Craniopharyngioma

Various methods for incorporating radioactive rhenium into suitable particles have been introduced. The earliest example is the use of 186Re-labeled sulfur colloid for radiation synovectomy. Commercial preparations for this application have been available for many years (CIS, France, 1987 catalog). 186Re-sulfur colloid has been used for endocavi-tary irradiation of cystic craniopharyngiomas, where irradiation of surrounding tissue is a risk that must be avoided [155]. 

Figure 18 Comparison of photon and proton dose distributions for the treatment of a craniopharyngioma in a child. Typical planning sections for a large suprasellar craniopharyngioma, in a 3-year-old child, treated with photons (A) or protons (B). The planning target volume (PTV) is indicated by the hatched area. For photons and protons, four equally weighted beams are used and the normalization point was chosen at the intersection of the beam axes. For the four proton beams, the Bragg peak was spread over 4 cm. (From Ref. 41.)...

The recurrence rate of intracranial tumors has been addressed in a number of large observational studies. Reports from the NCGS database (which includes 1262 children with brain tumors) and from England have shown no increase in intracranial tumor recurrence in patients treated with growth hormone (97,98). For patients with craniopharyngioma, postoperative irradiation reduced the recurrence rate, but growth hormone therapy did not increase the risk (99). 

Japanese patients with growth hormone deficiency who had taken part in a placebo-controlled study (n = 64) were entered into an open 48-week study of growth hormone one had a recurrence of craniopharyngioma and required withdrawal from the study (101). Whether growth hormone was contributory is not known. 

Price DA, Wilton P, Jonsson P, Albertsson-Wikland K, Chatelain P, Cutfield W, Ranke MB. Efficacy and safety of growth hormone treatment in children with prior craniopharyngioma an analysis of the Pharmacia and Upjohn International Growth Database (KIGS) from 1988 to 1996. Horm Res 1998 49(2) 91-7. 

Einhaus SI, Stanford RA (1999) Craniopharyngioma. In AL Albright, IF Pollack, PD Adelson (eds) Principles and practice of pediatric neurosurgery. Thieme, New York, 545-562... 

Schwartz WJ, Stakes JW, Hobson JA (1984) Transient cataplexy after removal of a craniopharyngioma. Neurology 34 1372-1375... 

Figure 20.1 shows a cerebral tumor from the lumbosacral region of a middle-aged woman. The H E-stained slide reveals a neoplasm with mainly epithelioid cells and a few clear cells, and abundant round to oval nuclei with fine chromatin (Table 20.6 see Fig. 20.lA). Its IHC is focally positive to epithelial membrane antigen (EMA) (see Fig. 20.IB). Boxes 20.1 and 20.2 show carcinoma, chordoma, craniopharyngioma, pituitary adenoma, and meningioma to be EMA positive. This tumor is negative for cytokeratin (CK) CAM5.2 (see Fig. 20.1C), so it does not fit the immunohistochemical...

CAMS.2 cytokeratin/BD/1 10 Carcinoma craniopharyngioma chordoma epithelia Ventana protease 2, 16 min... 

EMA/Dako/1 50 Carcinoma meningioma craniopharyngioma chordoma epithelia Mw 15 min in citrate, pH 6.0... 

Craniopharyngioma Squamous adamantinomatous Cytokeratin (-I-) Suprasellar sellar... 

Poor sampling of cystic craniopharyngioma may yield a few epithelial cells of unknown origin. In such cases, lack of cytokeratins 8 and 20 favor craniopharyngioma over epithelial cysts common in the same location. Craniopharyngiomas express more high molecular weight keratin than most carcinomas metastatic to brain (Fig. 20.57). 

Confusion can arise from sampling of only the intensely GFAP-positive gliotic margin of a craniopharyngioma, which may closely resemble a pilocytic... 

FIGURE 20.57 Most craniopharyngiomas are obvious from their structural features, as was this adamantinomatous craniopharyngioma. Nonetheless, its immunoreactivity for high molecular weight keratin 903 distinguishes it even further and emphasizes the cytoplasm of shrunken epithelial cells (stellate reticulum). 

Cysts differ from tumors in their lack of a solid nodule of tissue. This simple fact is critical to distinguishing glial cysts from gliomas and epithelial cysts from cystic craniopharyngiomas. Cysts specific to nervous tissue are emphasized here. Others are described in their primary chapters. 

Cystic Wall of adamantinomatous or incompletely craniopharyngioma keratinized squamous epithelium cyst contains "motor oil" Cytokeratin (-r) Suprasellar sella... 

Xin W, Rubin MA, McKeever PE. Differential expression of cytokeratins 8 and 20 distinguishes craniopharyngioma from Rathke cleft cyst. Arch Pathol Lab Med. 2002 126 1174-1178. 

The frequency of secondary or central hypothyroidism in adult patients is clearly lower than that of primary thyroid failure (Table 106.1). This form of hypothyroidism arises from diseases that interfere with the synthesis and release of hypothalamic TRH or pituitary TSH (Rose, 2001 Asteria et ai, 2001). The most common causes are pituitary adenomas and their therapy by surgery or radiotherapy. Less frequent causes of central hypothyroidism are hypothalamic tumors (craniopharyngioma), infiltrative diseases, head trauma, lymphocytic hypophysitis, infections, infarction and metastases (Beck-Peccoz et ai, 1996 Rose, 2001). 

The most common causes of central hypothyroidism are pituitary adenomas, craniopharyngiomas and the surgery and/or radiotherapy used to treat them. Central hypothyroidism can also result from other tumors (i.e., glioma, meningioma, chordoma, etc.), from a number of inffltra-tive conditions, such as sarcoidosis and histiocytosis, and from postpartum necrosis (Sheehans syndrome). 

In a prospective study of 117 patients with childhood craniopharyngiomas, who were evaluated after 3 years of follow-up, overall survival was 97% and event-free survival 46% the recurrence rate was 36% after complete resection in 47 patients and progression rate was 69% after incomplete resection in 64 irradiation was protective and growth hormone administration had no effect on event-free survival [2T],... 

In 56 patients with craniopharyngiomas, mean age at diagnosis 25 years, who took growth hormone for a mean of 14 years and 70 who did not take growth hormone, the 10-year tumor progression-free survival rate for the entire population was 72% it was 88% in those who took growth hormone compared with 57% in the cortirols [235],... 

Taguchi T, Takao T, Iwasaki Y, Pooh K, Okazaki M, Hashimoto K, Terada Y. Rapid recurrence of craniopharyngioma following recombinant human growth hormone replacement. J Neurooncol 2010 100(2) 321-2. 

Olsson DS, Buchfelder M, Wiendieck K, Kremenevskaja N, Bengtsson BA, Jakobsson KE, Jarfelt M, Johannsson G, Nilsson AG. Tumour recurrence and enlargement in patients with craniopharyngioma with and without GH replacement therapy during more than 10 years of follow-up. Eur J Endocrinol 2012 166 (6) 1061-8 [erratum 2012 167(1) 135]. 

Classical craniopharyngioma is a cystic tumor containing a clear yellowish or brownish fluid, and stuffed with dispersed solid gray nodules, which may accumulate cho-lesteroid material or be calcified... 

Kickingereder P et al (2012) Intracavity brachytherapy using sterotactically applied phosphorus-32 colloid for treatment of cystic craniopharyngiomas in 53 patients. J Neurooncol 109 365-374. doi 10.1007/s 11060-012-0902-8... 

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