Three Case Reports of Adult-onset Still Disease Associated with Breast Implantation.

Autoimmune syndrome induced by adjuvants (ASIA) is an uncommon clinical condition reported by Shoenfeld et al. Although this syndrome is not scientifically validated, numerous reports on it have been published, and the manifestations are postulated to be diverse, including generalized symptoms such as chronic fatigue, myalgia, arthralgia, or dry mouth, induced by exogenous substances, specifically adjuvants, which can encompass vaccines, organisms, and silicone. Concurrently, adult-onset Still disease (AOSD) is also an infrequent ailment, characterized by spiking fever, arthritis, skin rash, lymphadenopathy, and serositis. Although the precise pathogenesis remains incompletely understood, some case reports suggest that ASIA may be at the root of AOSD development with the same instigator. In this context, we present three cases of patients diagnosed with AOSD, which possibly could be considered an association with ASIA, years after undergoing breast reconstruction with silicone breast implants. In one case, the patient solely received medical treatment due to her refusal to have the implant removed, resulting in multiple flares and severe complications related to glucocorticoid therapy. Conversely, in the other two cases, a combination of immunosuppressive therapy and silicone breast implant explantation led to the complete resolution of clinical symptoms. To the best of our knowledge, there are only 10 documented case reports of AOSD associated with silicone breast implants insertion. We believe this report serves as a complementary addition to prior research and offers further insights into the ongoing debate about whether explantation should be carried out early in the clinical course or not.

Splenic rupture after surgical fixation of rib fractures with video-assisted thoracoscopic surgery: A case report.

INTRODUCTION: Recently, the utilization of surgical stabilization of rib fractures (SSRF) with video-assisted thoracoscopic surgery (VATS) has been increasing owing to its effectiveness. The present report describes the case of a patient who underwent SSRF with VATS and subsequently developed a splenic rupture that was speculated to be related to intrathoracic manipulation during surgery. PRESENTATION OF CASE: A 62-year-old male patient sustained injuries from a fallen festival car over his thoracoabdominal zone and was diagnosed with bilateral multiple rib fractures and burst fractures of the twelfth thoracic and first lumbar vertebrae. The patient underwent SSRF with VATS. Following surgery, the patient went into hemorrhagic shock due to a splenic rupture, necessitating an emergency open splenectomy. DISCUSSION: Despite no initial detection of splenic injury on contrast-enhanced CT, it is possible that a slight splenic injury existed at the time of the initial diagnosis. Moreover, during surgery, additional external forces may have been applied to the spleen due to positional changes, such as shifting to the lateral position or retracting the diaphragm using forceps; these manipulations could have potentially caused a slight splenic injury, possibly leading to splenic rupture. CONCLUSION: When performing SSRF through VATS, it is important to recognize that manipulation and traction of the diaphragm could potentially cause splenic rupture, even if a slight force is applied. Therefore, the diaphragm should be evaluated without traction and manipulation whenever possible.

Anti-MDA5 Antibody-Positive Clinically Amyopathic Dermatomyositis Associated With Multiple Heterologous Carcinomas: A Case Report.

Dermatomyositis (DM), an autoimmune disorder, is linked to increased malignancy risk. A 53-year-old man with anti-melanoma differentiation-associated gene 5 (MDA5)-positive clinically amyopathic dermatomyositis (CADM) and rapidly progressing interstitial lung disease (RP-ILD) developed heterochronous gastric and colorectal cancers. Early endoscopic screenings led to successful curative resections, preventing recurrence. Despite low cancer incidence assumptions in patients with anti-MDA5-positive RP-ILD, this case advocates for reevaluation and periodic cancer screenings to enhance management, considering the improved survival with intensive therapy. Vigilance for multiple carcinomas at various time points is vital in CADM management.

Early detection of occupational cholangiocarcinoma in a high-risk patient under intensive surveillance: a case study.

