The Tohoku journal of experimental medicine
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Tohoku J. Exp. Med. · Nov 2024
Review Case ReportsFocal Light Chain Proximal Tubulopathy Complicated by Monoclonal Gammopathy of Undetermined Significance/Smoldering Multiple Myeloma Successfully Diagnosed by Immunofluorescence on Pronase-Digested Paraffin Section: Reports of Two Cases and Review of the Literature.
Light chain proximal tubulopathy (LCPT) is a rare type of paraprotein-related disease (PRDs) characterized by monoclonal free light chain (FLC) deposition in proximal tubular epithelial cells (PTECs). A diagnosis of LCPT requires identification of FLC deposition in PTECs; however, FLC luminescence defects in immunofluorescence staining using frozen tissue (IF-F), regarded as "masked LCPT", are occasionally encountered. We describe two cases of focal masked LCPT in monoclonal gammopathy of undetermined significance (MGUS) or smoldering multiple myeloma (SMM) diagnosed by IF in formalin-fixed, paraffin-embedded tissue sections following pronase digestion (IF-P) rather than by IF-F. ⋯ According to a literature review of 33 cases, including our 2 cases, focal LCPT complicated by MGUS/SMM is relative rare. In PRD, evaluation with IF-P is desirable for assessing LCPT when FLC deposition is undetected by IF-F despite characteristic degenerative PTECs. We consider that early and definitive diagnosis of LCPT by IF-P rather than IF-F might result in favorite outcome since physicians could smoothly decide treatment strategy.
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Tohoku J. Exp. Med. · Dec 2023
Review Case ReportsSmall Intestinal Adenocarcinoma Arising at the Anastomotic Site after Kasai Operation for Biliary Atresia: A Case Report and Literature Review.
Biliary atresia is an obliterative cholangiopathy of unknown etiology. Hepatic portoenterostomy, in which obliterated extrahepatic bile ducts are resected and bile flow is restored, known as Kasai operation, is performed within 3 months after birth. While this operation enhances long-term survival of patients, the occurrence of primary malignant hepatic tumors has been increasing. ⋯ The patient had not experienced recurrence at 10 months after the operation. This is the first report of small intestinal adenocarcinoma arising at the anastomotic site after Kasai operation for biliary atresia. Special care should be taken for the patients after Kasai operation with acute progressive jaundice and liver dysfunction because there is a possibility of malignancy in their native liver.
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Tohoku J. Exp. Med. · Nov 2023
ReviewChronic Glucocorticoid Use is a Potential Risk Factor for Delayed Pancreatic Fistula after Laparoscopic Distal Pancreatectomy: A Retrospective Analysis.
Pancreatic fistula is a potentially morbid complication after distal pancreatectomy. Chronic glucocorticoid use is one of the risk factors for pancreatic fistula in pancreaticoduodenectomy, though it has not been reported in distal pancreatectomy. We explored whether chronic glucocorticoid use can be a risk factor for pancreatic fistula in distal pancreatectomy. ⋯ A comparison of patient with and without chronic glucocorticoid use showed that only patients with chronic glucocorticoid use developed delayed pancreatic fistula (0% vs. 16.7%; p < 0.001). In addition, delayed pancreatic fistula occurred in only laparoscopic distal pancreatectomy patients with chronic glucocorticoid use (0% vs. 25.0%; p < 0.001). Although sample size is small, it is reasonable to presume that chronic glucocorticoid use is a potential risk factor for delayed pancreatic fistula in laparoscopic distal pancreatectomy.
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Tohoku J. Exp. Med. · Oct 2023
Review Case ReportsLiving-Donor Liver Transplantation for Erythropoietic Protoporphyria: A Case Report and Literature Review.
Erythropoietic protoporphyria (EPP) is a very rare disease with an estimated prevalence of 1 in 200,000 individuals. Decreased ferrochelatase activity causes the accumulation of protoporphyrin in the body, and light exposure results in the generation of active oxygen, causing photosensitivity. Liver damage has the greatest influence on the prognosis, and liver transplantation is the only treatment option for patients with decompensated liver cirrhosis. ⋯ As a result, no adverse events occurred during the perioperative period, and the patient was discharged on the 46th post-operative day. Currently, the transplanted liver is functioning extremely well, and the patient is alive 3 years post-transplant. Herein, we describe a case of living donor liver transplantation for EPP with a brief literature review.
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Tohoku J. Exp. Med. · Sep 2023
Review Case ReportsTumor-to-Tumor Metastasis of Medullary Thyroid Carcinoma to Paraganglioma in a Multiple Endocrine Neoplasia Type 2B Patient: A Case Report and Literature Review.
Tumor-to-tumor metastasis is a rare phenomenon in which primary tumor cells metastasize to other tumors. Herein, we report an extremely rare case of tumor-to-tumor metastasis of medullary thyroid carcinoma to a paraganglioma in a patient with multiple endocrine neoplasia type 2B. Based on genetic examination, a 36-year-old woman was diagnosed with multiple endocrine neoplasia type 2B when she was 24 years old. ⋯ Inside the tumor, a white nodule positive for carcinoembryonic antigen, weakly positive for calcitonin, and negative for tyrosine hydroxylase, was identified and diagnosed as a metastatic medullary thyroid carcinoma with high malignant potential. The liver lesion was diagnosed as a metastasis of the medullary thyroid carcinoma. This is the first report of tumor-to-tumor metastasis of medullary thyroid carcinoma to paraganglioma in a patient with multiple endocrine neoplasia type 2B twenty years after total thyroidectomy.