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  • Case Report
  • Open Access

Primary adenocarcinoma of the appendix in a child: A case report

  • 1Email author,
  • 1,
  • 1,
  • 1 and
  • 1
Surgical Case Reports20184:109

https://doi.org/10.1186/s40792-018-0514-4

  • Received: 12 June 2018
  • Accepted: 22 August 2018
  • Published:

Abstract

Background

Primary adenocarcinoma of the appendix is a rare disease in clinical practice. Moreover, primary adenocarcinoma of the appendix in the pediatric age group is even rarer with very little cases being published. Here, we report a case of primary adenocarcinoma of the appendix with local invasion into adjacent organs in a child who was initially diagnosed as having an acute appendicitis.

Case presentation

A 13-year-old girl presented with abdominal pain of 3-month duration. Imaging study showed a mass including the fecalith that occupied her pelvic and right lower abdominal cavity. Drainage of the abscess and appendectomy were performed by the preoperative diagnosis of an acute appendicitis with an appendiceal mass. Postoperative histopathological examinations revealed the appendiceal adenocarcinoma. She then received the whole mass resection, ileocecal resection with lymph node dissection. The masses were tightly adherent with infiltration into the sigmoid colon, uterus, and right ovary. These organs were all dissected, and subsequent sigmoid colostomy was performed. We preserved the left ovary for her fertility. The pathological findings demonstrated negative margins and no lymph node invasions, and final pathological stage was pT4(SI)N0M0, stage II. After the operation, she received the chemotherapy with 6 cycles of 5-fluorouracil (5-FU), leucovorin (LV), and oxaliplatin (mFOLFOX6) and subsequent 6 cycles of simplified LV and 5-FU (sLV5FU2). The patient is doing well till today on follow-up without progression of the disease 5 years after the operation.

Conclusion

Primary adenocarcinoma of the appendix is exceedingly rare in children. In this report, we described one of the youngest primary adenocarcinomas of an appendix case ever reported. When encountering atypical cases of the appendicitis, we should consider the possibility of primary adenocarcinoma of the appendix as it has an extremely poor prognosis and is usually diagnosed in advanced stages.

Keywords

  • Primary adenocarcinoma
  • Appendix
  • Children

Background

Appendiceal cancer is a rare malignancy with an incidence of around 0.1 in 1,000,000, making up only 0.5% of all gastrointestinal malignancies [1]. The tumor itself is not aggressive but has the potential for rupture and spread throughout the peritoneum, which carries a poor prognosis [2]. This cancer is most often diagnosed following appendectomy for suspected acute appendicitis [3].

In the pediatric aged group, the incidence of colorectal cancer is rare compared with that in adults. Although early diagnosis and improved management have resulted in reduced mortality since 1980s [4], the treatment protocol of colorectal cancer in children is still controversial due to very little cases being published.

Here, we report a case of primary adenocarcinoma of the appendix (PAA) with local invasion into adjacent organs in a child who was initially diagnosed as having an acute appendicitis.

Case presentation

A patient

A 13-year-old girl presented with abdominal pain and bloody stool. For 3 months, she had repeated appearance of the abdominal pain and spontaneous disappearance of the symptom. At the beginning, the symptom was not strong and she had not seen a doctor. Her abdominal pain gradually got worse, and she determined to come to our hospital after she often had bloody stool. Imaging study showed a mass including the fecalith that occupied her pelvic and right lower abdominal cavity (Fig. 1). Drainage of the abscess and appendectomy were performed by the preoperative diagnosis of an acute appendicitis with an appendiceal mass (Fig. 2). Although the body of the appendix looked intact as well as the ileum and the cecum, the histopathological examination revealed PAA in the tip of the resected appendix. A radical operation for the residual tumor of PAA in the pelvic cavity was planned.
Fig. 1
Fig. 1

Imaging study showed a mass including the fecalith that occupied her pelvic and right lower abdominal cavity (a coronal, b horizontal)

