Pheochromocytoma as the first manifestation of MEN2A with RET mutation S891A: report of a case

We report a rare case with pheochromocytoma as the first manifestation of multiple endocrine neoplasia type 2A with RET mutation S891A. Bilateral pheochromocytomas were identified in a 54-year-old woman. Screening for RET revealed a rare S891A mutation located in the intracellular tyrosine kinase domain. This mutation was previously recognized as one of the mutations only in cases manifesting solely medullary thyroid carcinomas (MTCs). Since calcitonin stimulation test indicated positive result, total thyroidectomy was performed 1 year after the bilateral adrenalectomy, and C-cell hyperplasia was diagnosed by histopathological examination. Our report suggests that cases with S891A mutation, akin to those with other RET mutations, require screening for pheochromocytoma. In addition, it is indicated that calcitonin stimulation test should be performed even in the unaffected elder cases with S891A mutation although the mutation is classified as lowest risk group on MTC in guidelines.     

Extravasation on three-dimensional CT angiography in patients with acute subarachnoid hemorrhage and ruptured aneurysm

Three-dimensional computed tomographic angiography (CTA) is a noninvasive technique for detecting lesions after acute subarachnoid hemorrhage. We encountered extravasation on CTA, a finding that has not been reported previously. Three patients with saccular aneurysms showed extravasation on CTA performed within 3 h of the onset of hemorrhage, and all three patients died within 2 weeks. At autopsy, the site of rupture of the aneurysm was confirmed in all three cases. There were two patterns of extravasation shown by CTA, which seemed to depend on the direction of rupture. Extravasation on CTA might represent the natural progression of ruptured aneurysm and may indicate a poor prognosis.     

Potential of (99m)Tc-MIBI for detecting bone marrow metastases.

In this study, we evaluated the potential of (99m)Tc-hexakis-2-methoxyisobutylisonitrile (MIBI) for detecting bone metastases in comparison with a conventional bone tracer. METHODS: (99m)Tc-MIBI and (99m)Tc-hydroxymethylene diphosphonate (HMDP) scans were obtained from 99 patients with proven malignant diseases and suspected bone metastases. We compared 373 lesions that showed abnormal uptake on (99m)Tc-MIBI scans or (99m)Tc-HMDP scans (or both). RESULTS: Bone metastases were confirmed in 334 of 373 lesions. Thirty-nine lesions on (99m)Tc-HMDP scans had false-positive findings, but only 2 of these lesions had false-positive findings on (99m)Tc-MIBI scans. (99m)Tc-MIBI and (99m)Tc-HMDP scans were equivalent in 168 of 334 lesions (50.3%). (99m)Tc-MIBI scans correctly detected more lesions than (99m)Tc-HMDP scans: 284 lesions (85.0%) versus 218 lesions (65.3%) (P < 0.005), respectively. (99m)Tc-MIBI scans showed a markedly higher sensitivity for detecting metastases in the femur and humerus compared with (99m)Tc-HMDP scans: 97 of 98 lesions (99.0%) versus 35 of 98 lesions (35.7%) (P < 0.005) and 21 of 22 lesions (95.5%) versus 11 of 22 lesions (50.0%) (P < 0.005), respectively. (99m)Tc-HMDP scans of 17 patients showed no abnormal images. However, (99m)Tc-MIBI scans correctly detected bone metastases, and subsequent development of multiple lesions was observed on follow-up (99m)Tc-HMDP scans of 15 patients. (99m)Tc-MIBI scans were superior to (99m)Tc-HMDP scans in the detection of metastases attributed to breast cancer, multiple myeloma, and hepatoma. On the contrary, (99m)Tc-MIBI scans were less sensitive than (99m)Tc-HMDP scans for detecting bone metastases attributed to prostate cancer in the other skeletal sites except for femur and humerus. CONCLUSION: (99m)Tc-MIBI scans have better sensitivity for detecting bone metastases and provide more specific complementary findings than conventional bone scans. (99m)Tc-MIBI accumulation attributed to bone marrow metastases may occur at an early stage, before the bone remodeling process in the surrounding bone can be detected on conventional bone scans.     

Imaging features of maxillary osteoblastoma and its malignant transformation

We report two cases of osteoblastoma, one of them an unusual case in a 32-year-old woman in whom a maxillary tumor was confidently diagnosed as an osteoblastoma at the time of primary excision and subsequently transformed into an osteosarcoma 7 years after the onset of clinical symptoms. The other patient developed osteosarcoma arising in the maxilla, which was diagnosed 3 years after the primary excision and is very suggestive of malignant transformation in osteoblastoma. We present the radiological features, including computed tomographic and magnetic resonance imaging studies, of this unusual event of transformed tumor and compare imaging features of benign and dedifferentiated counterparts of this rare tumor complex.Members of the Group for the Image Diagnosis of Craniofacial Disorders     

Clinical impact of amyloid PET using 18F-florbetapir in patients with cognitive impairment and suspected Alzheimer’s disease: a multicenter study

Annals of Nuclear Medicine - Amyloid positron emission tomography (PET) can reliably detect senile plaques and fluorinated ligands are approved for clinical use. However, the clinical impact of...     

Postoperative liver dysfunction in living donors after left-sided graft hepatectomy: portal venous occlusion of the medial segment after lateral segmentectomy and hepatic venous congestion after left lobe hepatectomy

Background

The aim of this study was to evaluate how sacrifice of the portal vein and/or hepatic vein affects remnant liver dysfunction after lateral segmentectomy or left lobe hepatectomy.

