Robotic surgeries in pediatric patients: an early experience in a single center

Purpose@#Robotic surgery (RS) has the advantages of 3-dimensional view, optical magnification, motional scaling, and improved ergonomics and degree of freedom. Although RS has widely been performed on pediatric patients lately, there are still numerous restrictions and ambiguous indications. The purpose of this study was to report our early experience with RS on pediatric patients at a single center. @*Methods@#Electronic medical records of patients who underwent RS with the da Vinci Xi surgical platform (Intuitive Surgical, Inc.) in Seoul National University Children Hospital from November 2019 to August 2021 were reviewed retrospectively. The median follow-up was 21.0 months (range, 12.3–31.8 months). An online survey was conducted to investigate satisfaction with robotic surgical scars. @*Results@#Fifty-four patients underwent robotic surgeries (median age at operation, 11.1 years [range, 0.1–17.8 years]). In our hospital, patients had 20 different kinds of robotic surgeries, including choledochal cyst excision with hepaticojejunostomy, ovarian mass excision, and others. Median operation time and console time were 157.5 minutes (range, 45–505 minutes) and 40 minutes (range, 11–360 minutes), respectively. All cases were done without conversion into open or laparoscopic methods. Postoperative complications were found in 5 patients. According to an online survey, over half of patients (60.9%) answered that they felt satisfied with scars. @*Conclusion@#Our early experience demonstrated the safety and feasibility of RS in children with a range of diagnoses and complicated procedures. With more experience, RS could be an alternative to traditional open or laparoscopic operations in pediatric patients. Further studies are needed to clarify indications of pediatric RS.

Clinical course in children with equivocal appendicitis on computed tomography: a retrospective cohort study

Purpose@#Appendectomy is one of the most common surgeries in children. Although various radiological examinations are performed, they do not always reveal a definitive diagnosis of appendicitis. This study aimed to investigate the clinical course of equivocal appendicitis, identify the factors associated with appendectomy, and suggest appropriate management for these patients. @*Methods@#Patients younger than 19 years who visited Seoul National University Bundang Hospital with a differential diagnosis of appendicitis from January 2013 to December 2017 were included. All participants conducted ‘appendiceal CT’ with a scoring scale of 1–5. The higher the score, the higher the likelihood of a radiologic diagnosis of appendicitis. We defined the appendicitis CT score of 2–4 as equivocal appendicitis (n = 143). Medical records were reviewed retrospectively for demographics, further examination as abdominal ultrasonography, and appendectomy status (yes or no). The mean follow-up period was 15.6 ± 71 days. @*Results@#Equivocal appendicitis accounted for 16.7%. Additional ultrasonography test was performed in 24.5% (35 of 143). In total, 34 patients (23.8%) underwent appendectomy. Among the patients with appendiceal CT scores 2, 3, and 4, 4.9%, 50.0%, and 87.5% underwent appendectomy, respectively. Higher WBC count, higher appendicitis CT score, and readmission were significantly associated with appendectomy in patients with equivocal appendicitis. @*Conclusion@#Higher appendicitis CT score and WBC level were positively associated with appendectomy. Careful observation can be a treatment option in appendicitis CT score 2 or 3 groups. Appendectomy is the first-line treatment for patients with appendicitis score 4. Additional ultrasonography test is advisable to determine treatment modality for equivocal appendicitis.

Comparison of long-term biliary complications between open and laparoscopic choledochal cyst excision in children

Purpose@#Cyst excision with hepaticojejunostomy has been the classic procedure for treating choledochal cysts, and the use of laparoscopic treatment has been favored recently. The purpose of this study was to compare the long-term biliary complication of laparoscopic operation with open surgery for choledochal cyst presenting in children. @*Methods@#A retrospective study comparing the laparoscopic and open procedures was performed in 185 patients with choledochal cyst in a single children’s hospital. There were 109 patients who were operated with open surgery, and 76 patients operated with laparoscopic surgery. The primary outcome was long-term biliary complications and the secondary outcome included operative time, intraoperative transfusion, length of hospital stay, and other late postoperative complications. @*Results@#In the patient’s demographics, there was no significant difference between the 2 groups. Notably, it was shown that the operative time was longer in the laparoscopic group. The number of patients requiring blood transfusion intraoperatively was lower in the laparoscopic group. It was noted that the hospital stay was not statistically different. The duration to resumption of diet and duration of drainage were longer in the laparoscopic group. Biliary complications were shown to be significantly higher in the open group. The risk factor for long-term biliary complications was noted with the intraoperative transfusion. @*Conclusion@#The use of a laparoscopic choledochal cyst excision with hepaticojejunostomy is a safe and feasible technique in a young patient. The long-term biliary complication was lower compared to open surgery, rendering this a good option for pediatric patients.

