Pineal tumours

Author(s):  
Mueez Waqar ◽  
Samantha Mills ◽  
Conor L Mallucci ◽  
Michael D Jenkinson

Tumours of the pineal are very rare, tend to be more common in children and while there are a wide variety of pathologies the majority are germ cell tumours and pineal parenchymal tumours. These tumours usually present with hydrocephalus and endoscopic third ventriculostomy is the operation of choice. It is important to test for tumour markers in the blood and cerebrospinal fluid, since the diagnosis of a secreting germ cell tumour precludes the need for surgery. Surgical biopsy can be performed by endoscopy or with frame-based stereotaxy. For germ cell tumours chemotherapy and radiotherapy are the mainstay of treatment, but surgery has a role in the management of residual disease. For primary parenchymal tumours, maximum surgical resection is the first-line treatment and can be curative for pineocytoma. Pineoblastoma require adjuvant radiotherapy and for intermediate grade pineal tumours the role of radiotherapy is still being evaluated.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e16550-e16550
Author(s):  
Karen Murphy ◽  
Scheryll Paula Alken ◽  
John McCaffrey ◽  
Desmond Carney ◽  
Miriam O Connor

e16550 Background: Malignant ovarian germ cell tumours(OGCT) are a rare disease, accounting for 2% of all malignant ovarian tumours diagnosed in Ireland between 1994 - 2010. They affect a younger population than epithelial ovarian cancer, with the highest number of cases in the second and third decade. In this report, we present a review of patients treated in a tertiary referral centre. Methods: We performed a retrospective review over a 26 year period in our institution. We examined clinical records, pathology, pharmacy and surgical databases to identify patients with ovarian germ cell tumours who were treated in our institution. Results: During the period 1986 – 2012, twenty patients were treated and followed at this institution for malignant ovarian germ cell tumor (OGCT). Eighteen patients are alive and well at a median follow up of 14.5 years (range 0.5 – 26). Nineteen patients received platinum based chemotherapy with a median number of four cycles (range 2 – 6) required to achieve complete remission. Twenty percent of patients had yolk sac histology; the next commonest were immature teratomas and mixed germ cell tumour (10 & 15% respectively). The commonest chemotherapy regimen used was BEP (bleomyocin, etoposide and cistplatin). Six patients (30%) underwent resection of residual disease post chemo revealing mature teratoma/necrotic tissue. During follow up, four patients had progressive or recurrent disease. Two patients at resection had mature teratomas found on histological examination. The remaining two patients (10%) had progressive disease and died within a short time period. In our patient population, nine of nine attempting pregnancy have been successful. There have been 12 pregnancies carried to term; mother and child in each case are well. At a median follow up of 14.5 years (range 0.5 – 26), there have been no second malignancies and no toxicities attributable to chemotherapy. Conclusions: We have shown that platinum based chemo in OGCT is associated with preservation of fertility in these young women. In our series there have been no adverse affects in women or their infants. Platinum based chemo highly successful in curing OGCT and this confirms that it is associated with preserved fertility in these young women.


2020 ◽  
Vol 7 (2) ◽  
pp. 205-211
Author(s):  
Kaynat Fatima ◽  
Syed Tasleem Raza ◽  
Ale Eba ◽  
Sanchita Srivastava ◽  
Farzana Mahdi

The function of protein kinases is to transfer a γ-phosphate group from ATP to serine, threonine, or tyrosine residues. Many of these kinases are linked to the initiation and development of human cancer. The recent development of small molecule kinase inhibitors for the treatment of different types of cancer in clinical therapy has proven successful. Significantly, after the G-protein-coupled receptors, protein kinases are the second most active category of drug targets. Imatinib mesylate was the first tyrosine kinase inhibitor (TKI), approved for chronic myeloid leukemia (CML) treatment. Imatinib induces appropriate responses in ~60% of patients; with ~20% discontinuing therapy due to sensitivity, and ~20% developing drug resistance. The introduction of newer TKIs such as, nilotinib, dasatinib, bosutinib, and ponatinib has provided patients with multiple options. Such agents are more active, have specific profiles of side effects and are more likely to reach the necessary milestones. First-line treatment decisions must be focused on CML risk, patient preferences and comorbidities. Given the excellent result, half of the patients eventually fail to seek first-line treatment (due to discomfort or resistance), with many of them needing a third or even further therapy lines. In the present review, we will address the role of tyrosine kinase inhibitors in therapy for chronic myeloid leukemia.