Fig. 2
Fig. 2

Intraoperative finding: Drainage of the abscess and appendectomy were performed by the preoperative diagnosis of an acute appendicitis with an appendiceal mass. Although the body of the appendix looked intact as well as the ileum and the cecum, the histopathological examination revealed PAA in the tip of the resected appendix

Surgery

She then received the whole mass resection, ileocecal resection with lymph node dissection. At the first operation, we speculate that we resected body of the appendix but left the most of the mass and the tip of the appendix. Therefore, the mass which we resected in the second operation was derived from the tip of the appendix. The masses were tightly adherent with infiltration into the sigmoid colon, uterus, and right ovary. These organs were all dissected, and subsequent sigmoid colostomy was performed (Fig. 3). We preserved the left ovary for her fertility. The pathological findings demonstrated negative margins and no lymph node invasions, and final pathological stage was pT4(SI)N0M0, stage II (TNM-7th edition 2009). Postoperative course was uneventful.
Fig. 3
Fig. 3

Intraoperative finding: The patient received the whole mass resection, ileocecal resection with lymph node dissection, and subsequent sigmoidectomy and adnexectomy

Postoperative treatment

After the operation, she received the chemotherapy with 6 cycles of 5-fluorouracil (5-FU), leucovorin (LV), and oxaliplatin (mFOLFOX6) and subsequent 6 cycles of simplified LV and 5-FU (sLV5FU2). The patient is doing well till today on follow-up without progression of the disease 5 years after the operation. We performed the stoma closure operation 2 years after the tumor resection.

Discussion

Primary adenocarcinoma of the appendix (PAA) is an extremely rare disease in clinical practice [4]. There are no established risk factors for the development of appendix cancer. Malignant appendix tumors most often present with acute appendicitis and are diagnosed incidentally at histologic assessment of the surgical specimen. Appendix cancers may also be asymptomatic and be found incidentally. When symptoms are present, the disease process is often advanced [5].

Colorectal carcinoma in children, although rarely discovered, comprises approximately 1% of pediatric neoplasm [6]. It is also the most common primary gastrointestinal malignancy in children. However, due to the low awareness of the disease, diagnosis is usually delayed until the disease is in the advanced stage, causing prognosis to be extremely poor compared with that of adults [7].

In addition, there is no consensus for the goal of the operation for PAA in children. Generally, functional preservation operation has often been emphasized in pediatric surgery. Therefore, it is not easy to decide the grade of the extension in the radical operation for colorectal cancer such as PAA in children as there is very little reports being published. Furthermore, we have no guideline about the postoperative treatment protocol such as the resume of the chemotherapy.

We reviewed some recent literature which has been published since 2000 and found seven cases of the colorectal adenocarcinoma in children who is under 15 years old (Table 1). The tumors originate from the ascending colon in two cases, transverse colon in one case, descending colon in two cases, and rectum in two cases. Most of the cases received adjuvant chemotherapy. Although three cases had good postoperative courses, the patients in four cases died after the radical operation. The poor prognosis of the primary colorectal carcinoma in children has been re-recognized by this review [812].
Table 1

The reports of primary colorectal carcinoma in children since 2000

Case

Age (years)

Gender

Origin

Pathological results

Operation

Additional treatment

Outcome

1 [8]

13

M

A-colon

Well diff. adenocarcinoma

R-hemicolectomy

No recurrence

2 [9]

12

F

Rectum

Undiff. mucinous adenocarcinoma

Radiotherapy

Die

3 [10]

13

Unknown

D-colon

Adenocarcinoma with multiple metastasis

L-hemicolectomy and splenectomys

Chemotherapy

Die

4 [11]

9

M

Rectum

Mucinous adenocarcinoma

segmental resection

Chemotherapy

No recurrence

5 [11]

12

F

D-colon

Poorly diff. mucinous adenocarcinoma

resection of local tumor

Chemotherapy

No recurrence

6 [12]

13

M

A-colon

Poorly diff. mucinous adenocarcinoma

R-hemicolectomy

Chemotherapy

Die

7 [12]