Analysis of Associated Anomalies in Anorectal Malformation: Major and Minor Anomalies

Background@#Fifty to sixty percent of patients with anorectal malformation (ARM) have at least one associated anomaly (AA). We determined the incidence of AA with the subtypes of ARM classified in accordance with the Krickenbeck classification and analyzed differences in the incidence rates of major and minor AAs according to organ system. @*Methods@#From January 1999 to May 2017, we retrospectively analyzed congenital anomalies in patients who underwent an anoplasty for ARM at our institution. The AAs were divided into nine organ systems. To analyze the difference in the incidence of AAs, we calculated odds ratios (ORs) using cases of perineal fistula as the base group. @*Results@#Of the 460 patients, 256 (55.7%) were male, 299 (65%) had at least one anomaly, and 274 (59.6%) had major AAs. According to organ system, AAs were most common in the genitourinary (28%), cardiovascular (25%), and spinal/vertebral systems (22.6%). Major AA was most common in the cardiovascular (23%) and spinal/vertebral and genitourinary systems (19.3%). According to ARM subtype, AAs were common in the order of cloaca (93.9%), rectovaginal fistula (85.7%), and recto-bladder neck fistula (85%). For the incidence of AAs, cloaca (OR, 15.7) and recto-bladder neck fistula (OR, 5.74) showed significantly higher ORs. In the analysis of major AAs, the cloaca (OR, 19.77) showed the highest OR, followed by no fistula (OR, 4.78) and recto-bladder neck fistula (OR, 3.83). @*Conclusion@#A considerable number of patients with ARM had AAs. Our data are useful for predicting AAs in patients with ARM.

Analysis of Associated Anomalies in Anorectal Malformation: Major and Minor Anomalies

Background@#Fifty to sixty percent of patients with anorectal malformation (ARM) have at least one associated anomaly (AA). We determined the incidence of AA with the subtypes of ARM classified in accordance with the Krickenbeck classification and analyzed differences in the incidence rates of major and minor AAs according to organ system. @*Methods@#From January 1999 to May 2017, we retrospectively analyzed congenital anomalies in patients who underwent an anoplasty for ARM at our institution. The AAs were divided into nine organ systems. To analyze the difference in the incidence of AAs, we calculated odds ratios (ORs) using cases of perineal fistula as the base group. @*Results@#Of the 460 patients, 256 (55.7%) were male, 299 (65%) had at least one anomaly, and 274 (59.6%) had major AAs. According to organ system, AAs were most common in the genitourinary (28%), cardiovascular (25%), and spinal/vertebral systems (22.6%). Major AA was most common in the cardiovascular (23%) and spinal/vertebral and genitourinary systems (19.3%). According to ARM subtype, AAs were common in the order of cloaca (93.9%), rectovaginal fistula (85.7%), and recto-bladder neck fistula (85%). For the incidence of AAs, cloaca (OR, 15.7) and recto-bladder neck fistula (OR, 5.74) showed significantly higher ORs. In the analysis of major AAs, the cloaca (OR, 19.77) showed the highest OR, followed by no fistula (OR, 4.78) and recto-bladder neck fistula (OR, 3.83). @*Conclusion@#A considerable number of patients with ARM had AAs. Our data are useful for predicting AAs in patients with ARM.