2016 ◽  
Author(s):  
Jitendra Pariyar ◽  
Binuma Shrestha

Background: Germ cell malignancies account for about 5% of all ovarian cancers. These tumours grow rapidly and often produce symptoms quicker than the slow growing epithelial tumour. Commonly seen in the first two decades of life germ cell malignancies are highly chemosensitive and are potentially curable with surgery and chemotherapy. This study is the first of its kind regarding the epidemiology, management and outcome of patients with malignant germ cell tumour in Nepal. Objective: To analyze the clinical presentation and management outcomes of malignant germ cell tumours managed in B.P. Koirala Memorial Cancer Hospital, Nepal. Methodology: Descriptive study conducted in B.P. Koirala Memorial Cancer Hospital, Nepal. Case records of malignant germ cell tumours attending the hospital from January 1999 to December 2009 were analyzed regarding their illness history, clinical examination, investigations, treatment, follow-up and outcomes measured. Observations: Total 65 cases of malignant germ cell tumours with age range from 2 to 58 years (mean 21.7 years) were received. 42% cases were Tibeto-Burmese; 30% were Indo-Aryans. There were 15 cases (23%) of dysgeminoma, 21 endodermal sinus tumor (32%), 16 Immature Cystic Teratoma (24.5%), 9 (14%) Mixed Germ Cell, 2 unclassified GCT (3.5%) and 2 malignant transformation in teratoma (3.5%). 33 (49.5%) patients had early stage disease, 37 (57%) underwent fertility preserving surgery. 4 cases (9%) due to disseminated disease, underwent neoadjuvant chemotherapy followed by debulking surgery. 51 cases (78.5%) received adjuvant chemotherapy (BEP or EP regimen). The overall survival was 70%. Conclusion: Early stage germ cell malignancies can be safely managed by fertility preserving surgery followed by, chemotherapy if indicated. For advanced diseases, neoadjuvant chemotherapy followed by surgery can be undertaken with curable intent.


2021 ◽  
Vol 8 (25) ◽  
pp. 2180-2186
Author(s):  
Debarati Pathak ◽  
Abhijit Banerjee ◽  
Soma Ghosh ◽  
Arghya Bandyopadhyay ◽  
Tushar Kanti Das

BACKGROUND Mediastinal masses, an enigma to surgical pathologist are among the most complicated lesions explored and relatively inaccessible. They often connote a process with mass effect presenting with superior mediastinal syndrome. This is a challenging area faced by surgical pathologist as varied lesions are found here and often biopsies obtained are tiny and crushed. Appropriate therapy of various mediastinal tumours differs considerably and may significantly impact survival. We wanted to evaluate the various lesions in different compartments of mediastinum and categorise them according to anatomical location, and histopathology. METHODS Patients with mediastinal masses attending outpatient department were selected, history taken and relevant investigations done with radiological evaluation for proper anatomical location of lesion. Histopathological study done on tissues obtained by ultrasound / CT guided biopsy, open surgical biopsy were categorized according to histologic types. Immunohistochemistry was done wherever applicable. RESULTS A total of 58 cases of mediastinal lesions were studied where males predominated and age of patients ranged from 11 months to 68 yrs. All patients were symptomatic. Shortness of breath, superior vena cava syndrome was dominant in anterior and superior mediastinal lesions, middle and posterior mediastinal masses presented with chest pain. Most lesions were neoplastic. Germ cell tumours were found in (24.14 %) followed by lymphoma in (20.69 %) and thymic lesions in (18.97 %) of patients. Neurogenic tumours found in (13.79 %) were located in posterior mediastinum whereas, germ cell tumours and lymphomas were located in anterior mediastinum. Non neoplastic lesions included tuberculosis, sarcoidosis. Unsuspected lesion was metastatic deposit of adenoid cystic carcinoma. CONCLUSIONS A wide variety of non-neoplastic and neoplastic lesions can be found in different compartments of mediastinum and accurate diagnosis is considered necessary to formulate management strategies. KEYWORDS Mediastinum, Biopsy, Radiology, Histopathology


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