15

M

T-colon

Mucinous adenocarcinoma

Ileosigmoidostomy bypass

Chemotherapy

Die

M male, F female, A-colon ascending colon, D-colon descending colon, T-colon transverse colon, diff. differentiated, R-hemicolectomy right hemicolectomy, L-hemicolectomy left hemicolectomy

Furthermore, Table 2 shows the reports of primary adenocarcinoma of the appendix in the recent 10 years [1320]. The mean age at presentation for PAA is about 50 years. There is no sex predominance. Most PAAs are well differentiated, are slowly growing, and have pushing rather than infiltrating margin. The traditional treatment is right hemicolectomy. However, in the case of intra-abdominal metastasis, the treatment consists of aggressive debulking followed by chemo-radiotherapy along with it.
Table 2

The reports of primary adenocarcinoma of the appendix in recent 10 years

Case

Age (years)

Gender

Pathological results

Operation

Additional treatment

Outcome

1 [13]

49

M

Well diff. mucinous adenocarcinoma

Tumor resection

Chemotherapy

No recurrence for 15 months

2 [14]

57

M

Mucinous adenocarcinoma

R-hemicolectomy

Chemotherapy

Not mentioned

3 [15]

75

F

Adenocarcinoma arising focally within an appendiceal tubulovillous adenoma

Resection of the sigmoid colon, proximal rectum, and appendix

Not mentioned

Not mentioned

4 [16]

63

F

Mucinous adenocarcinoma

Ileocecal resection

No recurrence for 5 years

5 [17]

42

M

Low-moderate diff. mucinous adenocarcinoma

Resection of local tumor and partial bladder

Chemotherapy

No recurrence for 6 months

6 [18]

35

F

Well diff.mucinous adenocarcinoma

R-hemicolectomy

Chemotherapy

No recurrence

7 [19]

80

M

Signet ring cell adenocarcinoma

R-hemicolectomy

No recurrence

8 [20]

40

F

Mucinous adenocarcinoma

R-hemicolectomy

Not mentioned

M male, F female, diff. differentiated, R-hemicolectomy right hemicolectomy

In this report, we demonstrate a case of PAA with local invasion into adjacent organs in a child who was initially diagnosed as having an acute appendicitis. We performed radical operation and adjuvant chemotherapy. The patient is doing well without progression of the disease after 5 years. Although little has been understood about PAA, we believe our experience may provide new insights into the guideline of the standard treatment for PAA in children.

Conclusions

Primary adenocarcinoma of the appendix is exceedingly rare in children. In this report, we described one of the youngest primary adenocarcinoma of appendix case ever reported. When encountering atypical cases of the appendicitis, we should consider the possibility of primary adenocarcinoma of the appendix as it has an extremely poor prognosis and is usually diagnosed in advanced stages.

Abbreviations

5-FU: 

5-Fluorouracil

LV: 

Leucovorin

PAA: 

Primary adenocarcinoma of the appendix

Declarations

Authors’ contributions

TT acquired the data and drafted the manuscript. HN, GM, KO, and NU performed the operations. All other authors attended the patient postoperatively. All authors read and approved the final manuscript.

Ethics approval and consent to participate

The publication of the present study was undertaken in accordance with the ethical standards of our institution (2018020).

Consent for publication

Informed consent was obtained from the patient and his family for the publication of this report.

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

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Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

Authors’ Affiliations

(1)
Department of Pediatric Surgery, Shizuoka Children’s Hospital, 860 Urushiyama, Aoi-ku, Shizuoka City Shizuoka Prefecture, 420-8660, Japan

