Humans ; Lung Neoplasms/pathology* ; Neoplasms, Multiple Primary/pathology*

As the utilization of computed tomography in lung cancer screening becomes more prevalent in the post-pandemic era, the incidence of multiple primary lung cancer (MPLC) has surged in various countries and regions. Despite the continued application of advanced histologic and sequencing technologies in this research field, the differentiation between MPLC and intrapulmonary metastasis (IM) remains challenging. In recent years, the specific mechanisms of genetic and environmental factors in MPLC have gradually come to light. Lobectomy still predominates in the treatment of MPLC, but the observation that tumor-specific sublobar resection has not detrimentally impacted survival appears to be a viable option. With the evolution of paradigms, the amalgamated treatment, primarily surgical, is an emerging trend. Among these, stereotactic ablative radiotherapy (SABR) and lung ablation techniques have emerged as efficacious treatments for early unresectable tumors and control of residual lesions. Furthermore, targeted therapies for driver-positive mutations and immunotherapy have demonstrated promising outcomes in the postoperative adjuvant phase. In this manuscript, we intend to provide an overview of the management of MPLC based on the latest discoveries.
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Humans ; Lung Neoplasms/therapy* ; Early Detection of Cancer ; Lung/surgery* ; Treatment Outcome ; Radiosurgery/methods* ; Neoplasms, Multiple Primary/pathology*

Female ; Humans ; Melanoma/surgery* ; Neoplasms, Multiple Primary/pathology* ; Ovarian Neoplasms/pathology* ; Skin Neoplasms ; Teratoma/surgery*

BACKGROUND/AIMS: Unexpected diagnosis of synchronous second primary cancers (SPC) complicates physicians' decision-making because clinical details of squamous esophageal cancer (EC) patients with SPC have been limited. We evaluated clinical features and treatment outcomes of patients with synchronous SPC detected during the initial staging of squamous EC. METHODS: We identified a total of 317 consecutive patients diagnosed with squamous EC. Relevant clinical and cancer-specific information were reviewed retrospectively. RESULTS: EC patients with synchronous SPC were identified in 21 patients (6.6%). There were significant differences in median age (70 years vs. 63 years, p = 0.01), serum albumin level (3.3 g/dL vs. 3.9 g/dL, p < 0.01) and body mass index (20.4 kg/m2 vs. 22.8 kg/m2, p = 0.01) between EC patients with and without SPC. Head and neck, lung and gastric cancers accounted for 18.2%, 22.7%, and 18.2% of SPC, respectively. Positron emission tomography-computed tomography (PET-CT) detected four cases (18.2%) of SPC that were missed on CT. Management plans were altered in 13 of 21 patients (61.9%) with detected SPC. Curative esophagectomy was attempted in 28.6% of EC patients with SPC (vs. 59.1% of patients without SPC; p = 0.006). EC patients with SPC had significantly lower 5-year survival than patients without SPC (10.6% vs. 36.7%, p = 0.008). CONCLUSIONS: Synchronous SPC were found in 6.6% of squamous EC patients, and PET-CT contributed substantially to the detection of synchronous SPC. EC patients with SPC had poor survival due to challenges of providing stage-appropriate treatment.
Aged ; Carcinoma, Squamous Cell/diagnostic imaging/mortality/*pathology/therapy ; Esophageal Neoplasms/diagnostic imaging/mortality/*pathology/therapy ; Esophagectomy ; Esophagoscopy ; Female ; Humans ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Neoplasm Staging ; Neoplasms, Multiple Primary/diagnostic imaging/mortality/*pathology/therapy ; Positron Emission Tomography Computed Tomography ; Predictive Value of Tests ; Retrospective Studies ; Risk Factors ; Time Factors ; Treatment Outcome

