A case of rapidly progressing sarcomatous intrahepatic cholangiocarcinoma with suddenly appearing lymph node metastasis

Abstract Background The sarcomatous variant of carcinoma is relatively rare in intrahepatic cholangiocarcinoma (ICC). Sarcomatous ICC (SICC) is associated with a poorer prognosis compared with ICC. SICC is rarely diagnosed before surgery due to non-descriptive findings; it progresses rapidly, result...

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Main Authors: Eisho Kanemitsu, Rei Takahashi, Setsuko Nakanishi, Satoru Sueyoshi, Atsushi Kobayashi, Takao Nishimura, Hiromitsu Nagata
Format: Article
Language:English
Published: Japan Surgical Society 2024-01-01
Series:Surgical Case Reports
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Online Access:https://doi.org/10.1186/s40792-023-01804-7
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author Eisho Kanemitsu
Rei Takahashi
Setsuko Nakanishi
Satoru Sueyoshi
Atsushi Kobayashi
Takao Nishimura
Hiromitsu Nagata
author_facet Eisho Kanemitsu
Rei Takahashi
Setsuko Nakanishi
Satoru Sueyoshi
Atsushi Kobayashi
Takao Nishimura
Hiromitsu Nagata
author_sort Eisho Kanemitsu
collection DOAJ
description Abstract Background The sarcomatous variant of carcinoma is relatively rare in intrahepatic cholangiocarcinoma (ICC). Sarcomatous ICC (SICC) is associated with a poorer prognosis compared with ICC. SICC is rarely diagnosed before surgery due to non-descriptive findings; it progresses rapidly, resulting in miserable prognosis. Here, we report a case of rapidly progressing SICC that showed a clinically significant tumor growth rate. Case presentation A 77-year-old woman who had undergone ileocecal resection for cecal cancer 5 years previously was found to have elevated levels of the tumor marker carbohydrate antigen 19-9. Although an abdominal computed tomography (CT) scan did not detect any liver mass lesions until 3 months before this serum examination, the subsequent CT scan revealed a hypodensity 20 mm mass lesion in the right anterior section. Contrast-enhanced CT and magnetic resonance imaging revealed peripheral enhancement in the arterial-to-equilibrium phase. Fluorodeoxyglucose positron emission tomography revealed uptake in the lesion. None of the imaging modalities showed lymph node swelling or distant metastases. She underwent hepatectomy under the diagnosis of ICC or an atypical metastasis from previous cecal cancer. Although preoperative images showed no suspicious lymph node metastasis 3 weeks prior, the hilar lymph node swelled 3 cm and contained adenocarcinoma. Consequently, the patient underwent right anterior sectionectomy and lymph node dissection of the hepatoduodenal ligament. Histopathological examination revealed that the liver tumor was a poorly differentiated adenocarcinoma with sarcomatous pattern. While the patient received adjuvant gemcitabine and S-1 therapy, lymph node metastasis appeared in the mediastinum 13 months after the surgery. She received gemcitabine + cisplatin + S-1 therapy but died 20 months after surgery. Conclusion SICC and lymph node metastasis clinically appeared within 3 months and 3 weeks, respectively. Suspected ICC that rapidly progresses should be considered SICC and treated with early resection. SICC is often missed in clinical diagnosis and has a poor prognosis, even after curative resection. While an alternative strategy involving preoperative biopsy and neoadjuvant therapy may be beneficial, it should be approached with discretion due to the potential risks of tumor progression and peritoneal dissemination.
