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Stephen A. Hill, James M. Falko, Charles B. Wilson and William E. Hunt

mice results in “chromophobe” tumors (positive to periodic acid-Schiff (PAS) and aldehyde fuchsin stains), which produce and actively secrete TSH. 13, 14 As many as 23% of post-radiothyroidectomy patients display elevated TSH levels, despite biochemical euthyroidism. 4 Speculation that excessive TRH release provokes high levels of TSH and resultant hyperthyroidism exists, 10, 25, 32 but is unproven. Finally, hyperthyroidism caused by ectopic production of TSH by non-sellar neoplasms, mostly choriocarcinomas or molar pregnancies, is also known. 7, 19, 23 In 1980

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Transsphenoidal surgery following unsuccessful prior therapy

An assessment of benefits and risks in 158 patients

Edward R. Laws Jr., Nicolee C. Fode and Michael J. Redmond

(either open or stereotaxic), with some cases having adjunctive radiotherapy to the pituitary region. Prior therapy was considered “indirect” when it consisted of radiotherapy alone, adrenalectomy, or medical therapy with bromocriptine or adrenal toxins. Results The rationale for secondary transsphenoidal surgery was separable into several major categories ( Table 3 ). The most important category was that of patients with persistent or recurrent sellar neoplasms presenting with mass effect, usually progressive visual loss. The second largest group included

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David N. Louis, E. P. Richardson Jr., G. Richard Dickersin, Debra A. Petrucci, Andrew E. Rosenberg and Robert G. Ojemann

skull, and formed an encapsulated mass in the scalp. The authors suggested that the tumor arose from the smooth muscle in the dural vasculature. Only one primary cerebral leiomyosarcoma has, to our knowledge, been described previously. 2 The tumor was a sellar neoplasm in a 35-year-old man, and ultra-structural studies confirmed the smooth-muscle nature of the tumor. After subtotal resection of the tumor and postoperative radiation therapy, the patient was alive 2 years and 8 months after surgery. As far as we can determine, the present case represents the second

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Gilberto K. K. Leung, Maria-Beatriz S. Lopes, Michael O. Thorner, Mary Lee Vance and Edward R. Laws Jr.

postpartum women with characteristic imaging findings who also have panhypopituitarism 43 and patients with lymphocytic infundibuloneurohypophysitis, which classically presents with diabetes insipidus alone. 26 It can be difficult, however, to distinguish with a high degree of certainty between hypophysitis and other sellar neoplasms. 15, 22, 29 Hypophysitis may coexist with pituitary adenomas and cases of germinoma presenting as primary hypophysitis have been reported. 56 A definitive histological diagnosis may obviate the unnecessary use of high-dose steroid therapy

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Jonathan A. Forbes

that time demonstrated early evidence of bridging bone posteriorly with durable correction of deformity ( Fig. 5F ). Case 5 A 63-year-old professor from Kabul presented with a 1-year history of progressive headaches and visual deterioration. On examination, dense bitemporal hemianopsia was present. CT of the head with contrast demonstrated a large, enhancing sellar neoplasm resulting in severe impingement of the optic apparatus and retrochiasmal extension with mild associated hydrocephalus ( Fig. 6A and B ). A small amount of tumor extension posterior to the dorsum

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Jennifer L. Quon, Lily H. Kim, Peter H. Hwang, Zara M. Patel, Gerald A. Grant, Samuel H. Cheshier and Michael S. B. Edwards

OBJECTIVE

Transnasal endoscopic transsphenoidal approaches constitute an essential technique for the resection of skull base tumors in adults. However, in the pediatric population, sellar and suprasellar lesions have historically been treated by craniotomy. Transnasal endoscopic approaches are less invasive and thus may be preferable to craniotomy, especially in children. In this case series, the authors present their institutional experience with transnasal endoscopic transsphenoidal approaches for pediatric skull base tumors.

METHODS

The authors retrospectively reviewed pediatric patients (age ≤ 18 years) who had undergone transnasal endoscopic transsphenoidal approaches for either biopsy or resection of sellar or suprasellar lesions between 2007 and 2016. All operations were performed jointly by a team of pediatric neurosurgeons and skull base otolaryngologists, except for 8 cases performed by one neurosurgeon.

RESULTS

The series included 42 patients between 4 and 18 years old (average 12.5 years) who underwent 51 operations. Headache (45%), visual symptoms (69%), and symptoms related to hormonal abnormalities (71%) were the predominant presenting symptoms. Improvement in preoperative symptoms was seen in 92% of cases. Most patients had craniopharyngiomas (n = 16), followed by pituitary adenomas (n = 12), Rathke cleft cysts (n = 4), germinomas (n = 4), chordomas (n = 2), and other lesion subtypes (n = 4). Lesions ranged from 0.3 to 6.2 cm (median 2.5 cm) in their greatest dimension. Gross-total resection was primarily performed (63% of cases), with 5 subsequent recurrences. Nasoseptal flaps were used in 47% of cases, fat grafts in 37%, and lumbar drains in 47%. CSF space was entered intraoperatively in 15 cases, and postoperative CSF was observed only in lesions with suprasellar extension. There were 8 cases of new hormonal deficits and 3 cases of new cranial nerve deficits. Length of hospital stay ranged from 1 to 61 days (median 5 days). Patients were clinically followed up for a median of 46 months (range 1–120 months), accompanied by a median radiological follow-up period of 45 months (range 3.8–120 months). Most patients (76%) were offered adjuvant therapy.

CONCLUSIONS

In this single-institution report of the transnasal endoscopic transsphenoidal approach, the authors demonstrated that this technique is generally safe and effective for different types of pediatric skull base lesions. Favorable effects of surgery were sustained during a follow-up period of 4 years. Further refinement in technology will allow for more widespread use in the pediatric population.