BRMP-03. Pituitary carcinoma: a case of dramatic response to immunotherapy (ipilimumab + nivolumab) after failure with temozolomide

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi223-vi223
Author(s):  
Sanjit Shah ◽  
Saima Manzoor ◽  
Yehudit Rothman ◽  
Matthew Hagen ◽  
Luke Prater ◽  
...  
Keyword(s):  
Nerve Palsy ◽  
Treatment Guidelines ◽  
Single Case ◽  
Pituitary Tumors ◽  
Residual Tumor ◽  
Current Treatment ◽  
Tumor Burden ◽  
Pituitary Carcinoma ◽  
Third Nerve Palsy ◽  
Postoperative Mri

Abstract INTRODUCTION Pituitary carcinoma (PC) accounts for just 0.1% of all pituitary tumors, often recurs following resection, and has a median reported survival of 1 year. Current treatment guidelines are not standardized but combine surgical resection, radiation therapy, and chemotherapy [1]. Temozolomide is the only chemotherapeutic with documented effectiveness, and the only recommended agent for aggressive pituitary carcinomas in ESE clinical guidelines [3]. CASE: A 57-year-old male presented with visual deterioration over a three-month period. Ophthalmologic evaluation revealed bitemporal visual field deficits. MRI brain W/WO demonstrated a sellar mass suspected to be pituitary macroadenoma with displacement of the stalk and optic nerve impingement (Figure 1a). The patient underwent stereotactic endoscopic transsphenoidal resection of the mass [2]. Postoperative MRI demonstrated gross total resection (Figure 1b). Pathology revealed a sparsely granulated corticotroph-adenoma with malignant transformation (early in-situ PC). Immunohistochemistry showed LOE of MLH1 and PMS2 in the tumor cells; Genetic analysis revealed MGMT methylation. Proton therapy was recommended given the elevated Ki67 index (75%) and p53 positivity. Before radiotherapy, there was no evidence of residual tumor or metastasis radiographically. He received 6600cGy of radiation over 33 fractions. Surveillance MRI showed recurrence at 21 months postoperatively, and temozolomide was initiated. However, MRI demonstrated marked progression after 3 cycles, and at 44 months, he developed a new 6th nerve palsy (Figure1c). Next-generation sequencing using the MSK-IMPACT platform identified somatic mutations in MLH1 Y548lfs*9 and TP53 R337C[4]. Immunotherapy with ipilimumab/nivolumab was initiated [5], and the patient noted resolution of his third nerve palsy soon after. MRI demonstrated a dramatic response with only minimal residual tumor burden (Figure1d). CONCLUSION PC is a rare tumor with frequent recurrence and a short median expected length of survival. Here we demonstrate the utility of immunotherapy in a single case report of PC. This treatment helped our patient survive well beyond the expected median life expectancy of this aggressive disease.

scholarly journals SAT-LB51 The Unique Etiology and Management of a Third Cranial Nerve Palsy

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Maria Magar
Keyword(s):  
Cranial Nerve ◽  
Nerve Palsy ◽  
Visual Fields ◽  
Pituitary Tumors ◽  
Pituitary Surgery ◽  
Third Nerve Palsy ◽  
Cranial Nerve Palsies ◽  
Regular Menses ◽  
Third Cranial Nerve Palsy ◽  
One Year