BACKGROUND: Little is known of the mutation and tumor spectrum of Korean patients with Li-Fraumeni syndrome (LFS). Owing to the rarity of LFS, few cases have been reported in Korea thus far. This study aimed to retrospectively review the mutations and clinical characteristics of Korean patients with LFS. METHODS: TP53 mutation was screened in 89 unrelated individuals at the Samsung Medical Center in Korea, from 2004 to 2015. Six additional mutation carriers were obtained from the literature. RESULTS: We identified nine different mutations in 14 Korean patients (male to female ratio=0.3:1). Two such frameshift mutations (p.Pro98Leufs*25, p.Pro27Leufs*17) were novel. The recurrent mutations were located at codons 31 (n=2; p.Val31Ile), 175 (n=3; p.Arg175His), and 273 (n=4; p.Arg273His and p.Arg273Cys). The median age at the first tumor onset was 25 yr. Ten patients (71%) developed multiple primary tumors. A diverse spectrum of tumors was observed, including breast (n=6), osteosarcoma (n=4), brain (n=4), leukemia (n=2), stomach (n=2), thyroid (n=2), lung (n=2), skin (n=2), bladder (n=1), nasal cavity cancer (n=1), and adrenocortical carcinoma (n=1). CONCLUSIONS: There was considerable heterogeneity in the TP53 mutations and tumor spectrum in Korean patients with LFS. Our results suggest shared and different LFS characteristics between Caucasian and Korean patients. This is the first report on the mutation spectrum and clinical characteristics from the largest series of Korean LFS patients.
Adolescent ; Adult ; Asian Continental Ancestry Group/genetics ; Base Sequence ; Child ; Child, Preschool ; Codon ; Female ; Frameshift Mutation ; Germ-Line Mutation ; Humans ; Infant ; Li-Fraumeni Syndrome/*genetics/pathology ; Male ; Middle Aged ; Neoplasms, Multiple Primary ; Polymorphism, Genetic ; Republic of Korea ; Retrospective Studies ; Tumor Suppressor Protein p53/*genetics ; Young Adult

The records of 63 high-risk neuroblastoma patients with bone marrow (BM) tumors at diagnosis were retrospectively reviewed. All patients received nine cycles of induction chemotherapy followed by tandem high-dose chemotherapy and autologous stem cell transplantation (HDCT/auto-SCT). Follow-up BM examination was performed every three cycles during induction chemotherapy and every three months for one year after the second HDCT/auto-SCT. BM tumor cells persisted in 48.4%, 37.7%, 23.3%, and 20.4% of patients after three, six, and nine cycles of induction chemotherapy and three months after the second HDCT/auto-SCT, respectively. There was no difference in progression-free survival (PFS) rate between patients with persistent BM tumor and those without during the induction treatment. However, after tandem HDCT/auto-SCT, the PFS rate was worse in patients with persistent BM tumor than in those without (probability of 5-yr PFS 14.7% +/- 13.4% vs. 64.2% +/- 8.3%, P = 0.009). Persistent BM tumor during induction treatment is not associated with a worse prognosis when intensive tandem HDCT/auto-SCT is given as consolidation treatment. However, persistent BM tumor after tandem HDCT/auto-SCT is associated with a worse prognosis. Therefore, further treatment might be needed in patients with persistent BM tumor after tandem HDCT/auto-SCT.
Adolescent ; Antineoplastic Combined Chemotherapy Protocols/administration & dosage ; Bone Marrow Neoplasms/pathology/*secondary/*therapy ; Child ; Child, Preschool ; Combined Modality Therapy/methods ; Female ; Humans ; Induction Chemotherapy/methods ; Infant ; Infant, Newborn ; Male ; Neoplasms, Multiple Primary/pathology/*therapy ; Neuroblastoma/*pathology/*therapy ; Prognosis ; Retrospective Studies ; Risk Factors ; Stem Cell Transplantation/*methods ; Treatment Outcome ; Young Adult

