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Saksham Gupta, Wenya Linda Bi, Alexandra Giantini Larsen, Sally Al-Abdulmohsen, Malak Abedalthagafi, and Ian F. Dunn

OBJECTIVE

Craniopharyngiomas are among the most challenging of intracranial tumors to manage because of their pattern of growth, associated morbidities, and high recurrence rate. Complete resection on initial encounter can be curative, but it may be impeded by the risks posed by the involved neurovascular structures. Recurrent craniopharyngiomas, in turn, are frequently refractory to additional surgery and adjuvant radiation or chemotherapy.

METHODS

The authors conducted a review of primary literature.

RESULTS

Recent advances in the understanding of craniopharyngioma biology have illuminated potential oncogenic targets for pharmacotherapy. Specifically, distinct molecular profiles define two histological subtypes of craniopharyngioma: adamantinomatous and papillary. The discovery of overactive B-Raf signaling in the adult papillary subtype has led to reports of targeted inhibitors, with a growing acceptance for refractory cases. An expanding knowledge of the biological underpinnings of craniopharyngioma will continue to drive development of targeted therapies and immunotherapies that are personalized to the molecular signature of each individual tumor.

CONCLUSIONS

The rapid translation of genomic findings to medical therapies for recurrent craniopharyngiomas serves as a roadmap for other challenging neurooncological diseases.

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Jacob L. Goldberg, Ibrahim Hussain, Joseph A. Carnevale, Alexandra Giantini-Larsen, Ori Barzilai, and Mark H. Bilsky

OBJECTIVE

Paraspinal ganglioneuromas are rare tumors that arise from neural crest tissue and can cause morbidity via compression of adjacent organs and neurovascular structures. The authors investigated a case series of these tumors treated at their institution to determine clinical outcomes following resection.

METHODS

A retrospective review of a prospectively collected cohort of consecutive, pathology-confirmed, surgically treated paraspinal ganglioneuromas from 2001 to 2019 was performed at a tertiary cancer center.

RESULTS

Fifteen cases of paraspinal ganglioneuroma were identified: 47% were female and the median age at the time of surgery was 30 years (range 10–67 years). Resected tumors included 9 thoracic, 1 lumbar, and 5 sacral, with an average maximum tumor dimension of 6.8 cm (range 1–13.5 cm). Two patients had treated neuroblastomas that matured into ganglioneuromas. One patient had a secretory tumor causing systemic symptoms. Surgical approaches were anterior (n = 11), posterior (n = 2), or combined (n = 2). Seven (47%) and 5 (33%) patients underwent gross-total resection (GTR) or subtotal resection with minimal residual tumor, respectively. The complication rate was 20%, with no permanent neurological deficits or deaths. No patient had evidence of tumor recurrence or progression after a median follow-up of 68 months.

CONCLUSIONS

Surgical approaches and extent of resection for paraspinal ganglioneuromas must be heavily weighed against the advantages of aggressive debulking and decompression given the complication risk of these procedures. GTR can be curative, but even patients without complete tumor removal can show evidence of excellent long-term local control and clinical outcomes.

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Alexandra M. Giantini Larsen, David J. Cote, Hasan A. Zaidi, Wenya Linda Bi, Paul J. Schmitt, J. Bryan Iorgulescu, Michael B. Miller, Timothy R. Smith, M. Beatriz Lopes, John A. Jane Jr., and Edward R. Laws Jr.

OBJECTIVE

The authors report the diagnosis, management, and outcomes of 6 cases of spindle cell oncocytoma (SCO) in an effort to guide clinical diagnosis and management of these uncommon lesions.

METHODS

This study is a retrospective review of cases involving adult patients who underwent resection of pituitary lesions at the authors’ institutions between January 2000 and October 2017. The authors identified patients with histopathological confirmation of SCO and collected clinical data, including preoperative, perioperative, and postoperative management, complications, and outcomes.

RESULTS

Six patients with SCO were identified. Clinical findings at initial presentation included visual disturbances, dizziness, and headache. All patients underwent resection. Four resections were initially performed by the transsphenoidal approach, and 2 resections were performed by craniotomy at an outside institution with subsequent transsphenoidal reoperations. Neither necrosis nor increased mitotic activity was seen in the tumor samples. All samples stained positive for S100 protein and thyroid transcription factor 1 and negative for glial fibrillary acidic protein and pituitary hormones. Five of the samples stained positive for epithelial membrane antigen. The average MIB-1 index was 8.3% (range 2–17). Postoperatively, 3 of the 6 patients received further treatment for progression of residual tumor or for recurrence, 2 have stable residual tumor, and 1 has had no recurrence after gross-total resection. Two patients developed postoperative complications of transient sixth cranial nerve palsy and diplopia. There were no other complications.

CONCLUSIONS

SCO poses both a diagnostic and therapeutic challenge. These tumors are often initially misdiagnosed as nonfunctional pituitary adenomas because of their sellar location and nonspecific symptomatology. Postoperatively, SCO must also be distinguished from other neoplasms of the posterior pituitary gland through histopathological examination. Resection of SCO can be challenging, given its highly vascular and adherent nature. Long-term follow-up is critical, as the tumor is associated with higher recurrence and progression rates compared to other benign neoplasms of the sella.

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Constantin Tuleasca, Michaela Dedeciusova, Laura Negretti, Roy Thomas Daniel, and Marc Levivier