References

  1. Murphy EM, Farquharson SM, Moran BJ. Management of an unexpected appendiceal neoplasm. Br J Surg. 2006;93:83–92.View ArticleGoogle Scholar
  2. Marmor S, Portschy PR, Tuttle TM, Virnig BA. The rise in appendiceal cancer incidence: 2000-2009. J Gaastrointest Surg. 2015;19:743–92750.View ArticleGoogle Scholar
  3. Misdraji J. Mucinous epithelial neoplasms of the appendix and pseudomyxoma peritonei. Mod Pathol. 2015;28:S67–79.View ArticlePubMedGoogle Scholar
  4. Torre LA, Bray F, Siegel RL, Ferlay J, Lortet-Tieulent J, Jemal A. Grobal cancer statistics. CA Cancer J Clin. 2015;65:87–108.View ArticlePubMedGoogle Scholar
  5. Hamilton W, Round A, Sharp D, Peters TJ. Clinical features of colorectal cancer before diagnosis: a population-based case-control study. Br J Cancer. 2005;93:399–405.View ArticlePubMedPubMed CentralGoogle Scholar
  6. Salas-Valverde S, Lizano A, Gamboa Y, Vega S, Barrantes M, Santamaria S, Zamora JB. Colon carcinoma in children and adolescents: prognostic factors and outcome - a review of 11 cases. Pediatr Surg Int. 2009;25:1073–6.View ArticlePubMedGoogle Scholar
  7. Hill DA, Furman WL, Billups CA, Riedley SE, Cain AM, Rao BN, Pratt CB, Squnt SL. Colorectal carcinoma in childhood and adolescence: a clinicopathologic review. J Clin Oncol. 2007;25:5808–14.View ArticlePubMedGoogle Scholar
  8. Ahn CH, Kim SC. Two case reports: colorectal adenocarcinoma in children. Clin Case Rep. 2017;96(46):e8074.Google Scholar
  9. Tiwari C, Zadpe A, Rathi P, Shah H. An unusual presentation of rectal carcinoma in a child. Pediatr Gastroenterol Hepathol Nutr. 2018;21:72–5.View ArticleGoogle Scholar
  10. Singer G, Hoellwarth ME. Colorectal carcinomas in children: an institutional experience. Pediatr Surg Int. 2012;28:591–5.View ArticlePubMedGoogle Scholar
  11. Zamir N, Ahmed S, Akhtar J. Mucinous adenocarcinoma of colon. APSP J Case Rep. 2010;1:20.PubMedPubMed CentralGoogle Scholar
  12. Shih HH, Lu CC, Tiao MM, Ko SF, Chuang JH. Adenocarcinoma of the colon in children presenting as abdominal pain: report of two cases. Chang Gung Med J. 2002;25:349–6354.PubMedGoogle Scholar
  13. Dang SC, Sun MM, Liu LL, et al. Giant mucinous adenocarcinoma of the appendix: a case report. J Med Case Rep. 2017;11:206.View ArticlePubMedPubMed CentralGoogle Scholar
  14. Handler M, Anand N, Wei L, et al. Adenocarcinoma of the appendix presenting as a palpable right thigh mass. Radiol Case. 2017;11:20–9.View ArticleGoogle Scholar
  15. Fitzgerald E, Chen L, Guelrud M, et al. Appendical adenocarcinoma presenting as a rectal polyp. Case Rep Gastroenterol. 2016;10:24–9.View ArticlePubMedPubMed CentralGoogle Scholar
  16. Nagata H, Kondo Y, Kawai K, et al. A giant mucinous cystadenocarcinoma of the appendix: a case report and review of the literature. W J Surg Oncol. 2016;14:64.View ArticleGoogle Scholar
  17. Qu R, Jiang Y, Chen S, Dong Q. Primary appendical adenocarcinoma masquerading as primary bladder tumor: a case report and review of literatures. Indian J Surg. 2015;77:S16–8.View ArticleGoogle Scholar
  18. Koizumi N, Murayama Y, Kuriu Y, et al. Incidentally discovered adenocarcinoma in situ of the appendix in a young woman. Case Rep Gastroenterol. 2012;6:726–33.View ArticlePubMedPubMed CentralGoogle Scholar
  19. Fusari M, Sorrentino N, Bottazzi EC, et al. Primary signet ring cell carcinoma of the appendix mimicking acute appendicitis. Case Rep. 2012;1:29.Google Scholar
  20. Behera PK, Rath PK, Panda R, et al. Primary appendiceal mucinous adenocarcinoma. Indian J Surg. 2011;73:146–8.View ArticlePubMedGoogle Scholar

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© The Author(s). 2018

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