Abstract Background: Cranial nerve palsies (CNP) in association with a hypophyseal process can be uni/bilateral, single, multiple, with or without visual deficits and pain. It is reported 14-30% of pituitary tumors lead to a palsy. Often the acute cause of palsy is apoplexy (1) but other rare etiologies exist such as abscess or prolactinoma (2, 3). Clinical Case: A 40 year old woman with no PMH presented to the ED for acute on chronic headaches. Two days prior she started having blurry vision in her R eye. The headache improved with medications and she was sent home. Two days later she returned with new symptoms of the R eye deviating laterally and down, associated ptosis and a 6 mm nonreactive pupil. A stat MRI brain was obtained for her new CN III palsy, showing a 1.4 cm sellar mass with suprasellar extension. Further history revealed that two months prior her regular menses had stopped. Labs were obtained that included a prolactin 127 (nl 4.8-23.3); fT4 1.2 (nl .93-1.7); IGF1 129 (nl 52-328); 9 am cortisol 16.6 (nl 4-22); FSH 5.5 (nl 3.5-12.5); LH 3.8 (nl 2.4-12.6). Neurosurgery (NSGY) was consulted, recommending a trial of medical therapy since her visual fields were intact. Endocrine then advised cabergoline 0.5 mg twice weekly. In NSGY clinic ten days later, her prolactin was 0.2 but her symptoms remained the same. One month later, she was seen in Neuro-ophthalmology clinic where it was noted that her diplopia had subjectively resolved. Her eye exam was documented as normal except for sluggish pupils. Conclusion: CNP are more common in larger pituitary tumors and often associated with panhypopituitarism and necrosis. About 90% of cases resolve by one year after pituitary surgery. Although our patient had a tumor that was slightly larger than 1 cm, she did not need hormonal replacement, did not have hemorrhage/necrosis, and her symptoms resolved with a dopamine agonist. There is one other case in the literature of medically treated macroprolactinoma with resolution of CN III palsy (3). References: (1) Hage R, et al. Third, Fourth, and Sixth Cranial Nerve Palsies in Pituitary Apoplexy. World Neurosurgery. 2016; 94:447-452.(2) Wang L, et al. Pituitary abscess resulting in isolated abducens palsy. Journal of the Formosan Medical Association. 2016;115:134-135.(3) Harries A, et al. Resolution of third nerve palsy following treatment of prolactinoma with cabergoline. Acta Neurochir. 2010; 152:1741-1743.

scholarly journals Cavernous Sinus Epidermoid Cyst Removal through a No-Keyhole Pterional Craniotomy: Operative Video and Technical Nuances

2021 ◽  
Author(s):  
Carlos Candanedo ◽  
Samuel Moscovici ◽  
Sergey Spektor
Keyword(s):  
Cavernous Sinus ◽  
Epidermoid Cyst ◽  
Nerve Palsy ◽  
Lateral Wall ◽  
Residual Tumor ◽  
Benign Tumors ◽  
Third Nerve Palsy ◽  
Pterional Craniotomy ◽  
Epidermoid Cysts ◽  
Third Nerve

AbstractIntracranial epidermoid cysts are considered benign tumors with good general prognosis. However, their radical removal may be associated with certain morbidity, especially when the capsule is attached to neurovascular structures. Epidermoid cysts located in the cavernous sinus are very rare. We present an operative video of a 22-year-old female patient, who suffered a right-sided headache for 5 years. The video demonstrates main steps and surgical nuances of resection of a right interdural cavernous sinus epidermoid cyst, measuring 22 × 19 × 21 mm (4.3 cc) (Fig. 1A). On initial physical examination, the patient had a right partial third nerve palsy (mild ptosis with minimal diplopia), without any other cranial nerve deficit. A right no-keyhole pterional craniotomy was performed, followed by extradural anterior clinoidectomy and peeling of the outer dural layer of the lateral wall of the cavernous sinus. The dura matter was also detached from the distal carotid dural ring, which was exposed by the clinoidectomy (Fig. 2A). This maneuver provided excellent exposure of the interdural epidermoid cyst, which severely compressed the oculomotor nerve against the posterior petroclinoid dural fold (Fig. 2B). Gross total resection of the epidermoid cyst was achieved (Fig. 1B and C). The patient developed a transient worsening of the third nerve palsy, which recovered completely 3 months after the surgery. Postoperative magnetic resonance imaging revealed no signs of residual tumor.The link to the video can be found at: https://youtu.be/pobhYb5ZNig.

scholarly journals SUN-923 Combination Immune Checkpoint Inhibitor Therapy for ACTH-Secreting Pituitary Carcinoma

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Jeroen M K de Filette ◽  
Bastiaan Sol ◽  
Gil Awada ◽  
Sandrine Aspeslagh ◽  
Corina E Andreescu ◽  
...  
Keyword(s):  
Immune Checkpoint ◽  
Nerve Palsy ◽  
Elevated Serum ◽  
Sella Turcica ◽  
Pituitary Tumors ◽  
Pituitary Carcinoma ◽  
Oculomotor Nerve Palsy ◽  
First Case ◽  
Cortisol Levels ◽  
Acth Secreting

