Favorable long-term outcome of low-grade oligodendrogliomas irrespective of 1p/19q status when treated without radiotherapy

Despite the accumulating evidences of high chemosensitivity especially in anaplastic oligodendrogliomas with loss of chromosomes 1p and 19q, the optimal management strategy for low-grade tumors using the 1p/19q information remains controversial. We have treated all low-grade oligodendrogliomas by a chemotherapy-preceding strategy without radiotherapy, and here we analyzed the survival outcomes of 36 consecutive patients in relation to 1p/19q status. The treatment protocol was as follows: (1) simple observation after gross total resection, and (2) modified PCV chemotherapy for postoperative residual tumors or recurrence after total resection. The 1p and 19q status were analyzed by fluorescence in situ hybridization. The median follow-up period was 7.5 years and no patient was lost during the follow-up periods. 1p/19q co-deletion was observed in 72% of the patients, and there was no significant association between 1p/19q co-deletion and chemotherapy response rate. The 5- and 10-year progression-free survival (PFS) rate was 75.1 and 46.9%, respectively, and the median PFS was 121 months for 1p/19q-deleted tumors and 101 months for non-deleted tumors (log-rank test: P = 0.894). Extent of surgery did not affect PFS (P = 0.685). In contrast, the elder patients (>50) had significantly shorter PFS (P = 0.0458). Recurrent tumors were well controlled by chemotherapy irrespective of 1p/19q status, and 35 out of 36 patients survived without receiving radiotherapy. The 5- and 10-year overall survival rates were 100 and 93.8%, respectively. Two of the patients in their sixties (29%) suffered from severe cognitive dysfunctions and marked brain atrophy following chemotherapy alone. These results show that low-grade oligodendrogliomas could be successfully treated by surgical resection and nitrosourea-based chemotherapy alone without radiotherapy irrespective of 1p/19q status.     

Continuous intraoperative monitoring of abnormal muscle response in microvascular decompression for hemifacial spasm; a real-time navigator for complete relief

Intermittent monitoring of abnormal muscle response (iAMR) has been reported to be useful for improving the surgical outcome of microvascular decompression (MVD) for hemifacial spasm (HFS). However, iAMR has not elucidated the relationship between AMR change and the corresponding surgical procedure, or the pathogenesis of AMR and HFS. The purpose of this study is to clarify the usefulness of continuous AMR monitoring (cAMR) for improving the surgical results of MVD and for understanding the relationship between AMR change and corresponding surgical procedure, and the pathogenesis of AMR and HFS. Fifty consecutive patients with HFS treated by MVD under cAMR monitoring, which continuously records AMR every minute throughout the surgical period, were retrospectively analyzed. The patients were assessed for the presence of HFS 1 week after the surgery and at final follow-up. Forty-six patients showed the complete disappearance of HFS. In 32, AMR disappeared abruptly and simultaneously with decompression of an offending vessel. AMR showed dynamic and various changes including temporary disappearance, or sudden, gradual, or componential disappearance before and during the decompression procedure, and even during the dural and skin closure after the initial decompression procedure. Facial spasm remained in four patients despite permanent AMR disappearance. cAMR monitoring improves the outcome of MVD. Although the main cause of HFS and AMR is vascular compression at the facial nerve, hyperexcitability of the facial nucleus is also involved in the pathogenesis of HFS and AMR. The proportional involvement of these causes differs between patients.     

Bowel perforation due to metastatic lung cancer: a case report]

We report a case of small bowel perforation secondary to metastasis from a lung squamous cell carcinoma that occurred in a 72-year-old man. He was admitted to the hospital because of remittant hemoptysis after undergoing a right upper lobectomy. Fiberoptic bronchoscopy revealed bleeding from the right upper lobe bronchial stump that was due to recurrence of the tumor. He developed abdominal pain, nausea and vomiting four months after resection of the primary tumor. An abdominal radiograph demonstrated free air in the abdominal cavity. He was diagnosed as having intestinal perforation and was operated on. The operative findings indicated adherence and perforation of the jejunum and ileum. The pathological diagnosis of the removed tumor was poorly differentiated squamous cell carcinoma. The patient died 32 days after the second operation. Although abdominal metastasis from lung cancer is not an unusual postmortem finding, it is rare in clinical situations. If a patient with lung cancer complains of abdominal pain, it is important to consider the possibility of abdominal metastasis.     

