Differential Diagnosis of Pancreatobiliary Carcinoma from Autoimmune Pancreatitis-Related Diseases: A Report of Three Cases

Background

“Japanese clinical guidelines for autoimmune pancreatitis” advised to carefully differentiate between two conditions: autoimmune pancreatitis (AIP) and associated sclerosing cholangitis (SC), and pancreatobiliary malignancy.

Methodology

We report a series of three cases for which differential diagnosis of pancreatobiliary carcinoma from AIP and associated SC was crucial.

Result

Three patients presented with biliary stenosis secondary to pancreatic swelling or mass lesion, followed by further examinations: Case 1 was first diagnosed as having tumor-forming pancreatitis associated with AIP but eventually proven to be pancreatic head carcinoma; case 2 was operated for suspected bile duct cancer combined with AIP and associated cholangitis, and early cancer was found in the resected specimen; case 3 was operated on for presumed cholangiocarcinoma combined with AIP-associated SC, but no malignancy was found.

Conclusions

Current series of cases would raise an alert on diagnosis of AIP and associated SC, and pancreatobiliary malignancy should be carefully excluded by any means. Surgical intervention would be required in selected cases of this clinical entity.     

Non-contrast-enhanced hepatic MR angiography: Do two-dimensional parallel imaging and short tau inversion recovery methods shorten acquisition time without image quality deterioration?

Objective

To study whether shortening the acquisition time for selective hepatic artery visualization is feasible without image quality deterioration by adopting two-dimensional (2D) parallel imaging (PI) and short tau inversion recovery (STIR) methods.

Materials and methods

Twenty-four healthy volunteers were enrolled. 3D true steady-state free-precession imaging with a time spatial labeling inversion pulse was conducted using 1D or 2D-PI and fat suppression by chemical shift selective (CHESS) or STIR methods. Three groups of different scan conditions were assigned and compared: group A (1D-PI factor 2 and CHESS), group B (2D-PI factor 2 × 2 and CHESS), and group C (2D-PI factor 2 × 2 and STIR). The artery-to-liver contrast was quantified, and the quality of artery visualization and overall image quality were scored.

Results

The mean scan time was 9.5 ± 1.0 min (mean ± standard deviation), 5.9 ± 0.8 min, and 5.8 ± 0.5 min in groups A, B, and C, respectively, and was significantly shorter in groups B and C than in group A (P < 0.01). The artery-to-liver contrast was significantly better in group C than in groups A and B (P < 0.01). The scores for artery visualization and overall image quality were worse in group B than in groups A and C. The differences were statistically significant (P < 0.05) regarding the arterial branches of segments 4 and 8. Between group A and group C, which had similar scores, there were no statistically significant differences.

Conclusion

Shortening the acquisition time for selective hepatic artery visualization was feasible without deterioration of the image quality by the combination of 2D-PI and STIR methods. It will facilitate using non-contrast-enhanced MRA in clinical practice.     

Final Pathology Findings After Immediate or Delayed Surgery in Patients with Cytologically Benign or Follicular Thyroid Nodules

Background  

In the present study, we reviewed our experience with thyroid cytological findings showing benign or follicular tumor and evaluated the outcomes in patients who had undergone immediate or late surgical treatment.     

Our experience of treatment of cribriform morular variant of papillary thyroid carcinoma; difference in clinicopathological features of FAP-associated and sporadic patients

Cribriform-morular variant (CMV) is a comparably rare histological subtype of papillary thyroid carcinoma (PTC). This can be associated with familial adenomatous polyposis (FAP) due to APC gene mutations. In this study, we investigated the difference in the biological characteristics between FAP-associated and sporadic CMV. Between 1991 and 2010, 32 patients with CMV were treated in Kuma Hospital. Thirty-one of these underwent initial surgery for CMV in Kuma Hospital. Twelve patients were FAP-associated and the remaining 19 were sporadic CMV. All patients were female. Tumors of FAP-associated CMV were more frequently multiple than those of sporadic CMV. Patient age and tumor size did not differ between the two groups. Of 12 FAP-associated CMV, 5 were detected by thyroid nodule (thyroid precedent group) and 7 were detected by FAP (polyposis precedent group) as an initial manifestation. Patient age was younger and tumor size was smaller in the polyposis group than in the thyroid nodule group. All patients lacked extrathyroid extension on intraoperative finding and were node-negative on pathological examination. To date, two patients with FAP-associated CMV who initially underwent hemithyroidectomy (one in Kuma Hospital and one in another hospital) showed recurrence to the remnant thyroid during follow-up. None of the patients showed recurrence to other regions or died of carcinoma. Taken together, CMV is considered an indolent disease in our series. FAP-associated CMV showed multiple tumors more frequently than sporadic CMV. Total thyroidectomy is recommended for FAP-associated CMV, but extensive lymph node dissection is not necessary.     