BACKGROUND: Occupational cholangiocarcinoma is associated with exposure to organic solvents, such as dichloromethane (DCM) and 1,2-dichloropropane (DCP). This report describes a case of occupational cholangiocarcinoma detected through regularly imaging following the discovery of elevated serum γ-glutamyl trans peptidase (γ-GTP) levels revealed during regular checkup. CASE PRESENTATION: A 43-year-old man who had been working in a printing company with 15 years of exposure to organic solvents presented to our hospital owing to abnormalities found during a routine checkup. Ultrasound (US) imaging revealed thickening of the gallbladder wall accompanied by gallstones, although in the blood tests, γ-GTP levels were within normal range. Given the high risk of cholangiocarcinoma development, the patient underwent regular monitoring with abdominal US and blood tests at a local doctor's office. At the age of 48, his serum γ-GTP level mildly elevated for the first time, prompting the initiation of semi-annual magnetic resonance cholangiopancreatography (MRCP). By the age of 50 years, dilation in B8 was detected, and one and a half years later, a tumor on the central side of the B8 dilation appeared. The patient was diagnosed with intrahepatic cholangiocarcinoma, which was treated with anterior sectionectomy. Pathological examination revealed an adenocarcinoma with a papillary glandular ductal structure at the root of the B8. In addition, biliary intraepithelial neoplasia (BilIN) and dysplasia have been identified around the tumor and periphery bile ducts and in noncancerous bile ducts. Postoperatively, the patient received 6 months of adjuvant chemotherapy with S-1monotherapy. Eight months after surgery, the patient remained under observation with no signs of recurrence. CONCLUSIONS: We report a case of occupational cholangiocarcinoma detected during a prolonged period of regular follow-up after exposure to DCM and DCP. Given the delayed carcinogenesis process, occupational cholangiocarcinomas manifest long after exposure to organic solvents, therefore, ongoing screening is extremely important. Vigilance is essential to avoid underdiagnosis, particularly for individuals who are at an increased risk of developing this form of cancer. Continuous monitoring is key to the early detection and effective management of occupational cholangiocarcinoma.

A distal ileum malignant peripheral nerve sheath tumour after abdominal radiation therapy: case report of a rare tumour.

Malignant peripheral nerve sheath tumours (MPNSTs) are malignant tumours arising from a peripheral nerve or displaying nerve sheath differentiation. Most MPNSTs are found on the head, body trunk and extremities, whereas cases in the gastrointestinal are extremely rare. About half arise in neurofibromatosis type 1 patients and 10% arise post-irradiation. This is probably the first small bowel MPNST post-radiation therapy case reported. A 72-year-old female who received radiotherapy 30 years ago for cervical cancer was admitted with progressive abdominal pain and weight loss. Computed tomography revealed a mass with inhomogeneous enhancement in the lumen of the small intestine. Tumour excision was performed with ileocecal and sigmoid colon resection due to suspicion for peripheral tissue invasion. Histopathological examination revealed spindle-shaped cells with focal cartilage differentiation. Together with immunochemistry stain showing complete loss of H3K27me3, a final diagnosis of MPNST was made. The patient is presently under regular follow-ups, and has remained disease-free for 24 months.

[Providing Opportunities for Close Examination of Lynch Syndrome after Microsatellite Instability Testing in a Hospital Setting].

Microsatellite instability(MSI)testing is performed in cancer patients to determine the indication for chemotherapy with immune checkpoint inhibitors. We report on our scheme to ensure that Lynch syndrome patients are offered the opportunity for genetic counseling and genetic testing. Two hundred and eight cancer patients(107 males and 101 females, 20- 87 years, mean 63.3 years)underwent MSI testing at our hospital between February 2019 and November 2021. From February 2019 to December 2020, the MSI testing was performed with a consent document that included a commentary on Lynch syndrome, and the results were explained only by the attending cancer doctors. Eleven(8.6%)of the 136 cases had MSI-high, but none of them led to a visit to the genetic medicine department. The Genome Center in our hospital, which was operational from April 2020, undertook information sharing by multiple professions and established a system to provide appropriate support to cancer doctors. Consecutively, 72 MSI tests were performed between January and November 2021, and 2 patients(2.8%)with MSI-high(1 with endometrial cancer and 1 with colorectal cancer)were referred to the Department of Clinical Genetics for genetic counseling. Through genetic testing, both were diagnosed with Lynch syndrome, and information on future surveillance and health care for blood relatives was provided.

A rare intrahepatic splenosis mimicking hepatocellular carcinoma: A case report.

Intrahepatic splenosis (IHS) is a rare disease that is considered to result from heterotopic autotransplantation or implantation of splenic tissue after splenic trauma or surgery. A 46-year-old man with a treatment history of a left lateral liver segmentectomy and splenectomy for a road traffic injury 30 years earlier presented to Sakai City Medical Center (Sakai, Japan) with acute abdominal pain in November 2019. Physical examination showed no significant signs, and serum data were normal. Computed tomography revealed a hypodense mass measuring 2.5x1.7 cm in segment 7 of the liver. Gadoxetic acid-enhanced magnetic resonance imaging showed early enhancement in the arterial phase and washout in the delayed phase. Therefore, laparoscopic surgery was performed with a preoperative diagnosis of hepatocellular carcinoma. Pathological examination of the tumor showed IHS. The postoperative course was uneventful, and the patient developed no new abnormal region in the liver during 2 years of follow-up. The present study presented a case of IHS assumed to be hepatocellular carcinoma. IHS should be considered as a differential diagnosis of a liver mass detected years after splenic trauma or surgery, even in cases with imaging patterns suggesting malignancy.