Pediatric Adenocarcinoma in Korea: A Multicenter Study / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#Adenocarcinoma is an extremely rare malignancy in the pediatric population. Research regarding pediatric adenocarcinoma is very rare in Korea. This study aimed to investigate the clinical features of pediatric adenocarcinomas of various primary organ sites in Korea. @*Materials and Methods@#Pediatric patients under 18 years, diagnosed with adenocarcinoma of various sites between January 1995 and December 2016, were included. We retrospectively reviewed patient and tumor characteristics and calculated survival estimates, reported as 5-year survival rate and 95% confidence interval. @*Results@#Of 80 patients (median age, 15 years; range, 10 to 17 years), 37 (46.3%) were men, and 24 (30%) had a family history of cancer or underlying disease relevant to malignancy. The cancer locations were the colon and rectum (n=32), ovaries (n=18), stomach (n=15), lung (n=4), small bowel (n=1), and other sites (n=10). Totally, 54.8% patients (42/77) had stage 3 or 4 disease. The median follow-up period was 2.0 years (range, 0 to 20.4). The 5-year overall survival estimate for all patients, and for those with stomach, colorectal, ovarian, and other cancer sites were 57.9%±11.5%, 58.2%±25.7%, 41.5%±18.2%, 87.5%±16.2%, and 64.0%±34.4%, respectively. The 5-year survival rate differed significantly between categories of adenocarcinomas into gastrointestinal (GI) (44.7%) and non-GI adenocarcinomas (78.8%) (p=0.007). The 5-year survival rate also differed significantly according to carcinoembryonic antigen level (69.3% in 3 ng/mL; p < 0.001). @*Conclusion@#In pediatric patients, adenocarcinomas arise from various organs and are often diagnosed at advanced stages. Large, prospective studies for their accurate clinical characteristics and prognostic factors are needed.

Transverse Colon Volvulus around the Gastrostomy Tube in a Pediatric Situs Inversus Patient / 소아외과

An 18-year-old male patient with cerebral palsy and scoliokyphosis came to the emergency department with abdominal distension and vomiting. He was a situs inversus patient with a feeding gastrostomy tube. Sigmoid volvulus was initially suspected, so rectal tube insertion and endoscopic decompression were attempted, but failed. So he went through explorative laparotomy, and transverse colonic adhesion and twisting around the gastrostomy tube and gastric wall was identified. Adhesiolysis and resection with redundant transverse colon and end-to-end colocolic anastomosis was performed. He discharged with symptom free. Suspecting transverse colonic volvulus is important when the patient has anatomical anomalies and feeding gastrostomy tube. Timely diagnosis with proper radiologic imaging should be made. Surgical resection of the redundant colon is needed for successful management of transverse colonic volvulus.

Is Preoperative Ultrasonography Necessary in Pediatric Inguinal Hernia? / 소아외과

PURPOSE@#Preoperative ultrasonography (USG) in pediatric inguinal hernia has controversy. In this study, we analyzed the cases of pediatric inguinal hernia with/without preoperative USG and discussed whether USG is necessary.@*METHODS@#We reviewed medical records of 1,441 patients who underwent inguinal hernia repair in Seoul National University Children's Hospital between January 2011 and August 2016 retrospectively.@*RESULTS@#Male were 69.3% and age at operation was 37.8±36.5 months old. There were 150 patients (10.4%) performed USG preoperatively. The department ordered to perform USG included department of surgery (n=71), emergency medicine (n=42), pediatrics (n=26), urology (n=10) and outside hospital (n=1). The reasons of performing USG included evaluation for hernia laterality (n=82), incarceration (n=28), testis (n=15), request of parents (n=14), scrotal mass (n=6) and incidentally found during evaluation for another disease (n=5). Excepting 5 cases of incidental finding, of 145 cases with USG, 12 (8.3%) cases changed the surgical plan; change to bilateral repair from unilateral repair (n=5), emergency operation due to incarceration (n=4) which include 1 salpingo-oophorectomy, 1 open abdomen surgery and 2 hernia repair after reduction of ovary, change to co-operation of orchiopexy (n=2) and change to laparoscopic surgery from open surgery due to herniation of both ovaries into one inguinal canal (n=1). In group without USG (n=1,291), 5 patients (0.4%) had unexpected problems during operation; 2 co-operation of orchiopexy because of transverse testicular ectopia (n=1) and right undescended testis (n=1), 2 hypertrophy of major labia and 1 retroperitoneal lymphangioma at inguinal area misdiagnosed as inguinal hernia.@*CONCLUSION@#It was difficult to interpret the meaning of preoperative USG because not all patients had performed it. In this study, 10.4% of patients performed USG and 8.3% of them changed surgical plan. About 0.4% of patients without preoperative USG would have benefited from it for surgery if they had performed it. Since the percentage is too low, it is unreasonable to conclude that USG has diagnostic utility in inguinal hernia in this study.