Abstract Introduction Pituitary carcinoma is a rare yet serious entity with poor prognosis despite multimodal therapies. Cerebrospinal and/or systemic metastases are present by definition, making adjuvant systemic therapy necessary. Novel treatments are urgently needed for refractory cases. Immunotherapy with immune checkpoint inhibitors (ICI) targeting cytotoxic T-lymphocyte antigen-4 (CTLA-4), programmed cell death 1 (PD-1) or its ligand (PD-L1) has been a revolution in multiple malignancies. The expression of CTLA-4 and PD-L1 has been elucidated in pituitary adenomas and could be implicated in pituitary carcinomas as well. Hypophysitis is also a frequent endocrine immune-related adverse event, especially during CTLA-4 blockade (with ipilimumab) or combination ICI. However, the efficacy of ICI in the treatment of refractory pituitary tumors has yet to be established. In 2018, Lin et al. successfully treated a first case of a hypermutated aggressive ACTH-secreting pituitary carcinoma with ipilimumab (anti-CTLA-4) and nivolumab (anti-PD-1) combination immunotherapy. Clinical Case We report a 40-year old male, diagnosed with an invasive ACTH-secreting pituitary macroadenoma in 2012, initially treated by transsphenoidal and transcranial surgery, followed by adjuvant stereotactic radiotherapy and several courses of ketoconazole. In 2017, he presented to our clinic for a recurrent Cushing’s phenotype despite maximal dosing of ketoconazole. Therapy both with pasireotide and cabergoline was unable to normalize cortisol levels and a bilateral (subtotal) adrenalectomy was performed. In June 2018, he presented to our emergency department with acute diplopia due to a left abducens nerve palsy. Imaging revealed recurrent invasion of the tumor into the sella turcica and cavernous sinus, together with cerebellar and drop metastases at the cervical spine. Temozolomide (TMZ) was initiated for a total of 9 cycles. Progressive disease was observed with development of new onset right oculomotor nerve palsy after the last TMZ cycle, and persistence of elevated serum ACTH-cortisol and urinary cortisol levels, despite the absence of radiological progression. Therefore, he was started in a compassionate use setting with a combination ICI therapy with ipilimumab 3 mg/kg and nivolumab 1 mg/kg (for 4 cycles), followed by maintenance nivolumab therapy (240 mg) every two weeks. He has stable disease (both radiographically and hormonally) five months after the initiation of the immunotherapy. Clinical Lesson(s) or Conclusion(s) We report the second case of ACTH-secreting pituitary carcinoma treated with combination ICI therapy. The disease status of the patient is stable up until now, suggesting at least disease control by the immunotherapy. Checkpoint blockade inhibitors are a promising novel treatment modality for refractory pituitary tumors and should be further studied.

Left suboccipital supracerebellar transtentorial approach for resection of tectal cavernous malformation

2019 ◽  
Vol 1 (2) ◽  
pp. V19
Author(s):  
Hussam Abou-Al-Shaar ◽  
Timothy G. White ◽  
Ivo Peto ◽  
Amir R. Dehdashti
Keyword(s):  
Nerve Palsy ◽  
Oculomotor Nerve ◽  
Oculomotor Nerve Palsy ◽  
Entry Zone ◽  
Third Nerve Palsy ◽  
History Of ◽  
Tectal Plate ◽  
The Right ◽  
Transtentorial Approach ◽  
Third Nerve

A 64-year-old man with a midbrain cavernoma and prior bleeding presented with a 1-week history of diplopia, partial left oculomotor nerve palsy, and worsening dysmetria and right-sided weakness. MRI revealed a hemorrhagic left tectal plate and midbrain cavernoma. A left suboccipital supracerebellar transtentorial approach in the sitting position was performed for resection of his lesion utilizing the lateral mesencephalic sulcus safe entry zone. Postoperatively, he developed a partial right oculomotor nerve palsy; imaging depicted complete resection of the cavernoma. He recovered from the right third nerve palsy, weakness, and dysmetria, with significant improvement of his partial left third nerve palsy.The video can be found here: https://youtu.be/ofj8zFWNUGU.

Microsurgical resection of foramen magnum meningioma through a transcondylar approach: three-dimensional operative video

2019 ◽  
Vol 1 (2) ◽  
pp. V3
Author(s):  
Guilherme H. W. Ceccato ◽  
Rodolfo F. M. da Rocha ◽  
Duarte N. C. Cândido ◽  
Wladimir O. Melo ◽  
Marcio S. Rassi ◽  
...  
Keyword(s):  
Nerve Palsy ◽  
Three Dimensional ◽  
Foramen Magnum ◽  
Complete Resection ◽  
Neurophysiological Monitoring ◽  
Cervical Instability ◽  
Transcondylar Approach ◽  
Postoperative Mri ◽  
Microsurgical Resection