Diabetic nephropathy with marked extra-capillary cell proliferation: a case report.

BACKGROUND: Extra-capillary hypercellularity is a common finding in crescentic glomerulonephritis (GN) and focal segmental glomerulosclerosis (FSGS). In diabetic nephropathy (DN), extra-capillary hypercellularity is often observed as a finding of complications such as IgA nephropathy or microscopic polyangiitis superimposed on DN. However, in rare cases, epithelial cell proliferation may accompany DN. We experienced a case of nodular diabetic glomerulosclerosis with marked extra-capillary hypercellularity and revealed the origin of this atypical lesion using immunostainings. CASE PRESENTATION: A man in his 50 s was admitted to the hospital with nephrotic syndrome, and a renal biopsy was performed. Diffuse nodular lesions and extra-capillary hypercellularity were observed, but the results of serological examination or immunofluorescent assays did not implicate any other crescentic GN. Immunostaining for claudin-1 and nephrin was performed to identify the origin of the extra-capillary lesions. Given the clinical course and pathological findings, a diagnosis of DN-associated extra-capillary cell proliferation was made. CONCLUSIONS: Extra-capillary hypercellularity, which resembles FSGS or crescentic GN, is a rare finding in DN and should therefore be treated with caution. In such cases, co-staining for claudin-1 and nephrin may facilitate the diagnosis of DN.

High Infiltration of CD163-Positive Macrophages in Intratumor Compartment Predicts Poor Prognosis in Patients With Upper Urinary Tract Urothelial Carcinoma and Radical Nephroureterectomy.

OBJECTIVES: To investigate the prognostic value of CD68- and CD163-positive macrophages in patients with upper urinary tract urothelial carcinoma (UTUC). PATIENTS AND METHODS: This retrospective study enrolled 50 patients (34 men and 16 women) with UTUC who received radical nephroureterectomy (RNU). We evaluated the expression of CD68 and CD163 in the intratumor compartment by immunohistochemistry. The Kaplan-Meier method and Cox proportional hazards regression model were used to evaluate overall survival (OS), cancer-specific survival (CSS), recurrence-free survival (RFS), and bladder recurrence-free survival (BRFS). RESULTS: High infiltration of CD163-positive macrophages in patients with UTUC was significantly correlated with worse OS, CSS, and RFS (P < .05 for all). Multivariate analysis showed that high infiltration of CD163-positive macrophages was an independent negative prognostic factor of OS and CSS in patients with UTUC who received RNU. Lymphovascular invasion was an independent negative prognostic factor of RFS, and high infiltration of CD68-positive macrophages was an independent positive prognostic factor of BRFS. CONCLUSION: This study indicated that high infiltration of CD163-positive macrophages in the intratumor compartment might be a useful prognostic marker for survival in patients with UTUC who receive RNU. Further, high infiltration of CD68-positive macrophages in the intratumoral compartment might be a useful prognostic marker for bladder recurrence in these patients.

A case of thoracic SMARCA4-Deficient undifferentiated tumor successfully treated with combination Ipilimumab-Nivolumab.

Thoracic SMARCA4-deficient undifferentiated tumors are rare, with poor prognosis. A 73-year-old man presented to our hospital with dyspnea. Computed tomography-guided biopsy revealed a SMARCA4-deficient undifferentiated tumor. The patient was treated with combination ipilimumab-nivolumab. The tumor reduced in size after two courses.

Squamous Cell Carcinoma of the Scalp Arising in a Site of Synthetic Hair Grafts.