Transverse Colon Volvulus around the Gastrostomy Tube in a Pediatric Situs Inversus Patient

An 18-year-old male patient with cerebral palsy and scoliokyphosis came to the emergency department with abdominal distension and vomiting. He was a situs inversus patient with a feeding gastrostomy tube. Sigmoid volvulus was initially suspected, so rectal tube insertion and endoscopic decompression were attempted, but failed. So he went through explorative laparotomy, and transverse colonic adhesion and twisting around the gastrostomy tube and gastric wall was identified. Adhesiolysis and resection with redundant transverse colon and end-to-end colocolic anastomosis was performed. He discharged with symptom free. Suspecting transverse colonic volvulus is important when the patient has anatomical anomalies and feeding gastrostomy tube. Timely diagnosis with proper radiologic imaging should be made. Surgical resection of the redundant colon is needed for successful management of transverse colonic volvulus.

Is Preoperative Ultrasonography Necessary in Pediatric Inguinal Hernia?

PURPOSE: Preoperative ultrasonography (USG) in pediatric inguinal hernia has controversy. In this study, we analyzed the cases of pediatric inguinal hernia with/without preoperative USG and discussed whether USG is necessary. METHODS: We reviewed medical records of 1,441 patients who underwent inguinal hernia repair in Seoul National University Children's Hospital between January 2011 and August 2016 retrospectively. RESULTS: Male were 69.3% and age at operation was 37.8±36.5 months old. There were 150 patients (10.4%) performed USG preoperatively. The department ordered to perform USG included department of surgery (n=71), emergency medicine (n=42), pediatrics (n=26), urology (n=10) and outside hospital (n=1). The reasons of performing USG included evaluation for hernia laterality (n=82), incarceration (n=28), testis (n=15), request of parents (n=14), scrotal mass (n=6) and incidentally found during evaluation for another disease (n=5). Excepting 5 cases of incidental finding, of 145 cases with USG, 12 (8.3%) cases changed the surgical plan; change to bilateral repair from unilateral repair (n=5), emergency operation due to incarceration (n=4) which include 1 salpingo-oophorectomy, 1 open abdomen surgery and 2 hernia repair after reduction of ovary, change to co-operation of orchiopexy (n=2) and change to laparoscopic surgery from open surgery due to herniation of both ovaries into one inguinal canal (n=1). In group without USG (n=1,291), 5 patients (0.4%) had unexpected problems during operation; 2 co-operation of orchiopexy because of transverse testicular ectopia (n=1) and right undescended testis (n=1), 2 hypertrophy of major labia and 1 retroperitoneal lymphangioma at inguinal area misdiagnosed as inguinal hernia. CONCLUSION: It was difficult to interpret the meaning of preoperative USG because not all patients had performed it. In this study, 10.4% of patients performed USG and 8.3% of them changed surgical plan. About 0.4% of patients without preoperative USG would have benefited from it for surgery if they had performed it. Since the percentage is too low, it is unreasonable to conclude that USG has diagnostic utility in inguinal hernia in this study.

Esophageal Atresia with Bronchogenic Cyst / 소아외과

A baby was diagnosed with esophageal atresia (EA) with tracheoesophageal fistula (TEF) on the next day after birth, and end-to-end anastomosis of esophagus with TEF ligation was performed. The distance between proximal and distal esophageal pouch was checked as 3 vertebral body lengths and a 1 cm-sized bronchogenic cyst (BC) was identified near carina on the right side, just below the proximal esophageal pouch. This case report described the baby who have a BC was located between the both esophageal pouch and a longer esophageal gap than usual EA with distal TEF.

A Case of Extramedullary Hematopoiesis Associated with Congenital Dyserythropoietic Anemia / 소아외과

It has been known that extramedullary hematopoiesis occurring after birth can be developed in various diseases, and it is often found in hematologic diseases. Among these, congenital dyserythropoietic anemia is a rare disease characterized with increase of ineffective hematopoiesis and morphological abnormalities of erythroblasts. In congenital dyserythropoietic anemia, extramedullary hematopoiesis is very rare and only a few cases have been reported. Although treatment is not required if there is no symptom in extramedullary hematopoiesis, surgery or radiation therapy is effective in case that there is symptom or unresponsive anemia despite blood transfusion. This case report is about surgical treatment for extramedullary hematopoiesis in 23-year-old patients diagnosed of congenital dyserythropoietic anemia.

