Loeys-Dietz syndrome (LDS) is a rare genetic disorder characterized by systemic connective tissue abnormalities. Among its subtypes, LDS type 3 is associated with SMAD3 gene mutations and often presents with vascular and skeletal abnormalities. Narrow chest is a relative contraindication for minimally invasive cardiac surgery (MICS), yet this approach can be advantageous in connective tissue disorders where repeated surgeries may be anticipated. A 63-year-old woman with a previously unreported SMAD3 variant was diagnosed with LDS type 3. She presented with severe mitral regurgitation due to A2-3 prolapse. Her skeletal features included a narrow chest (anteroposterior diameter: 5 cm), scoliosis, and pectus excavatum. Totally endoscopic 3D mitral valve plasty was performed via a right minithoracotomy using a 2-port, 1-window approach. Mitral repair was successfully completed using artificial chordae and ring annuloplasty. Adequate exposure was achieved despite the narrow chest by retracting the pericardium and displacing the aorta using gauze packing. The mitral valve was clearly visualized using the 3D endoscopic camera, allowing safe repair of the A2-3 prolapse with four artificial chordae and a 29-mm Tailor ring. The patient was extubated 3.5 hours postoperatively and had an uneventful recovery except for transient atrial fibrillation. She was discharged on postoperative day 13 in sinus rhythm. Totally endoscopic MICS-MVP is feasible and beneficial even in patients with challenging thoracic anatomy due to connective tissue disease. It enables chest wall preservation and minimizes surgical trauma, which is particularly advantageous for LDS patients with lifelong surgical risk.

Introduction: Pancreatic cancer often recurs as local recurrence or peritoneal dissemination, causing severe abdominal and back pain. We report a case of postoperative skull metastasis of pancreatic cancer that caused headache and was treated effectively with radiotherapy. Case: A 67-year-old woman underwent surgery for pancreatic cancer. She had epigastric pain due to local recurrence and peritoneal dissemination, which was controlled with loxoprofen and fentanyl transdermal patch. She had right-sided headache, which gradually increased in intensity, but lacked intracranial hypertension and focal neurological symptoms. Acetaminophen and Goreisan did not provide adequate pain relief. Although CT showed no brain or bone metastasis, MRI showed metastasis in the right frontal bone. Palliative radiotherapy was administered. Subsequently, headache decreased from 7–8/10 to 2–3/10 on the Numerical Rating Scale. Conclusion: Skull metastases in cancer patients may be diagnosed using MRI, even when they remain undetectable by CT.

The Fourth Edition of the Guidelines for Treatment of Uterine Body Neoplasm was published in 2018. These guidelines include 9 chapters: 1. Overview of the guidelines, 2. Initial treatment for endometrial cancer, 3. Postoperative adjuvant therapy for endometrial cancer, 4. Post-treatment surveillance for endometrial cancer, 5. Treatment for advanced or recurrent endometrial cancer, 6. Fertility-sparing therapy, 7. Treatment of uterine carcinosarcoma and uterine sarcoma, 8. Treatment of trophoblastic disease, 9. Document collection; and nine algorithms: 1-3. Initial treatment of endometrial cancer, 4. Postoperative adjuvant treatment for endometrial cancer, 5. Treatment of recurrent endometrial cancer, 6. Fertility-sparing therapy, 7. Treatment for uterine carcinosarcoma, 8. Treatment for uterine sarcoma, 9. Treatment for choriocarcinoma. Each chapter includes overviews and clinical questions, and recommendations, objectives, explanation, and references are provided for each clinical question. This revision has no major changes compared to the 3rd edition, but does have some differences: 1) an explanation of the recommendation decision process and conflict of interest considerations have been added in the overview, 2) nurses, pharmacists and patients participated in creation of the guidelines, in addition to physicians, 3) the approach to evidence collection is listed at the end of the guidelines, and 4) for clinical questions that lack evidence or clinical validation, the opinion of the Guidelines Committee is given as a “Recommendations for tomorrow”.

Carcinosarcoma ; Choriocarcinoma ; Conflict of Interest ; Endometrial Neoplasms ; Female ; Gestational Trophoblastic Disease ; Humans ; Japan ; Pharmacists ; Pregnancy ; Sarcoma ; Trophoblasts