Synthetic hair implantation was developed in the 1970s and initially gained popularity until major cutaneous complications, such as facial swelling, severe dermatitis, recurrent cellulitis, and cicatricial alopecia, became an issue. In particular, the procedure has been suggested to have a possible causal relationship with squamous cell carcinoma (SCC). This article describes the third reported case in the English literature of SCC arising in a site of synthetic hair grafts. The patient was an 80-year-old man with a prominent verrucous tumor in the parietal region; he had undergone synthetic hair implantation for the past 28 years. The pathological diagnosis of SCC was made by dermal punch biopsy, and computed tomography images revealed cranial osteolytic changes, with possible direct dural tumor invasion. Extensive resection of the tumor and reconstruction were performed following downsizing radiotherapy. The protruding tumor was excised with the adjacent portion of the parietal bone. Several synthetic hair grafts were found stuck in the cranium. The defect area was reconstructed with a synthetic bone material and a free latissimus dorsi muscle flap with skin graft. The pathological examination revealed well-differentiated SCC surrounded by numerous synthetic hair grafts inducing inflammatory cell infiltration and severe cicatrizing fibrous changes. The postoperative course was uneventful, and no recurrence or metastasis was observed at 9 months postoperatively.

Disseminated Verruconis gallopava infection in a patient with systemic lupus erythematosus in Japan: A case report, literature review, and autopsy case.

Disseminated Verruconis gallopava infection is often reported in patients with severe immunodeficiency, such as those who have received an organ transplant or have hematological malignancies. The present report describes the first case of disseminated V. gallopava in a patient with systemic lupus erythematosus who used FK506 (Tacrolimus). In this case, ß-D glucan was useful for diagnosis.

Successful laparoscopic conversion surgery for gastric cancer with para-aortic lymph node metastasis after third-line chemotherapy: a case report.

We herein reported a case of advanced gastric cancer (GC) with para-aortic lymph node (PALN) metastases who successful achieved downstaging following systemic chemotherapy and underwent curative laparoscopic conversion surgery. A 74-year-old male patient diagnosed with advanced GC and PALN metastases [cT4N3M1(LYM), stage IVA] was administered chemotherapy and immunotherapy for 28 months. After 27 courses of nivolumab as third-line chemotherapy, PALN enlargement was resolved, for which conversion surgery was planned. Subsequently, laparoscopic distal D2 gastrectomy with sampling para-aortic lymphadenectomy was performed, after which a pathological diagnosis of type V moderately differentiated tubular adenocarcinoma with mucinous adenocarcinoma, stage ypT3 (SS), ly1c, and v0, was established. The pathological proximal and distal tumor margins were negative. One lymph node metastasis was observed (No. 6; 1/25). The sampled lymph nodes were negative (No. 16a1: 0/2). The therapeutic effect was categorized as Grade 1a. The postoperative course was uneventful, with the patient receiving nivolumab to control for potential PALN metastases. Postoperatively, no recurrence was observed over 11 months. Laparoscopic conversion gastrectomy was successfully performed in a patient with advanced GC that was originally unresectable, suggesting that minimally invasive surgery may be a good option for originally unresectable advanced GC that becomes resectable.

[Case of Robot-Assisted Low Anterior Resection with Total Cystectomy for Rectal Cancer Invading the Urinary Bladder/Prostate in Collaboration with Urologists].

We present a case of a 72-year-old man diagnosed with rectal cancer invading the urinary bladder/prostate. Preoperative chemoradiotherapy substantially reduced the tumor size. In collaboration with urologists, robot-assisted low anterior resection with total cystectomy was performed using the da Vinci Xi system. Depending on the surgical situation, the colorectal surgeon and urologist could smoothly and rapidly play the role of a console surgeon. Although the first robot-assisted multi-organ resection of our institution, the surgery was completed safely without any complications. Although the patient developed urinary tract infection postoperatively, he recovered and was discharged after postoperative 23 days. In conclusion, robot-assisted surgery would be useful in pelvic surgery involving multiple departments such as colorectal surgery, urology, and gynecology.

[Successful Treatment with TACE and RFA for a Hepatocellular Carcinoma Case with Lung Metastasis].

We report a hepatocellular carcinoma(HCC)case with lung metastasis that was successfully treated with transarterial chemoembolization(TACE)and percutaneous radiofrequency ablation(RFA). A man in his 60s took right robe liver resection for HCC after TACE for its rupture. Lung metastasis occurred at S1+2 and S6 in the left lung, and an adverse event interrupted standard molecular target therapies. Because extrahepatic metastasis had been seen only in these two locations for a long time, TACE was performed for both metastases. The feeders for both lesions were each intercostal artery, and controlling the drug inflow was necessary to avoid drug influx into the spinal cord branches when S6 metastasis was treated. The viable lesion remained in the S6 lesion, so RFA was added for both lung metastases. 100% tumor necrosis has been observed since the RFA.

[Systemic sarcoidosis developed after chemoradiotherapy for localized thyroid diffuse large B-cell lymphoma].