Wall composition analysis of the human hepatic artery by intravascular ultrasound

Purpose  The purpose of this study was to elucidate the wall layer appearance of the human hepatic artery shown by intravascular ultrasound (IVUS). Methods  We evaluated the wall layer appearance of 57 human hepatic arteries from 36 cadavers. The thickness of the inner high-echoic layer and the second low-echoic layer of the arteries was measured by IVUS, and it was compared with that of the arterial intima and media, respectively. Results  The thickness of the inner high-echoic layer was 0.2 ± 0.1 mm and that of the second low-echoic layer was 0.4 ± 0.1 mm, on IVUS. However, the histological thickness of the intima was 120 ± 45 μm and that of the media was 258 ± 71 μm. The media of the hepatic artery correlated significantly with the second low-echoic layer on IVUS (r = 0.62, P < 0.01). The diameter of the vessels measured on IVUS correlated significantly with that of the histological specimens (r = 0.89, P < 0.01). Conclusion  From our results, the three layers of the hepatic artery detected with IVUS may correspond to the intima, media, and adventitia, respectively.     

Excellent Prognosis of Patients with Nonhereditary Medullary Thyroid Carcinoma with Ultrasonographic Findings of Follicular Tumor or Benign Nodule

Background  Medullary thyroid carcinoma (MTC) accounts for only 1.4% of all thyroid malignancies in Japan. Generally, MTC shows ultrasonographic findings typical of thyroid carcinoma. However, in our experience, some MTC may be diagnosed as a follicular tumor or a benign nodule on ultrasonography because ultrasonographic findings of malignancy are lacking. In this study we investigated differences in biological behavior between these two types of MTC. Methods  Seventy-seven patients with nonhereditary MTC who underwent surgery in our department between 1988 and 2007 were enrolled in this study. Of these patients, 54 were diagnosed as having thyroid carcinoma (malignant, or M-type) but the remaining 23 were diagnosed as having follicular tumor or benign nodule (benign, or B-type) on ultrasonography. Results  Clinically apparent lateral node metastasis, extrathyroid extension, and extranodal tumor extension were observed in 37%, 17%, and 11% of M-type patients, respectively, but none of the B-type patients showed any of these features. All B-type patients but only 59% of M-type patients were biochemically cured. Lymph node metastasis was pathologically confirmed in 38 and 65% of B-type and M-type patients, respectively. Eight patients showed recurrence and three have died of carcinoma to date; all of these patients were M-type patients. Conclusions  B-type MTC is highly indolent and shows an excellent prognosis. However, thyroidectomy and lymph node dissection for B-type MTC should be the same as for M-type MTC because 38% of B-type MTC showed pathologic node metastasis.     

α-Synuclein pathology in the amyotrophic lateral sclerosis/parkinsonism dementia complex in the Kii Peninsula, Japan

α-Synuclein pathology was examined in the brains and spinal cords of 10 patients with amyotrophic lateral sclerosis (ALS)/parkinsonism-dementia complex (PDC) in the Kii Peninsula, Japan. Various types of phosphorylated α-synuclein-positive structures including neuronal cytoplasmic inclusions, dystrophic neurites, and glial cytoplasmic inclusions were found in all ALS/PDC cases. There were phosphorylated α-synuclein-positive neurons in 8 cases (80%), and the amygdala was most severely affected. Phosphorylated α-synuclein was distributed mainly in the limbic system and brainstem; tau pathology was more prevalent than α-synuclein pathology in most affected areas. In the substantia nigra, periaqueductal gray, locus coeruleus, raphe nuclei, dorsal nucleus of the vagus nerve, hypoglossal nucleus or ventral horn, and intermediolateral nucleus of the spinal cord, α-synuclein pathology was more predominant than tau pathology in only 1 or 2 patients. Phosphorylated α-synuclein- positive structures were not found in the molecular layer of the cerebellum. Phosphorylated α-synuclein frequently colocalized with tau in neuron cell bodies, neurites, and glia. Immunoblots of sarkosyl-insoluble fractions extracted from the brain of 1 patient showed a triplet of α-synuclein-immunoreactive bands that were ubiquitinated. These results suggest that interaction between tau and α-synuclein be involved in the pathogenesis of Kii ALS/PDC.