IgG4 antibodies in patients with atopic dermatitis

The role of IgG4 in atopic dermatitis was investigated by determining the total amounts of IgG4 and of IgG4 specific for ovalbumin (a food allergen), Dermatophagoides farinae mite antigen and house dust (inhalant allergens) and Candida. These were related to the amounts of total and antigen specific IgE in patients with atopic dermatitis and normal healthy controls. Most patients with atopic dermatitis had greater amounts of total IgG4 and of antigen-specific IgG4 than did normal control individuals. Patients who had received hyposensitization treatment injections had greater amounts of IgG4 than the atopic dermatitis patients not so treated. In patients treated by hyposensitization there was a large increase in the amount of blocking antibody detected by incubating the antigen with the serum overnight before injecting the mixture into the skin of a patient sensitive to the antigen. Blocking activity was also examined by partial inhibition by the serum of IgE-mediated mast cell degranulation and by injection of serum into the skin of sensitive patients before challenge with antigens. In all tests the blocking activity of the serum was related to the amount of antigen-specific IgG4 but not related to total IgG4. In patients with atopic dermatitis who were sensitive to mite antigen, severe cases had small amounts of specific IgG4 and large amounts of specific IgE but in mild cases there was an opposite trend with relatively large amounts of specific IgG4. Large amounts of IgG4 ovalbumin specific antibody were found in children and adults with atopic dermatitis and egg allergy but small amounts of IgE. In infants most of the anti-ovalbumin antibody was IgE with little or no IgG4. The work of others has confirmed that increased amounts of total and antigen-specific IgG4 occur in atopic dermatitis, and it is concluded that IgG4 is a blocking antibody for anaphylactic sensitization responses.     

Surgical treatment of a calcified Rathke's cleft cyst with endoscopic extended transsphenoidal surgery--case report

A 34-year-old male presented with a rare case of Rathke's cleft cyst (RCC) with calcification manifesting as persistent high fever and impaired consciousness. Physical findings revealed panhypopituitarism and bitemporal hemianopsia. Computed tomography showed mass lesions with marked calcification within the sella turcica and the suprasellar region. Magnetic resonance imaging showed solid and cystic components compressing the optic nerve. The preoperative diagnosis was craniopharyngioma. Initial endonasal transsphenoidal surgery (TSS) was performed with a surgical microscope, but the mass was extremely hard, so only partial removal was possible. Second endonasal extended TSS was performed with a neuroendoscope. The solid components were totally removed, but calcifications adhering to the optic nerve could not be removed completely. The histological diagnosis was RCC with marked granulation reaction. RCC with calcification is rare and difficult to differentiate from craniopharyngioma on neuroimages. Extremely thick calcification of the sella turcica enclosing granulation tissue and the cyst similar to armor, here called "armor-like calcification," is a characteristic imaging finding of RCC with calcification. The most important aspect is choosing a surgical approach to carefully and effectively relieve pressure upon the optic nerve. Endonasal extended TSS with an endoscope was effective in the present case.     

Craniofacial Resection for Sinonasal Malignant Tumors: Statistical Analysis of Surgical Outcome over 17 Years at a Single Institution

We present a retrospective analysis of surgical outcome of sinonasal malignant tumors. Overall survival (OS), disease-specific survival (DSS), local control (LC), and disease-free survival (DFS) were calculated in 32 patients. Prognostic factors for survival and functional outcomes were investigated. The median follow-up period was 70 months. At 5 years, OS, DSS, LC, and DFS rates were 0.722, 0.745, 0.851, and 0.707, respectively. Prognostic factors for poor OS were involvement of the frontal sinus (p = 0.023), T classification (T4, p = 0.025), surgical complications (p = 0.029), chemotherapy (p = 0.035) postsurgical infection (p = 0.043), involvement of the orbit (p = 0.048), histology (squamous cell carcinoma, p = 0.049), and radiotherapy (p = 0.043). Prognostic factors for poor DSS were radiotherapy (p = 0.030), chemotherapy (p = 0.036), positive surgical margin (p = 0.034), and T classification (T4, p = 0.050). LC was adversely influenced by surgical procedure (combined frontotemporal resection, p = 0.035) and positive surgical margin (p = 0.049). DFS was adversely influenced by positive surgical margin (p = 0.001). Prognostic factors for poor functional outcome were postsurgical infection (p = 0.039), postsurgical complications (p = 0.040), tumor location (maxillary sinus, p = 0.042, orbit, p = 0.0002), number of sinuses involved (number of sinuses involved was inversely proportional to functional outcome, p = 0.027), T classification (T4 p = 0.007), pathology (squamous cell carcinoma, p = 0.023), and chemotherapy (p = 0.048). Craniofacial resection was an effective surgical option.     

Successful pregnancy and delivery after removal of gonadotrope adenoma secreting follicle-stimulating hormone in a 29-year-old amenorrheic woman

We report a case of ovarian hyperstimulation related to a gonadotroph adenoma in a 29-year-old woman. The patient presented with amenorrhea and large cystic ovaries. Her serum estradiol was markedly elevated (up to 31,100 pmol/l). Serum LH was low, but serum FSH and PRL were normal. Cranial magnetic resonance imaging study revealed a pituitary macroadenoma. After successful removal of the pituitary tumor, FSH, LH and estradiol normalized and fluctuated within normal ranges thereafter. The patient resumed regular cycles of menstruation and conceived spontaneously. During pregnancy, estradiol increased and FSH and LH decreased. The finding confirms restoration of negative feedback of estradiol on FSH and LH secretion. The pregnancy course was uneventful and enlargement of ovaries did not occur.