Birt-Hogg-Dubé Syndrome with Renal Cancer Treated as Multiple Metastases of Cancer of Unknown Primary

A 60-year-old woman presented with multiple lung and bone metastases with unknown primary cancer. Chest CT images showed multiple pulmonary cysts, predominantly of the middle and lower lobes. She also had a history of pneumothorax. Four years after chemotherapy and radiation therapy, multiple hypervascular tumors eventually developed in the bilateral kidneys, suggesting the possibility of Birt-Hogg-Dubé (BHD) syndrome. Genetic testing revealed a folliculin mutation, which confirmed the diagnosis of BHD syndrome. When we encounter cancer of unknown primary with multiple pulmonary cysts in a patient with a history of pneumothorax, thorough imaging of the kidneys and genetic testing for BHD syndrome is necessary.     

Urinary protein as a marker for systolic blood pressure reduction in patients with type 2 diabetes mellitus participating in an in-hospital diabetes education program

Increased blood pressure (BP) and urinary protein (UP)/microalbuminuria are risk factors for cardiovascular disease in patients with diabetes. Although the management of BP in patients with diabetes should involve a multidisciplinary therapy, there are no reports in which modulators have been identified in an in-hospital diabetes education program. The aim of the present study was to investigate the change in BP levels in patients with type 2 diabetes mellitus (T2DM) during a short-term (2-week) in-hospital education program on lifestyle modifications. A total of 167 patients with T2DM (101 men, 66 women; mean age, 61.1 years; glycated hemoglobin, 9.2%) were divided into 2 groups on the basis of their urinary albumin levels: 1 group without UP (urinary albumin level < 30 mg/day) and 1 group with UP (urinary albumin level ≥ 30 mg/day). We defined efficacy in reducing BP as a 20-mm Hg reduction in systolic BP (SBP) and a 10-mm Hg reduction in diastolic BP, and compared the changes between the 2 groups. Although the group with UP had higher SBP levels than the group without UP at baseline, this difference disappeared at the end of the program. Adjusted multivariate logistic regression analysis showed that UP at baseline was associated with a reduction in SBP (odds ratio, 3.361; 95% confidence interval, 1.29-8.79; P = 0.013). The data suggest that UP may be a marker related to the management of SBP through lifestyle modifications in patients with T2DM.     

Kaposiform hemangioendothelioma arising in the deltoid muscle without the Kasabach–Merritt phenomenon

Kaposiform hemangioendothelioma (KHE) is a rare tumor that occurs nearly exclusively during infancy and childhood. It has features common to both capillary hemangioma and Kaposi sarcoma and for that reason many terms have been used for these tumors including “Kaposi-like infantile hemangioendothelioma” and “hemangioma with Kaposi sarcoma-like features.” KHE typically presents as an ill-defined, red to purple, indurated plaque and is often complicated by the Kasabach–Merritt phenomenon (KMP), a condition of severe thrombocytopenia and consumptive coagulopathy. Knowledge of the radiological findings of this uncommon tumor might be helpful for diagnosis. We present the MRI features of a case of KHE with neither typical skin lesions nor the Kasabach–Merritt phenomenon.     

Uterine Arteriovenous Fistula Treated With Repetitive Transcatheter Embolization: Case Report

Uterine arteriovenous fistula (AVF) is a rare entity, but may lead to life-threatening hemorrhage. Although transcatheter embolization, surgical ligation, or hysterectomy would be considered for treatment of uterine AVF, there is poor knowledge as to how gynecologists can manage the uterine AVF with multiple large inflow arteries. Herein we report a uterine AVF successfully treated using multiple-step transcatheter embolization. The patient, a 58-year-old postmenopausal woman with a history of dilation and curettage, had intermittent massive uterine bleeding. Radiologic imaging revealed the presence of a large vasculature mass. The mass occupied the entire pelvis, and the source of hemorrhage was identified as an accompanying AVF. We thought that surgical intervention was contraindicated because of the potential risk of uncontrollable intraoperative bleeding. Multiple-step transcatheter embolization was performed, with complete resolution of the AVF. Thereafter, the patient had no further uterine bleeding. Multiple-step transcatheter embolization might be the most beneficial and efficient treatment option for a uterine AVF with multiple large inflow arteries.     

Diameter of dominant leiomyoma is a possible determinant to predict coexistent endometriosis

Objective

To identify the frequency and assess risk factors for unexpected discovery of peritoneal endometriotic implants in patients who underwent myomectomy or hysterectomy for symptomatic uterine leiomyomas.

Study design

We retrospectively collected medical records of 829 patients with symptomatic leiomyomas in The University of Tokyo Hospital. All the patients underwent abdominal or laparoscopic surgeries between January 2001 and December 2010 and the presence or absence of endometriosis during surgery was analyzed. Possible determinant to predict coexistent endometriosis was statistically investigated.

Results

In total, 105 leiomyoma cases (12.7% in 829 patients) were diagnosed with endometriosis. Patients with small dominant leiomyomas were significantly complicated by peritoneal endometriotic implants (small leiomyomas were classified as <8 cm). The patients with both diagnoses were more likely to be infertile and at age 39 years or younger than those with leiomyoma alone.

Conclusions

Women undergoing myomectomy or hysterectomy with both endometriosis and leiomyomas have several different clinical features compared with women with only leiomyomas. The size of largest leiomyoma may provide an important clue for coexistent endometriosis. Women with substantial infertility despite a smaller leiomyomas burden may be more likely to have a surgical indication for concomitant endometriosis.     

A radiographical classification of Stafne's bone cavity

So-called Stafne's bone cavity of the mandible were detected by panoramic radiography in 24 patients seen at the Second Department of Oral and Maxillofacial Surgery, Meikai University Hospital, and five associated hospitals. Unilateral and asymptomatic, all lesions were situated near the angle of the mandible, except for the one case that involved the anterior portion of the mandible. The mean age of the patients was 53 years with a range of 31 to 78 years, and included 22 men and 2 women. In this report, radiographic interpretation of the lesion was done, and a radiographic classification was made as follows:

1. (Indentation type): The inferior border of the mandible is involved.
2. (Medium type): The lesion is situated above the inferior border of the mandible but below the mandibular canal.
3. (Deviation type): Deviation of the mandibular canal is suspected.
4. (Anterior type): The radiolucency is located in the anterior portion of the mandible.