Q:

9/4/2012
42 year old male with x-linked agammaglobulinemia has recently been diagnosed with rectal adenocarcinoma. Can TNM classification be used in these patients as they do not have significant lymph nodes? Thanks.

A:

Thank you for your inquiry.

In x-linked agammaglobulinemia, the tonsils, adenoids, and peripheral lymph nodes are not absent, but are very small. This is due to the absence of germinal centers.

Therefore, since these lymph nodes exist, at least on a theoretical basis, staging could be used. I cannot, however, answer your question with complete confidence in this regard because I do not think that we know all of the forces attracting cancer cells to the lymph nodes, and perhaps there is a diminished attraction in the absence of follicles, but I am unaware of any such effect and could find no mention of it in the literature.

This issue has been approached in patients with x-linked agammaglobulinemia who have developed gastrointestinal carcinoma . There is a report of a patient with x-linked agammaglobulinemia who had a polypoid lesion near the pylorus. Upper abdominal lymph nodes were resected for investigation, and histological examination of the polypoid specimens revealed a highly differentiated gastric adenocarcinoma. Thirty-two abdominal lymph nodes revealed typical architectural derangements, but no metastasis were noted (1).

Using this case as a prototype, obviously lymph nodes can be assessed histologically, and a search for metastasis is feasible. Thus, as noted above, barring unknown forces which might diminish lymph node metastasis, due to the fact that lymph nodes are still present in this disorder, staging should still be possible.

Thank you again for your inquiry and we hope this response is helpful to you.

Reference:
1. Lackmann GM, et al. A teenager with x-linked agammaglobulinemia and vitamin B12 deficiency anemia. Journal of Pediatric Gastroenterology and Nutrition 2005; 41(3):360-362.

Sincerely,
Phil Lieberman, M.D.

AAAAI - American Academy of Allergy Asthma & Immunology