Early Experience of Pediatric Thoracoscopic Surgery Performed by a Pediatric Surgeon / 소아외과

PURPOSE: Minimally invasive surgery (MIS) in abdomen and thorax has been widely accepted for pediatric diseases. Thoracoscopic surgery has the advantage of less pain, better cosmetic outcomes and less musculoskeletal sequelae in comparison to open surgery. We would like to share our initial experience with thoracoscopic surgery performed by one pediatric surgeon. METHODS: We performed a retrospective review of patients who underwent thoracoscopic surgery by one pediatric surgeon between April 2010 and August 2017 in Department of Pediatric Surgery, Seoul National University Children's Hospital. RESULTS: There were totally 18 cases; 8 cases for esophageal atresia, 3 cases for congenital diaphragm hernia, 2 cases for diaphragm eventration, 2 cases for esophageal duplication cyst, 2 cases for pleural mass and 1 case for esophageal bronchus. At the operation, median age was 9.5 months (range, 0-259 months) and median body weight was 9.4 kg (range, 1.9-49.4 kg). Median operative time was 157.5 minutes (range, 45-335 minutes). There was no case of open conversion and 2 cases of minor leakage at anastomosis site in case of esophageal atresia. Median follow-up month was 5 months (range, 0-87 months). During follow-up, 4 cases of esophageal atresia showed anastomosis site narrowing and average 2.5 times (range, 1-5 times) of esophageal balloon dilatation was done. CONCLUSION: We performed thoracoscopic surgery in case of esophageal, diaphragm disease and pleural mass. Thoracoscopic surgery can be an effective and feasible option of treatment for well-selected pediatric patients of intra-thoracic disease including esophagus, diaphragm and mediastinum disease.

Soft Tissue Hemangioendothelioma in Children / 소아외과

PURPOSE: Soft tissue hemangioendothelioma (STHE) is a rare vascular tumor, which has a similar prognosis to borderline malignancy. The disease is poorly understood in pediatric cases because of its low incidence; therefore, we investigated treatment strategies for STHE in children. METHODS: We retrospectively analyzed 8 patients with STHE, who were pathologically confirmed between January 1995 and June 2015. The median duration of follow-up was 72 months. RESULTS: Five were male and the median age at the time of surgery was 1.2 years. Six patients presented with a palpable mass, and 2 patients experienced facial paralysis. The median tumor size was 4.0 cm. The following tumor locations were observed head (2 patients), neck (2 patients), chest wall (1 patient), sacrococcyx (1 patient), upper limb (1 patient), and lower limb (1 patient). The patients underwent either microscopic complete resection (R0) (3 patients), macroscopic complete resection (R1) (2 patients), or macroscopic incomplete resection (R2) (3 patients). After histopathological examination, 6 patients were diagnosed with kaposiform hemangioendothelioma (HE), one with retiform HE, and one with epithelioid HE. Postoperative sequelae occurred as gait disturbance, hearing impairment, and vocal cord palsy. Tumor recurrence or regrowth occurred in 4 patients. These patients underwent reoperation and IFN therapy; however, in the patient with epithelioid HE, metastasis to the scalp occurred after these therapies. The patient with the head tumor who underwent R2 resection, underwent resection three more times, but died 11 months after the first surgery. CONCLUSION: When treating STHE in children, R0 resection should be first considered, but recurrence and metastasis should be monitored depending on the size, pathology, and location of the lesion. When major sequelae are expected, function-preserving surgery could be considered, depending on tumor location, size, and nearby organs.

Nutritional Support for Pediatric Patients with Biliary Atresia

Biliary atresia (BA) is a major cause of extrahepatic biliary obstruction in children. Malnutrition is a significant clinical problem in children with BA. BA may induce the malabsorption of fat and fat-soluble vitamins, resulting in cholestasis and an impaired nutritional status. For the treatment of BA, it is most important to reconstruct the bile flow as early as possible by performing a Kasai portoenterostomy. After the Kasai operation, growth and nutrition are restored, but to follow normal growth and development, it is necessary to evaluate the nutritional status and support. Therefore, the purpose of nutritional support in children with BA is to normalize growth and development, prevent further liver damage and deterioration of the patient's nutritional status, avoid vitamin and mineral deficiencies, and improve the quality of life of patients.

