Review

. 2016 Mar 5:14:64.

doi: 10.1186/s12957-016-0828-2.

A giant mucinous cystadenocarcinoma of the appendix: a case report and review of the literature

Hiroshi Nagata¹, Yuji Kondo^{2

3}, Kazushige Kawai⁴, Soichiro Ishihara⁵, Shinsuke Kazama⁶, Takako Nirei^{7

8}, Daisuke Soma^{9

10}, Jun Yamada¹¹, Eiji Sunami¹², Joji Kitayama¹³, Yoshiro Kubota¹⁴, Toshiaki Watanabe¹⁵

Affiliations

¹ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. hinagata-tky@umin.ac.jp.
² Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. kondou-ash@umin.org.
³ Department of Surgery, Kikkoman General Hospital, 100 Miyazaki, Noda-city, Chiba, 278-0005, Japan. kondou-ash@umin.org.
⁴ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. kawaik-sur@h.u-tokyo.ac.jp.
⁵ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. ishihara-1su@h.u-tokyo.ac.jp.
⁶ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. kaz-tky@umin.ac.jp.
⁷ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. nirei_takako@yahoo.co.jp.
⁸ Department of Surgery, Kikkoman General Hospital, 100 Miyazaki, Noda-city, Chiba, 278-0005, Japan. nirei_takako@yahoo.co.jp.
⁹ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. dsouma-tky@umin.ac.jp.
¹⁰ Department of Surgery, Kikkoman General Hospital, 100 Miyazaki, Noda-city, Chiba, 278-0005, Japan. dsouma-tky@umin.ac.jp.
¹¹ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. jymda-tky@umin.ac.jp.
¹² Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. eijisunami@gmail.com.
¹³ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. kitayama-tky@umin.ac.jp.
¹⁴ Department of Surgery, Kikkoman General Hospital, 100 Miyazaki, Noda-city, Chiba, 278-0005, Japan. ykubota@mail.kikkoman.co.jp.
¹⁵ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. toshwatanabe@yahoo.co.jp.

PMID: 26945579
PMCID: PMC4779566
DOI: 10.1186/s12957-016-0828-2

Review

A giant mucinous cystadenocarcinoma of the appendix: a case report and review of the literature

Hiroshi Nagata et al. World J Surg Oncol. 2016.

. 2016 Mar 5:14:64.

doi: 10.1186/s12957-016-0828-2.

Authors

Affiliations

¹ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. hinagata-tky@umin.ac.jp.
² Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. kondou-ash@umin.org.
³ Department of Surgery, Kikkoman General Hospital, 100 Miyazaki, Noda-city, Chiba, 278-0005, Japan. kondou-ash@umin.org.
⁴ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. kawaik-sur@h.u-tokyo.ac.jp.
⁵ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. ishihara-1su@h.u-tokyo.ac.jp.
⁶ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. kaz-tky@umin.ac.jp.
⁷ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. nirei_takako@yahoo.co.jp.
⁸ Department of Surgery, Kikkoman General Hospital, 100 Miyazaki, Noda-city, Chiba, 278-0005, Japan. nirei_takako@yahoo.co.jp.
⁹ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. dsouma-tky@umin.ac.jp.
¹⁰ Department of Surgery, Kikkoman General Hospital, 100 Miyazaki, Noda-city, Chiba, 278-0005, Japan. dsouma-tky@umin.ac.jp.
¹¹ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. jymda-tky@umin.ac.jp.
¹² Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. eijisunami@gmail.com.
¹³ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. kitayama-tky@umin.ac.jp.
¹⁴ Department of Surgery, Kikkoman General Hospital, 100 Miyazaki, Noda-city, Chiba, 278-0005, Japan. ykubota@mail.kikkoman.co.jp.
¹⁵ Division of Surgical Oncology, Department of Surgery, Faculty of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. toshwatanabe@yahoo.co.jp.

PMID: 26945579
PMCID: PMC4779566
DOI: 10.1186/s12957-016-0828-2

Abstract

Background: Mucinous cystadenocarcinoma is the second most common etiology of appendiceal mucocele. We report a relatively rare case of a giant appendiceal mucocele caused by mucinous cystadenocarcinoma, which occupied the entire abdomen of an adult woman.

Case presentation: A 63-year-old woman presented with a chief complaint of abdominal distention. Imaging studies showed a giant cystic mass occupying her entire abdomen. Laparotomy confirmed a giant appendiceal mucocele, and the patient underwent ileocecal resection. A mucinous deposit was not found in her abdominal cavity, and the ovaries were grossly normal bilaterally. The pathological diagnosis was mucinous adenocarcinoma with a low-grade mucinous neoplasm that invaded the subserosa. Regional lymph node metastasis was not found. She has had recurrence-free survival for 5 years.

Conclusions: The present case is the largest appendiceal cystadenocarcinoma ever reported. The optimal treatment of an appendiceal neoplasm requires further research based on consensus terminology of an appendiceal mucocele.

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Figures

**Fig. 1**
Contrast-enhanced computed tomography showed a well-encapsulated, unilocular cystic mass which occupied the entire abdomen from just below the diaphragm to the pelvis. *Arrow* showed a dendritic structure protruding into the tumor cavity

**Fig. 2**
Magnetic resonance imaging demonstrated a giant unilocular cystic mass with high signal intensity on T2-weighted images (a) and intermediate signal intensity on T1-weighted images (b)

**Fig. 3**
a A cross-section of the mass showed a cystically dilated lumen with the portion protruding from the appendiceal lumen (*arrow*). b A magnified view of the dendritic structure which appeared as a villous tumor

**Fig. 4**
a Microscopical view of the tumor showed a mucinous adenocarcinoma, and a low-grade mucinous neoplasm is intermingled (hematoxylin and eosin stain, ×20). b Adenocarcinoma is observed to invade the subserosa of the appendix (hematoxylin and eosin stain, ×50)

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A giant mucinous cystadenocarcinoma of the appendix: a case report and review of the literature

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A giant mucinous cystadenocarcinoma of the appendix: a case report and review of the literature

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