Cases of pancreatic ductal adenocarcinoma with multiple masses accompanying underlying pancreatic diseases, such as intraductal papillary mucinous neoplasm, have been reported. However, synchronous invasion without underlying pancreatic disease is very rare. A 61-year-old female with abdominal discomfort and jaundice was admitted to our hospital. Abdominal computed tomography (CT) revealed cancer of the pancreatic head with direct invasion of the duodenal loop and common bile duct. However, positron emission tomography-CT showed an increased standardized uptake value (SUV) in the pancreatic head and tail. We performed endoscopic ultrasound-guided fine needle biopsy (EUS-FNB) for the histopathologic diagnosis of the pancreatic head and the evaluation of the increased SUV in the tail portion of the pancreas, as the characteristics of these lesions could affect the extent of surgery. As a result, pancreatic ductal adenocarcinomas were confirmed by both cytologic and histologic analyses. In addition, immunohistochemical analysis of the biopsy specimens was positive for carcinoembryonic antigen and p53 in both masses. The two masses were ultimately diagnosed as pancreatic ductal adenocarcinoma, stage IIB, based on EUS-FNB and imaging studies. In conclusion, the entire pancreas must be evaluated in a patient with a pancreatic mass to detect the rare but possible presence of synchronous pancreatic ductal adenocarcinoma. Additionally, EUS-FNB can provide pathologic confirmation in a single procedure.
Adenocarcinoma, Mucinous/pathology/*ultrasonography ; Carcinoma, Pancreatic Ductal/pathology/*ultrasonography ; *Endoscopic Ultrasound-Guided Fine Needle Aspiration ; Female ; Humans ; Middle Aged ; Neoplasms, Multiple Primary/pathology/*ultrasonography ; Pancreas/pathology ; Pancreatic Neoplasms/pathology/*ultrasonography

Carcinoma ; pathology ; Endometrial Neoplasms ; pathology ; Female ; Humans ; Neoplasms, Multiple Primary ; pathology ; Ovarian Neoplasms ; pathology ; Sex Cord-Gonadal Stromal Tumors ; pathology

Angiomyolipoma ; pathology ; Humans ; Kidney Neoplasms ; pathology ; Lung Neoplasms ; pathology ; Lymphangioleiomyomatosis ; pathology ; Neoplasms, Multiple Primary ; pathology

OBJECTIVETo analyze the imaging characteristics of hepatic epithelioid hemangioendothelioma (EHE) and their correlation with histopathological findings.

METHODSCT and MRI imaging and histopathological characteristics of five patients with hepatic EHE were retrospectively reviewed and the correlation of their imaging characteristics with pathological findings was analyzed.

RESULTSA total of 92 lesions were found in the 5 patients, all presenting with multiple nodules. All the 92 lesions were located within a 2-cm zone heneath the hepatic capsule, i.e., the shortest distance from the horder of lesions to the hepatic capsule. 28 of the 92 lesions showed the "capsular retraction" sign. 36 lesions were found in three patients receiving MRI. 77.8% of the 36 lesions demonstrated the "halo" sign on a fat-suppression T2- weighted image, while 91.7% after contrast enhancement. A peripheral dark rim was found in 91.7% of the lesions on a fat-suppression T2-weighted image. In addition, 36.1% of the lesions showed slight internal or edge enhancement at the hepatohiliary phase. In the two patients receiving CT examination, 7 of 56 lesions demonstrated the "halo" sign.

CONCLUSIONSHepatic EHE may manifest as nodular lesions with predilection of peripheral subcapsular growth and nodular confluence, together with "halo" sign and " capsular retraction". The peripheral dark rim on a fat-suppression T2-weighted image and slight enhancement at the hepatobiliary phase can help to improve the accuracy of diagnosis and differential diagnosis of this hepatic tumor. MRI is superior to CT imaging to denict their intra-lesional characteristics.

Hemangioendothelioma, Epithelioid ; diagnosis ; diagnostic imaging ; pathology ; Humans ; Liver Neoplasms ; diagnosis ; diagnostic imaging ; pathology ; Magnetic Resonance Imaging ; Neoplasms, Multiple Primary ; diagnosis ; diagnostic imaging ; pathology ; Physical Examination ; Retrospective Studies ; Tomography, X-Ray Computed