Primary Appendix Lymphoma : Case Report and Review of the Literature

Appendectomy is the most frequent emergent surgical procedure in childhood. Carcinomas are the most frequent neoplasms in the appendix, other neoplasms such as carcinoid tumors and lymphomas may seen less frequently. Primary tumors of the appendix are very rare. We report a patient presented with acute appendicitis diagnosed with B-cell lymphoma after pathological examination.


IntRoduCtIon
Appendectomy is the most frequent emergency surgical procedure in childhood [1].Pathological examination of the appendectomy specimens usually demonstrate diagnosis of acute appendicitis [2].Appendiceal neoplasms may present with appendicitis [3].Carcinomas are the most frequent neoplasms in the appendix, other neoplasms such as carcinoid tumors and lymphomas may seen less frequently [2].Primary tumors of the appendix are very rare [4].We report a patient presented with acute appendicitis is diagnosed with lymphoma after pathological observation.This is a rare form of non-Hodgkin's (NHL) lymphoma.

Literature review
PubMed Central (US National Library of Medicine, 8600 Rockville Pike, Bethesda, Maryland, USA) and Google Scholar (Google Inc., 1600 Amphitheatre Parkway, Mountain View, California, USA) searches plus references of these articles revealed cases of appendicitis, lymphoma.All cases were reviewed and patient details (age, sex, pathologic diagnosis, clinical appendicitis, surgical methods, and accompanying diseases) were noted, SPSS 16.0 was used for statistical analysis (mean value, standart deviation, minimum and maximum value).lower quadrant and scoliosis.The patient was operated with the initial diagnosis of acute appendicitis.There were plenty of serous fluid in peritoneal cavity.The appendix and cecum were widely thickened (Figure 1).Appendectomy was performed.Postoperative serum level of LDH 324 IU/L (115-257 IU/L) was slightly higher than normal.Peripheral blood smear and bone marrow smear were normal.Postoperative course was uneventful.Oral feeding was started on the postoperative day.Pathological examination of appendix revealed centroblastic subtype of diffuse large B-cell lymphoma (Figure 2).The patient was discharged on the fifth postoperative day and he is on chemotherapy in the pediatric oncology department.

CASE REPoRt
An 11-year-old boy was admitted to our clinic with the complaint of abdominal pain and nausea.Intermittent abdominal pain, which was aggravated in last day, was learned from the history.Physical examination revealed right lower quadrant tenderness and guarding.The patient had skin rash secondary to psoriasis on his lower extremities.In laboratory assessment, white blood cell count and C-reactive protein levels were 7900/ µL (5000-11800 µL) and 23.5 mg / L (0-8mg/L) respectively.Blood biochemistry parameters were in normal range.Abdominal X-ray demonstrated air-fluid levels in the right
Colon lymphoma is usually seen in the elderly and associated with inflammatory bowel disease, HIV/ AIDS and immunosuppressive treatment [10].In this study, which examined the appendix lymphoma cases, there was only one case of AIDS and three cases were on immunosuppresive agents.Also, our patient had psoriasis.Psoriasis is a chronic inflammatory disease and it might be at increased risk of cancer due to chronic inflammation and immunosuppressive drugs.A study that is systematic literature review showed that the risk of non-Hodgkin lymphoma appears to be slightly increased in psoriasis.
The T-cell lymphoma is extremely rare.It is associated with immune compromised conditions.The gastrointestinal tract is the most common site for extranodal involvement of non-Hodgkin's lymphoma; appendiceal lymphoma is exceedingly rare [11].Hodgkin's diseases, was found 2% in appendix lymphomas (Figure 4).
In literature, the most common surgical method is appendectomy (Figure 5).On the other hand, extensive excision may be needed.What should be the surgical method for appendiceal malignancies?Our recommendation is not to make a wide excision without a pathological diagnosis.

ConFLICt oF IntERESt
The authors declare that there are no conflicts of interest in this study.

Figure 1 .
Figure 1.The Appendix was widely thickened.Figure 2. Diffuse large B-cell lymphoma.Centroblastic variant.The tumour cells have a polymorphic and polylobated appearance.

Figure 2 .
Figure 1.The Appendix was widely thickened.Figure 2. Diffuse large B-cell lymphoma.Centroblastic variant.The tumour cells have a polymorphic and polylobated appearance.

7 Table 1 .
Appendiceal lymphoma clinical data in the literature

Table 1 .
Appendiceal lymphoma clinical data in the literature