Materials and methods

Among 130 patients who underwent donor hepatectomy between March 2002 and July 2011, we enrolled lateral segment (n = 15) and left lobe donors (n = 40). We evaluated the postoperative courses and the territory of venous obstruction or congestion based on the sacrificed portal vein or hepatic vein after the donor operation: lateral segment grafts (P4a, P4b, LV4) and left lobe grafts (MV5, MV8) according to the results analyzed by MeVis Distant Service.

Results

Among lateral segment donors, the predicted sacrificed territory of portal vein and hepatic vein was 14.3% (7.3%-19.4%) in P4a + 4b: (P4a: 8.6%, P4b: 5.8%) and 2.9% (0%-8.4%) in LV4, respectively. On the other hand, in left lobe donors, the predicted congestive territory of the hepatic vein was 17.6% (2.8%-33.0%) in MV5 + 8 (7.8% in MV5 and 9.8% in MV8, respectively). The incidence of patients whose postoperative peak aspartate aminotransferase (AST) or alanine aminotransferase levels were higher than 500 IU/L was 20% in the lateral segment donors and 5% in the left lobe donors. The peak postoperative AST levels and territory of MV5 + 8 showed a significant positive correlation (R = 0.569, P < .05) among left lobe donors.

Conclusion

Territories of P4 in lateral segment donors and MV5 + 8 in left lobe donors impacted postoperative liver dysfunction. It is important to recognize the precise territory of the portal vein and the hepatic vein before the donor operation.     

Living donor liver transplantation for the patients with portal vein thrombosis: use of an interpositional venous graft passed posteriorly to the pancreatic parenchyma without using jump graft

Background

It is difficult to reconstruct the portal vein (PV) using a long interpositional venous graft in living donor liver transplant (LDLT) patients with portal vein thrombosis (PVT), which involves the confluence of the superior mesenteric vein (SMV) and splenic vein (SV). We successfully performed LDLT for three patients with PVT using an interpositional vascular conduit passing posterior to the pancreas without a jump graft.

Methods

Three of 130 patients who underwent LDLT in our hospital between March 2002 and June 2011 required this technique. After indentifying the location of the SMV, SV and gastrocolic trunk, we ligated and cut the posterior superior pancreaticoduodenal vein and other short branches from the PV. The PV was drawn inferiorly to the pancreas and transected at the confluence of SMV and SV. The external iliac vein or internal jugular vein was sacrificed as a graft for anastomosis to the cut end of the SMV using 6-0 polypropylene running sutures. Then the venous graft was drawn superiorly to the pancreas by passing it posterior to the pancreas parenchyma for anastomosis to the liver graft PV. The interpositional vein was placed posterior to the pancreas where the PV used to be.

Results

All three patients displayed favorable postoperative courses with the Doppler ultrasound demonstrating good portal flow perioperatively. The postoperative portogram demonstrated patency of the vascular graft.

Conclusion

This method is easy and helpful to treat portal vein thrombosis, by providing the shortest route between the PV of the donor and the SMV of the recipient.     

Evaluation of anorectal functions of children with anorectal malformations using fecoflowmetry

Background/Purpose: A simple and objective method evaluating the bowel functions of patients with anorectal anomalies is necessary. The authors applied fecoflowmetry and saline enama test for patients with anorectal anomalies to evaluate the fecodynamics and anorectal motilities in these children. Methods: The bowel functions of 16 patients who underwent repair for anorectal malformations and 5 normal controls were evaluated by saline enema test and fecoflowmetry. The correlations between the clinical scores for the bowel functions and the parameters in fecodynamic studies were investigated. Results: Seven of 16 patients exhibited periodical contractions of the rectum synchronized with relaxations of the anal canal during saline infusion as did the controls and had significantly higher clinical scores than the other patients. Two patients with severe chronic constipation lacked rectal contractions. Among the fecoflowmetric parameters, the maximum flow, average flow, and tolerable volume of saline infused into the rectum were significantly lower in the patients with low clinical scores than those of the controls. The maximal squeeze pressure and resting anal pressure were not significantly different between the patients and controls. Conclusion: Fecodynamic studies, such as fecoflowmetry and saline enema test, help in obtaining clinical indicators for the bowel functions of patients with anorectal anomalies.     

Recurrent subdural hematoma caused by cerebrospinal fluid leakage. Case report

The authors report a case of a recurrent subdural hematoma (SDH) that was caused by a persistent cerebrospinal fluid (CSF) leak from an L1-2 fistula. A 34-year-old man experienced severe headaches due to SDH, and he underwent aspiration of subdural fluid four times due to recurrent collections. Further evaluation with computerized tomography (CT) myelography demonstrated extradural extravasation of contrast through an L1-2 fistula. The patient underwent an L1-2 laminectomy; a small dural defect with CSF leakage at the left nerve root sleeve was found and was repaired. Following the repair, the patient had no further recurrence of SDH. Recurrent SDH, caused by spontaneous CSF leakage through a lumbar CSF fistula, is extremely rare. In cases of recurrent SDH, radiographic workup with spinal CT myelography should be considered.     

Symptomatic ossification of the anterior longitudinal ligament with stenosis of the cervical spine: a report of seven cases

Seven men with a mean age of 63.9 years (59 to 67) developed dysphagia because of oesophageal compression with ossification of the anterior longitudinal ligament (OALL) and radiculomyelopathy due to associated stenosis of the cervical spine. The diagnosis of OALL was made by plain lateral radiography and classified into three types; segmental, continuous and mixed. Five patients had associated OALL in the thoracic and lumbar spine without ossification of the ligamentum flavum. All underwent removal of the OALL and six had simultaneous decompression by removal of ossification of the posterior longitudinal ligament or a bony spur. All had improvement of their dysphagia. Because symptomatic OALL may be associated with spinal stenosis, precise neurological examination is critical. A simultaneous microsurgical operation for patients with OALL and spinal stenosis gives good results without serious complications.