Localized thyroid diffuse large B-cell lymphoma stage with stage IE according to the Ann Arbor clinical staging system was diagnosed in a 75-year-old woman. The patient was treated with three courses of chemotherapy followed by radiotherapy. Positron emission tomography/computed tomography (PET-CT) using 2-deoxy-2-[F-18] fluoro-D-glucose (FDG) PET-CT was performed two months after chemotherapy showed increased FDG uptakes in systemic lymph nodes and gluteal muscles. Standardized uptake value in the region of interest ranged from 7.1-26.1. Since it seemed too sudden to be a recurrence, a repeat biopsy was performed from inguinal lymph nodes. Histology revealed that there were no malignant lymphoma cells but noncaseous epithelioid granuloma with multinucleated giant cells. Taken together with the findings of bilateral hilar mediastinal lymphadenopathy and elevated serum angiotensin converting enzyme (ACE) levels (32.4 U/l), sarcoidosis secondary to lymphoma was diagnosed in this patient. Subsequently, both FDG uptake and serum ACE gradually improved without any therapy. The present case strongly suggests the importance of a re-biopsy when the clinical course of recurrence is unusual.

Gastric epithelial neoplasm of fundic-gland mucosa lineage: proposal for a new classification in association with gastric adenocarcinoma of fundic-gland type.

BACKGROUND: Gastric adenocarcinoma of fundic-gland type (GA-FG) is a rare variant of gastric neoplasia. However, the etiology, classification, and clinicopathological features of gastric epithelial neoplasm of fundic-gland mucosa lineage (GEN-FGML; generic term of GA-FG related neoplasm) are not fully elucidated. We performed a large, multicenter, retrospective study to establish a new classification and clarify the clinicopathological features of GEN-FGML. METHODS: One hundred GEN-FGML lesions in 94 patients were collected from 35 institutions between 2008 and 2019. We designed a new histopathological classification of GEN-FGML using immunohistochemical analysis and analyzed via clinicopathological, immunohistochemical, and genetic evaluation. RESULTS: GEN-FGML was classified into 3 major types; oxyntic gland adenoma (OGA), GA-FG, and gastric adenocarcinoma of fundic-gland mucosa type (GA-FGM). In addition, GA-FGM was classified into 3 subtypes; Type 1 (organized with exposure type), Type 2 (disorganized with exposure type), and Type 3 (disorganized with non-exposure type). OGA and GA-FG demonstrated low-grade epithelial neoplasm, and GA-FGM should be categorized as an aggressive variant of GEN-FGML that demonstrated high-grade epithelial neoplasm (Type 2 > 1, 3). The frequent presence of GNAS mutation was a characteristic genetic feature of GEN-FGML (7/34, 20.6%; OGA 1/3, 33.3%; GA-FG 3/24, 12.5%; GA-FGM 3/7, 42.9%) in mutation analysis using next-generation sequencing. CONCLUSIONS: We have established a new histopathological classification of GEN-FGML and propose a new lineage of gastric epithelial neoplasm that harbors recurrent GNAS mutation. This classification will be useful to estimate the malignant potential of GEN-FGML and establish an appropriate standard therapeutic approach.

Mesothelioma in Situ of the Spermatic Cord Arising from a Patent Processus Vaginalis: A Case Report.

Mesothelioma is an aggressive tumor originating from mesothelial cells. Mesothelioma of the spermatic cord is a very rare disease, and the most common presentation of this disease is that of aggressive mesothelioma with no description of mesothelioma in situ. We report an extremely rare case of mesothelioma in situ of the spermatic cord arising from a patent processus vaginalis. To our best knowledge, this is the first report of this finding. The identification of a patent processus vaginalis and investigation of single-layered atypical mesothelial cells led to the final diagnosis.

[A Case of Prostate Cancer with High Levels of Prostate-Specific Antigen Undetected by Prostate Biopsy].

A 79 year-old-man visited our hospital with right back pain. Computed tomography suggested external iliac and para-aortic lymphadenopathy. Serum prostate specific antigen (PSA) increased to 335 ng/ml and prostate cancer was highly suspected. We performed transperineal prostate biopsies two times, but could not detect prostate carcinoma cells. Multiparametric magnetic resonance imaging (MRI) indicated no suspicious malignant lesions in the prostate. Laparoscopic biopsy of the right obturator lymph nodes was performed and histological examination, including immunohistochemical staining with PSA, confirmed lymphnode metastasis from prostate cancer. After endocrine therapy was started, serum PSA levels declined and lymph nodes shrunk. In cases of negative prostate biopsies despite high serum PSA levels, aggressive indication for biopsy of metastatic lesion and histological inspection is highly recommended.