytes of the posterior pituitary. To date, the clinical and pathological correlates of pituicytoma have not been investigated. This study was thus designed to examine the correlation between pituicytoma and the normal pituitary gland. Methods: The records of patients who underwent pituitary surgery at Chang Gung Memorial Hospital in Linkou, Taiwan between 2000 and 2016 were reviewed. Patients who received a pathological diagnosis of pituicytoma were included; however, those with inadequate specimens for pathological study were excluded. Clinical information, including patients’ presenting symptoms, serum hormone levels, neuroimages, and specimens, were collected. Hematoxylin and eosin stains and immunohistochemical (IHC) stains were performed for differential diagnosis. Results: Among the 1532 patients who underwent pituitary surgery, nine (0.59%) received a pathological diagnosis of pituicytoma. Two patients were excluded due to inadequate specimens. Among the seven remaining patients, six presented with hormone changes. The IHC stains revealed that pituicytoma has no secretory function; however, the resected pituitary glands showed positive results for hormone change. Coexisting pituicytoma and adrenocorticotropic hormone adenoma were identified in one patient with a diagnosis of Cushing disease. Conclusions: Pituicytoma revealed a negative endocrine secretory function through IHC staining. Additionally, pituicytoma is associated with hypersecretion of the pituitary gland both clinically and pathologically. Diagnosing pituicytoma before pathological confirmation is difficult because the tumour may present with hormone dysfunction. Therefore, IHC staining of specimens is useful to exclude the possibility of coexisting pituicytoma and pituitary adenoma.

Adult supratentorial extra-pineal primitive neuro-ectodermal tumors.

Primitive neuro-ectodermal tumors (PNET) are rare malignant brain tumors, mostly undifferentiated, that tend to spread through the cerebrospinal fluid (CSF). Extra-pineal supratentorial PNET in adults are very rare. Published guidelines for adult PNET were not available until 2011, and at our institute surgeons and oncologists did not have consensus on imaging evaluation or treatment protocols between 1994 to 2008. Twenty-two consecutive adult patients with extra-pineal supratentorial PNET from this period were reviewed in this retrospective study. Their clinical profiles and radiologic images were evaluated. A pathological review based on the 2007 World Health Organization criteria was also conducted. Prognostic factors were analyzed. The 1 and 3 year overall survival rates were 64% and 32% for adult extra-pineal supratentorial PNET, respectively. Limited by the small number of tumors in this series, we suggest that negative prognostic factors are multiplicity at onset, initial CSF seeding, and tumor differentiation. Although age of onset, extent of resection, radiation and chemotherapy were assumed to be good prognostic factors, the analysis did not reveal strong significance for overall survival with univariate and multivariate analysis. We believe more detailed investigations on the genetic/molecular basis of supratentorial PNET and their clinical outcomes are warranted.

Female sex, good performance status, and bevacizumab‐induced hypertension associated with survival benefit in Asian patients with recurrent glioblastoma treated with bevacizumab

The goals of this study were to assess the activity and safety profile of bevacizumab in Taiwan Chinese patients with recurrent glioblastoma, to determine whether their response differed from that reported in other clinical trials, and to examine potential prognostic factors for survival.

Neurenteric Cyst or Neuroendodermal Cyst? Immunohistochemical Study and Pathogenesis

BACKGROUNDnNeurenteric cysts are rare central nervous system lesions derived from an endodermal origin. There is no consensus concerning pathogenesis because of the paucity of occurrences. We report an immunohistochemical study of 10 cases with neurenteric cysts and postulate its pathogenesis.nnnMETHODSnTen patients underwent surgical treatment for neurenteric cysts from 1995 to 2015. We retrospectively reviewed clinical, radiologic, operative, and pathologic findings for these patients. Immunohistochemical stains were completed in all cases to distinguish cell type and origin.nnnRESULTSnThree cell types were identified: pseudostratified-ciliated, goblet-columnar, and simple cuboidal cells. All cases were positive for cytokeratin 7, and negative for cytokeratin 20, caudal-type homeobox 2, mucin 2, thyroid transcription factor 1, human chorionic gonadotropin, placental alkaline phosphatase, and cluster of differentiation 31. Four of them had positive staining for mucin 5AC, with expression only in goblet-columnar cells. According to the immunohistochemical results, the cells resembled the respiratory tract (pseudostratified-ciliated), stomach (goblet-columnar), and respiratory bronchioles (simple cuboidal). Seventy-five percent of cases with recurrence had a goblet-columnar component, emphasizing the importance of total resection of the cyst and complete pathologic examination.nnnCONCLUSIONSnWe postulate that the cystic tumor was derived from multipotent endodermal cells that migrated and traveled along the neuroectoderm, with incomplete differentiation into various cell types as a result of an unsuitable microenvironment. Because the neurenteric canal was only the channel of migration rather than a component of the cysts, the term neuroendodermal cysts is more precise in presenting the embryopathogenesis.

Intradural intraneural hemorrhagic cyst resulting in progressive cauda equina syndrome after anticoagulation therapy.

Study Design. Case report. Objective. To report a case of lumbar intraneural hemorrhagic cyst after anticoagulation therapy that caused progressive radiculopathy and cauda equina syndrome. The possible pathogenic mechanism, associated diseases, and treatment options are discussed. Summary of Background Data. Various pathological processes can cause progressive cauda equina syndrome. However, there have been no reports of progressive cauda equina syndrome due to compression from an intraneural hemorrhagic cyst after anticoagulation therapy. Methods. A case of lumbar intradural intraneural hemorrhagic cyst with progressive cauda equina syndrome after anticoagulation therapy is presented. Results. A 42-year-old-female patient complained at presentation of progressive bilateral lower extremity radiating pain, numbness, and urinary difficulty during the previous 2 months. Lumbar magnetic resonance imaging revealed an L1 cystic lesion with marked mass effect on the surrounding nerve roots. Complete drainage and excision of the lesion was performed, which resulted in excellent postoperative symptoms relief. Pathological examination revealed no definite neoplastic process except some nerve fibers with hemosiderin stain along the cyst wall. On the basis of a combination of intraoperative findings and pathology, an intradural intraneural hemorrhagic cyst that developed after systemic anticoagulation therapy was diagnosed. Conclusion. This is the first report of an intradural intraneural hemorrhagic cyst causing progressive cauda equina syndrome due to anticoagulation therapy. Surgical excision of the cyst is the definite treatment of choice. Level of Evidence: N/A