• Title/Summary/Keyword: Congenital neuroblastoma

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A Therapeutic Experience of Congenital Bilateral Neuroblastoma (선천성 양측성 신경모세포종의 치료경험 1례)

  • Seo, Yeon Kyong;Kim, Heung Sik;Kwon, Kun Young;Lee, Hee Jung;Koo, Hong Hoe
    • Clinical and Experimental Pediatrics
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    • v.46 no.12
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    • pp.1279-1282
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    • 2003
  • Neuroblastoma is the most common intraabdominal malignant tumor of childhood, with 40% arising from the adrenal gland. Bilateral adrenal involvement from synchronous development or metastatic spread of tumor is rarely seen in children with neuroblastoma. The patient was born with a spontaneous vaginal delivery. Birth weight was 3,200 g. Fetal ultrasonography showed a left adrenal cystic mass. At two weeks of age, she was admitted due to a massive abdominal distension and tachypnea. Percutaneous ultrasonography guided biopsy of the left adrenal mass was performed. The result of the biopsy was neuroblastoma. Vincristine and cyclophosphamide were administerd intravenously and 450 cGy of irradiation was added. Left adrenalectomy was accomplished and postoperative course was uneventful. The patient received cancer chemotherapy with a combination of carboplatin, ifosfamide and VP-16 and is now being followed up for three months. We have experienced a case of congenital bilateral neuroblastoma and report the case with brief review of related literatures.

Mirror Syndrome Resulting from Metastatic Congenital Neuroblastoma to Placenta

  • Park, Sung Hyeon;Namgoong, Jung-Man;Ko, Kyeong Nam;Kim, Chong Jai;Lee, Pil-Ryang;Jung, Euiseok;Lee, Byong Sop;Kim, Ki-Soo;Kim, Ellen Ai-Rhan
    • Perinatology
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    • v.29 no.4
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    • pp.189-194
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    • 2018
  • Congenital neuroblastoma is a rare disease. Placental metastasis is extremely rare and poor prognosis has been reported in neonates. Mirror syndrome could occur in mother with placental metastasis with possibilities of hypertension and edema. We report a case of detection of left suprarenal mass in fetus at $31^{+5}weeks^{\prime}$ gestation. Mother presented with palpitation, edema, headache, and hypertension. Maternal 24 hours urine vanillylmandelic acid (VMA) and normetanephrine (NME) level at 34 weeks' gestation were elevated. Consequently, emergent cesarean section was done. Based on abdominal ultrasonography and whole body magnetic resonance imaging, left adrenal tumor with liver metastasis was suspected. Neuroblastoma was confirmed by liver and placenta biopsy. Chemotherapy was started with Pediatric Oncology Group 9243 at day 7 and changed into Children's Oncology Group 3961 due to cholestasis and poor response during 2nd cycle. Plasma exchange was done for aggravated direct hyperbilirubinemia. The baby expired at 73 days due to multi-organ failure. Maternal symptoms were completely resolved in 2 weeks after delivery along with normalization of the elevated level of VMA and NME. We report a first case of mirror syndrome in Korean mother and fetus resulting from metastatic congenital neuroblastoma to placenta.

An Intra abdominal Pulmonary Sequestration Containing Congenital Cystic Adenomatoid Malformation (Congenital, Cystic Adenomatoid Malformation을 보이는 복강내 폐분리증)

  • Lee, Suk-Koo;Lee, Woo-Yong;Kim, Hyun-Hahk
    • Advances in pediatric surgery
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    • v.2 no.2
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    • pp.138-142
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    • 1996
  • Pulmonary sequestration is a complex anomaly involving the pulmonary parenchymal tissue and its vasculature. It presents as a cystic mass of nonfunctional lung tissue without communication with the tracheobronchial system. Usually, it receives blood supply from anomalous systemic vessels. Therefore, preoperative diagnosis of the pulmonary sequestration is difficult, especially when it is located in the abdomen and combined with congenital cystic adenomatoid malformation(CCAM). We encountered such a mass(CCAM type 2) detected prenatally by ultrasonography. It was a kidney bean shaped, pinkish mass straddling the thorax and abdomen on the right side. Because of the sonographic appearance, neuroblastoma was diagnosed preoperatively. The mass was completely extirpated without difficulty.

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Clinical Features and Surgical Outcome of Congenital Neuroblastoma of Adrenal Gland (부신 기원의 선천성 신경모세포종의 치료 경험)

  • Park, Hyo-Jun;Moon, Suk-Bae;Seo, Jeong-Meen;Lee, Suk-Koo
    • Advances in pediatric surgery
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    • v.15 no.1
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    • pp.38-43
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    • 2009
  • With the widespread use of the obstetrical ultrasound, identification of a fetal suprarenal mass becomes more common. Most of these masses prove to be congenital neuroblastomas (CNB) postnatally. However, the diagnosis is often confused with other benign lesions and the post-natal management remains controversial. The medical records of 13 patients that underwent primary surgical excision for an antenatally detected adrenal CNB, between January 1995 and April 2009, were reviewed retrospectively. The clinical, radiological, surgical, and pathological data on the suprarenal mass were collected. Staging evaluation was performed after histological confirmation of the CNB. Most of the CNBs were stage I (N=11), with 1 stage IV and 1 stage IV-S. Four patients (3 stage I and 1 stage IV-S) had N-myc gene amplification. The stage I patients were cured by surgery alone, and stage IV patients underwent 9 cycles of adjuvant chemotherapy and currently have no evidence of disease after 39 months of follow-up. The patient with stage IV-S is currently receiving chemotherapy. There were no post-operative complications. For early diagnosis and treatment, surgical excision should be considered as the primary therapy for an adrenal CNB detected before birth. The surgery can be safely performed during the neonatal period and provides a cure in most cases. Surgical diagnosis and treatment of CNB is recommended in neonatal period.

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