A Case Report of Gardner-Associated Fibroma in 19-Month-Old Girl / 소아외과

Gardner-associated fibroma (GAF) is a benign lesion of soft tissue which has recently been described and is exceedingly rare in children. GAF is associated with adenomatous polyposis coli gene mutation, familial adenomatous polyposis and desmoid. We report a case of patient with soft tissue tumor on her lower back which was turned out to be GAF. The patient was a 19-month-old female who visited out-patient clinic with palpable mass on her lower back and we performed surgical excision. The tumor was located at subcutaneous and we excised the tumor including surrounding soft tissue. She discharged without any complication on surgery day. The pathologic report showed dense collagenous tissue with spindle cell and adipose tissue, suggestive of GAF. We are planning to check gene study and to perform endoscopy and abdominal ultrasonography for at the age of 4.

Anterior Anorectocolonic Tubular Duplication Presenting as Rectovestibular Fistula in an Infant / 소아외과

Anorectal duplications account for only 5% of gastrointestinal duplications, and cases with involvement of the anal canal are much rarer. Nearly all anorectal duplications are posterior to the rectum; duplications located anterior to the normal rectum are highly unusual, and only a few cases have been reported. We report the case of an anterior anorectocolonic duplication presenting as a rectovaginal fistula in a 2-month-old infant. After diagnosis, the duplication was excised completely without further intestinal complications.

Thyroid Cancer in Pediatric Age: A Single Institution Experience / 소아외과

PURPOSE: Thyroid cancer is a rare disease in pediatric population, but its incidence rate is increasing. The aim of this report is to present a single institution experience of pediatric thyroid cancer and to identify clinical features, predisposing factors, and postoperative course of pediatric thyroid cancer. METHODS: We retrospectively reviewed 35 pediatric patients who underwent operation due to thyroid cancer at Seoul National University Children's Hospital between May 1997 and January 2017. The median follow-up period was 70 months (range, 5–238 months). RESULTS: The mean age at operation was 12.0±5.91 years and 27 patients were female. The underlying conditions in patients included history of chemoradiotherapy for previous other malignancies (n=4), hypothyroidism (n=3), history of chemotherapy (n=2), family history of thyroid cancer (n=1) and history of radiation therapy (n=1). The initial symptoms were palpable neck mass (n=21) and incidental findings (n=11). Total thyroidectomy (n=30) or unilateral lobectomy (n=5) were performed. There were 15 postoperative complications including transient hypocalcemia in 14 patients and Horner's syndrome in 1 patient. The most common pathologic cell type was papillary thyroid cancer (n=29). Extrathyroid extension and lymph node invasion were found in 25 patients and 27 patients, respectively. Thirteen patients showed multifocality. During follow-up period, 5 patients underwent additional operation because of tumor recurrence in lymph nodes. Lung metastasis was detected in 3 patients at the time of diagnosis and in 3 patients during follow-up period. The mortality rate was zero and mean disease-free survival was 83.7±47.9 months. CONCLUSION: Pediatric thyroid cancer has lower mortality rate and recurrence rate as seen in this study despite the advanced stage at diagnosis. A thorough follow-up of patients with an underlying condition such as history of chemoradiotherapy and understanding new pediatric guideline can be helpful to maximize patients' survival and prognosis.

Isolated cystic duct cyst with associated stones in a 4-month-old boy

Isolated cystic duct cysts are rare entities, with few cases having been reported. We present the case of a 4-month-old male patient presenting with abdominal pain and vomiting. Ultrasonography and magnetic resonance cholangiopancreatography revealed an isolated cystic duct cyst with associated stones. The patient underwent open cholecystectomy with complete cyst excision and cystic duct transection; there were no postoperative complications. While lesions like the one described herein are extremely rare, they should be included as a separate category in classifications of choledochal cysts.

A Case of Pediatric Nodal Marginal Zone Lymphoma / 소아외과

Lymph node enlargement is a common finding in children suggesting normal or benign. Palpable nodes which are large, hard or fixed must be examined carefully to rule out malignant diseases. In this case, a 15-year-old boy presented to our hospital to inspect the palpable mass at his post-auricular area being found 2 months ago. It was diagnosed as nodal marginal zone lymphoma (NMZL) through excisional biopsy and immunohistochemistry. NMZL is very rare, especially in children and young adults, but occurs locally in most cases with a good prognosis compared to adults. We described a rare case of NMZL diagnosed in adolescent.