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spelling doaj-art-d746ff49771441e4bb1696c1ea69f5232025-08-20T03:57:40ZengJapan Surgical SocietySurgical Case Reports2198-77932024-01-011011910.1186/s40792-023-01804-7A case of rapidly progressing sarcomatous intrahepatic cholangiocarcinoma with suddenly appearing lymph node metastasisEisho Kanemitsu0Rei Takahashi1Setsuko Nakanishi2Satoru Sueyoshi3Atsushi Kobayashi4Takao Nishimura5Hiromitsu Nagata6Department of Surgery, JCHO Yamatokoriyama HospitalGraduate School of Pharmaceutical Science, Doshisha Women’s College of Liberal ArtsDepartment of Radiology, JCHO Yamatokoriyama HospitalDepartment of Radiology, Saiseikai Chuwa HospitalDepartment of Surgery, JCHO Yamatokoriyama HospitalDepartment of Surgery, JCHO Yamatokoriyama HospitalDepartment of Surgery, JCHO Yamatokoriyama HospitalAbstract Background The sarcomatous variant of carcinoma is relatively rare in intrahepatic cholangiocarcinoma (ICC). Sarcomatous ICC (SICC) is associated with a poorer prognosis compared with ICC. SICC is rarely diagnosed before surgery due to non-descriptive findings; it progresses rapidly, resulting in miserable prognosis. Here, we report a case of rapidly progressing SICC that showed a clinically significant tumor growth rate. Case presentation A 77-year-old woman who had undergone ileocecal resection for cecal cancer 5 years previously was found to have elevated levels of the tumor marker carbohydrate antigen 19-9. Although an abdominal computed tomography (CT) scan did not detect any liver mass lesions until 3 months before this serum examination, the subsequent CT scan revealed a hypodensity 20 mm mass lesion in the right anterior section. Contrast-enhanced CT and magnetic resonance imaging revealed peripheral enhancement in the arterial-to-equilibrium phase. Fluorodeoxyglucose positron emission tomography revealed uptake in the lesion. None of the imaging modalities showed lymph node swelling or distant metastases. She underwent hepatectomy under the diagnosis of ICC or an atypical metastasis from previous cecal cancer. Although preoperative images showed no suspicious lymph node metastasis 3 weeks prior, the hilar lymph node swelled 3 cm and contained adenocarcinoma. Consequently, the patient underwent right anterior sectionectomy and lymph node dissection of the hepatoduodenal ligament. Histopathological examination revealed that the liver tumor was a poorly differentiated adenocarcinoma with sarcomatous pattern. While the patient received adjuvant gemcitabine and S-1 therapy, lymph node metastasis appeared in the mediastinum 13 months after the surgery. She received gemcitabine + cisplatin + S-1 therapy but died 20 months after surgery. Conclusion SICC and lymph node metastasis clinically appeared within 3 months and 3 weeks, respectively. Suspected ICC that rapidly progresses should be considered SICC and treated with early resection. SICC is often missed in clinical diagnosis and has a poor prognosis, even after curative resection. While an alternative strategy involving preoperative biopsy and neoadjuvant therapy may be beneficial, it should be approached with discretion due to the potential risks of tumor progression and peritoneal dissemination.https://doi.org/10.1186/s40792-023-01804-7Intrahepatic cholangiocarcinomaSarcomatous patternRapid progressionTumor growth rate
spellingShingle Eisho Kanemitsu
Rei Takahashi
Setsuko Nakanishi
Satoru Sueyoshi
Atsushi Kobayashi
Takao Nishimura
Hiromitsu Nagata
A case of rapidly progressing sarcomatous intrahepatic cholangiocarcinoma with suddenly appearing lymph node metastasis
Surgical Case Reports
Intrahepatic cholangiocarcinoma
Sarcomatous pattern
Rapid progression
Tumor growth rate
title A case of rapidly progressing sarcomatous intrahepatic cholangiocarcinoma with suddenly appearing lymph node metastasis
title_full A case of rapidly progressing sarcomatous intrahepatic cholangiocarcinoma with suddenly appearing lymph node metastasis
title_fullStr A case of rapidly progressing sarcomatous intrahepatic cholangiocarcinoma with suddenly appearing lymph node metastasis
title_full_unstemmed A case of rapidly progressing sarcomatous intrahepatic cholangiocarcinoma with suddenly appearing lymph node metastasis
title_short A case of rapidly progressing sarcomatous intrahepatic cholangiocarcinoma with suddenly appearing lymph node metastasis
title_sort case of rapidly progressing sarcomatous intrahepatic cholangiocarcinoma with suddenly appearing lymph node metastasis
topic Intrahepatic cholangiocarcinoma
Sarcomatous pattern
Rapid progression
Tumor growth rate
url https://doi.org/10.1186/s40792-023-01804-7
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