Foramen magnum (FM) meningiomas are challenging lesions. We present the case of a 38-year-old female with neck pain, dysphonia, and slight twelfth nerve palsy. Imaging workup was highly suggestive of an FM meningioma, and microsurgical resection with the aid of intraoperative neurophysiological monitoring was indicated. A transcondylar approach was employed, the vertebral artery was mobilized, and the tumor was completely removed. Postoperative MRI demonstrated complete resection. There were no signs of cervical instability. The patient presented with improvement of her symptoms and no new neurological deficit on follow-up. FM meningiomas can be successfully resected using a transcondylar approach, since it increases the exposure of the ventral FM, allowing the surgeon to work parallel to the skull base and flush with the tumor’s attachment. Informed consent was obtained from the patient for publication of this operative video.The video can be found here: https://youtu.be/itfUOB-6zM0.

scholarly journals Incidental Papillary Thyroid Cancer Identified During Parathyroidectomy

2020 ◽  
pp. 014556132098269
Author(s):  
Megan L. Crenshaw ◽  
Dana Goldenberg ◽  
Darrin V. Bann
Keyword(s):  
Papillary Thyroid Carcinoma ◽  
Thyroid Carcinoma ◽  
Treatment Guidelines ◽  
Surgical Excision ◽  
Current Treatment ◽  
Papillary Thyroid ◽  
Disease Extent ◽  
Lack Of Information ◽  
Thyroid Lobectomy ◽  
Short And Long Term

Current treatment guidelines recommend surgical excision of papillary thyroid carcinoma. However, the precise surgical treatment, including thyroid lobectomy, total thyroidectomy, and the need for neck dissection, is dictated by disease extent and tumor cytology. Incidental papillary thyroid carcinoma discovered during another surgery therefore presents a surgical conundrum due to lack of information. Surgeons must consider short- and long-term surgical morbidities, as well as individual patient factors, when deciding how to treat an unexpected thyroid carcinoma.

scholarly journals Pituitary Apoplexy Causing Compression of Third Cranial Nerve—Management

2019 ◽  
Vol 08 (02) ◽  
pp. 119-122
Author(s):  
Václav Masopust
Keyword(s):  
Cavernous Sinus ◽  
Nerve Palsy ◽  
Clinical Symptoms ◽  
Rapid Development ◽  
Sella Turcica ◽  
Oculomotor Nerve ◽  
Oculomotor Nerve Palsy ◽  
Future Research ◽  
Third Nerve Palsy ◽  
Increased Pressure

AbstractLesions of the oculomotor nerve as the first sign of pituitary adenoma are rare. The cause of such lesions without other clinical symptoms is discussed in this study. A small cohort of 4 patients (3.1%) with oculomotor nerve palsy (third nerve palsy) as the only neurologic deficit, from 129 patients who got operated upon for pituitary adenomas, is presented. In this group (mean age: 55 years, range: 36–65 years), all patients (two women and two men) underwent surgery. In two cases, there was arrested pneumatization and thickened bone. In the remaining two cases, a macroscopically visible, very solid opaque diaphragm was present, after the removal of the tumor and thickened bone. Complete adjustment was observed in all patients within 1 week after the surgery. Two factors that seem to increase the high risk for the development of oculomotor nerve palsy are that the cavernous sinus may be the only weak structure surrounding the sella turcica when the diaphragm and bone are thickened; and the rapid development of increased pressure in this region. The increased pressure on the cavernous sinus during the anatomical variations is the primary cause for lesions on the oculomotor nerve. However, this conjecture cannot be statistically demonstrated because of the small number of cases. Future research should be conducted on larger samples to increase statistical inference and generalizability.

Aneurysm-induced third nerve palsy

1972 ◽  
Vol 36 (5) ◽  
pp. 548-551 ◽  
Author(s):  
Iftikhar A. Raja
Keyword(s):  
Subarachnoid Hemorrhage ◽  
Functional Recovery ◽  
Poor Prognosis ◽  
Nerve Palsy ◽  
Nerve Function ◽  
Content Type ◽  
Third Nerve Palsy ◽  
Carotid Ligation ◽  
Third Nerve ◽  
Fine Print

✓ Forty-two patients with aneurysm-induced third nerve palsy are described. After carotid ligation, 58% showed satisfactory and 42% unsatisfactory functional recovery. In some patients the deficit continued to increase even after carotid ligation. Early ligation provided a better chance of recovery of third nerve function. Patients in whom third nerve palsy began after subarachnoid hemorrhage had a poor prognosis. No relationship was noted between the size of the aneurysm and the recovery of third nerve function.

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