[size=20px]A 12-year-old girl presents with acute appendicitis. She undergoes an uneventful laparoscopic appendectomy. Pathologic review reveals a 1 cm carcinoid/neuroendocrine tumor at the base of the appendix with perineural invasion but no evidence of lymphatic or vascular invasion. One lymph node was included in the specimen and was negative for tumor. What is the best next step in the management of this patient?

 

A Octreotide scan

 

B Observation alone

 

C Right Hemicolectomy

 

D Ileocecectomy

 

E MRI of the abdomen[/size]

B

B

C only due to the perineural invasion... but would need clarity on the differentiation of the tumour as well

C

B

E

A

B

B

Answer is B

As tumor is small less than 2 cm, there is no evidence of lymphatic or vascular invasion. Involved lymph node negative for tumor so need observation alone.

B

D

E ..... Because of the perneural invasion

Correct answer

B Observation alone

 

Carcinoid tumors are rare neuroendocrine tumors in children. In children and young adults, carcinoid tumors are most often found in the appendix or in the lung. After lymphoma they are the most common tumor of the gastrointestinal tract in children. They are typically found incidentally with a reported incidence of 0.08 to 0.17% in appendiceal specimens. The true incidence of the tumors in children is most likely not known given their indolent course. Due to the rarity of this diagnosis in children, treatment algorithms have commonly followed adult guidelines. Appendectomy is the mainstay of therapy for specimens less than 2 cm. Adult guidelines call for a right hemicolectomy in the following situations:

• size equal to or more than 2 cm in diameter
  
• involvement of the mesoappendix
  
• vascular or lymphatic invasion
  
• raised mitotic index or high proliferative index (Ki‐67)
  

 

 

 

Guidelines also advocate for a partial cecectomy or ileocecal resection (ICR) is indicated for incompletely removed tumors.

 

Recent literature has focused on developing pediatric specific guidelines. These have been driven by the finding that neuroendocrine tumors in children tend to behave differently than in adults with excellent long term outcomes and rare second primary malignancies following complete resection of the tumor. A recent retrospective review of patients with appendiceal carcinoid in France showed that of 114 patients, 85 patients had no criterion for secondary surgery. Twenty-nine patients had criteria for right hemicolectomy but only 10 underwent surgery. None of the resected specimens showed carcinoid tumors. Three patients had positive lymph nodes. At the last follow-up, all patients were alive and disease free. A meta-analysis and systematic review was conducted in 2018 including 958 cases. The authors did note that there was a 28-fold increase in the risk of having a positive lymph node if the tumor size was >2 cm compared with the risk of having a positive lymph node if the tumor size was ≤2 cm. However, they also noted that there was no recurrence or mortality for those with criteria for secondary surgery who were observed after appendectomy alone when compared to those that had secondary surgery. The median follow-up time was 4.8 years. Both studies found that follow-up is not only unnecessary, but its means, whether clinical, radiological or biological, are ineffective.

 

Parikh et al. utilized the Surveillance, Epidemiology, and End Results (SEER) Registry for pediatric appendiceal tumors from 1973 to 2011. They found that 209 patients had an appendiceal tumor, of which 72% were carcinoids. There was no significant difference in 15-year survival between tumor size groups ≥ 2 and < 2 cm (both 100%) and presence or absence of lymph node sampling (96% and 97%; p = 0.833) for all patients with a carcinoid tumor.

 

The patient above has a completely resected appendiceal carcinoid. In children, despite the size, appendectomy appears to be comparable to right hemicolectomy and no further surgery or imaging is indicated at this time.