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This case is unusual in that the primary LAMN and the pulmonary metastasis are present at the time of diagnosis. The sequencing result showed that both tumors harbored the common tumor mutations, KRAS (p.G12D), GNAS (p.R201H), and BRAF (p.R735Q), which indicated that the pulmonary tumor was a metastasis of LAMN.Conclusion Then we performed next-generation sequencing (NGS) to clarify the relationship between the two tumors. The postoperative pathology results revealed pulmonary mucinous adenocarcinoma and LAMN. PET/CT revealed two lesions located in the left lung and appendix.
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Although peritoneal dissemination is common due to tumor rupture and mucinous deposits on the visceral peritoneal surface, distant involvement, such as lung, is rarely seen due to lack of invasiveness.Case PresentationA 70-year-old woman presented to the hospital due to continuously elevated carcinoembryonic antigen (CEA) levels for 10 months without any symptoms. Low-grade appendiceal mucinous neoplasms (LAMNs) are indolent tumors with low-grade cytology. In selected patients, surgical resection of metastatic disease can result in long survival. Systemic metastases did not (P = 0.861).Īfter CRS/HIPEC, systemic metastases occur in a small but clinically relevant number of patients, and the risk increases with incomplete cytoreduction and aggressive histology. At multivariate analysis, PSOGI histological subtypes (P = 0.001), completeness of cytoreduction (P = 0.001), and preoperative systemic chemotherapy (P = 0.020) correlated with poorer survival.
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Seven are disease-free at a median of 27.4 months (range 2.0–110.4). Eight of eleven patients who had curative-intent surgery are presently alive at a median of 52.5 months (range 2.0–112.7). Median OS was 139.0 months (95%CI = 56.6–161.9) for patients who experienced systemic metastases, and 213.8 months (95%CI = 148.7-not reached) for those who did not (P = 0.159). Systemic metastases independently correlated with PSOGI histological subtypes (P = 0.001), and incomplete cytoreduction (P = 0.026). Metastatic disease involved the lung (n = 12), bone (n = 1), liver (n = 4), distant nodes (n = 3), both lung and distant nodes (n = 1). Eleven patients were affected by low-grade PMP, and ten by high-grade PMP. Haematogenous metastases, and non-regional lymph-node involvement were considered as systemic metastases.Īfter a median follow-up of 74.8 months (95% confidence interval = 68.0–94.8), systemic metastases occurred in 21 patients. PMP was graded according to the Peritoneal Surface Oncology Group International (PSOGI) classification. We assessed incidence, impact on prognosis, treatments, and outcomes of systemic metastases after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC).Ī prospective database of 327 patients undergoing CRS/HIPEC for PMP of appendiceal origin was reviewed. The metastasizing potential of pseudomyxoma peritonei (PMP) is largely unknown. Although rare, mucinous neoplasms from PP may involve the thorax. None of the 5 patients developed thoracic lymph nodal metastases. The smear backgrounds contained wispy mucin. Pleural cytologic examination revealed high-grade adenocarcinoma cells singly, in small clusters, and in large spheres. Neither patient had documented injury to the diaphragm. In the 2 patients with high-grade MCP, right-sided pleural effusions developed. The third patient with low-grade MCP had direct extension through the left diaphragm involving the left pleural and pericardial spaces without pulmonary parenchymal involvement. The lack of pleural involvement argued against transdiaphragmatic tumor extension. In 2 cases, 1 or more pulmonary parenchymal metastases of low histologic grade developed. Of 5 patients, 3 had low-grade histologic features in the peritoneum these showed variably proliferative, bland-appearing neoplastic cells arising from low-grade appendiceal mucinous neoplasms. We subsequently examined another patient with pleural invasion. Four patients had pathologically documented pleuropulmonary involvement. In that study, we suggested mucinous carcinoma peritonei (MCP) as the pathologic terminology for all cases of PP. We reviewed 101 patients uniformly treated at our institution for PP of appendiceal origin. Intrathoracic spread in patients with pseudomyxoma peritonei (PP) is rare.