Laparoscopic Resection of Large Adrenal Ganglioneuroma

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Abstract

We report on a 23-year-old woman with a right adrenal tumor 13 cm in diameter who was treated by laparoscopy. The patient was asymptomatic, and the tumor was incidentally diagnosed on abdominal ultrasonography. A subsequent computed tomography (CT) of the abdomen confirmed a 12×7×8-cm homogenous mass of the right adrenal. Magnetic resonance imaging (MRI) showed a solid mass measuring 13×7×7.5 cm arising from the right adrenal. Laparoscopic complete excision of the mass was accomplished through a transabdominal lateral approach. The postoperative period was uneventful, and the patient was discharged on the second postoperative day. Histology was consistent with an adrenal ganglioneuroma. Two years later, there is no evidence of recurrence on abdominal CT scan.

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Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation.

Gael Lonergan, Cornelia M Schwab, Eric S Suarez … (2002)

Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma are tumors of the sympathetic nervous system that arise from primitive sympathogonia and are referred to collectively as neuroblastic tumors. They arise wherever sympathetic tissue exists and may be seen in the neck, posterior mediastinum, adrenal gland, retroperitoneum, and pelvis. The three tumors differ in their degree of cellular and extracellular maturation; immature tumors tend to be aggressive and occur in younger patients (median age, just under 2 years), whereas mature tumors occur in older children (median age, approximately 7 years) and tend to behave in a benign fashion. The most benign tumor is the ganglioneuroma, which is composed of gangliocytes and mature stroma. Ganglioneuroblastoma is composed of both mature gangliocytes and immature neuroblasts and has intermediate malignant potential. Neuroblastoma is the most immature, undifferentiated, and malignant tumor of the three. Neuroblastoma, however, may have a relatively benign course, even when metastatic. Thus, these neuroblastic tumors vary widely in their biologic behavior. Features such as DNA content, tumor proto-oncogenes, and catecholamine synthesis influence prognosis, and their presence or absence aids in categorizing patients as high, intermediate, or low risk. Treatment consists of surgery and, usually, chemotherapy. Despite recent advances in treatment, including bone marrow transplantation, neuroblastoma remains a relatively lethal tumor, accounting for 10% of pediatric cancers but 15% of cancer deaths in children. Copyright RSNA, 2002

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Neurogenic tumors in the abdomen: tumor types and imaging characteristics.

Sung Eun Rha, Jae Young Byun, Seung Eun Jung … (2003)

There is a broad spectrum of neurogenic tumors that involve the abdomen. These tumors can be classified as those of (a) ganglion cell origin (ganglioneuromas, ganglioneuroblastomas, neuroblastomas), (b) paraganglionic system origin (pheochromocytomas, paragangliomas), and (c) nerve sheath origin (neurilemmomas, neurofibromas, neurofibromatosis, malignant nerve sheath tumors). Abdominal neurogenic tumors are most commonly located in the retroperitoneum, especially in the paraspinal areas and adrenal glands. All of these tumors except neuroblastomas and ganglioneuroblastomas are seen in adult patients. Abdominal neurogenic tumor commonly manifests radiologically as a well-defined, smooth or lobulated mass. Calcification may be seen in all types of neurogenic tumors. The diagnosis of abdominal neurogenic tumor is suggested by the imaging appearance of the lesion, including its location, shape, and internal architecture. Benign and malignant neurogenic tumors are difficult to differentiate unless distant metastatic foci are seen. For malignant tumors, imaging modalities other than computed tomography (CT) and magnetic resonance (MR) imaging may be necessary for staging. However, because most neurogenic tumors in adults are benign, CT and MR imaging can be used to develop a differential diagnosis and help determine the immediate local extent of tumor. Copyright RSNA, 2003.

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Metabolic activity and clinical features of primary ganglioneuromas.

B Geoerger, K. Scheidhauer, B Hero … (2001)

Ganglioneuroma (GN) is considered by most to be a benign tumor of neural crest origin. It may evolve from differentiating neuroblastoma or may be diagnosed as primary ganglioneuroma. The rarity of this tumor and the lack of understanding of its biology often lead to inaccurate diagnosis and treatment. The authors analyzed clinical features and biologic behavior of primary ganglioneuroma in 49 patients who were registered with but were not part of the national neuroblastoma trials. Data included age and symptoms at diagnosis, gender, tumor localization and size, (123)I-metaiodobenzylguanidine (mIBG) scintigraphy, secretion of catecholamines, histology, treatment, and outcome, whenever available. Patients with primary ganglioneuroma were significantly older than patients with neuroblastoma. Median age at diagnosis was 79 months compared with 16 months (P < 0.0001). Ganglioneuroma were equally distributed between males and females (1.13:1). A preference of thoracic (41.5%) and abdominal, nonadrenal tumors (37.5%) was observed compared with adrenal GN (21%). At diagnosis, thoracic tumors appeared larger than nonthoracic ones. Local lymph node metastases occurred in two patients. One ganglioneuroma had metastasized to soft tissues. (123)I-mIBG scintigraphy detected mIBG uptake at tumor site in 57% of the GN tumors. Levels of catecholamines in plasma and/or urine were increased in 39%. Slight immaturity of ganglion cells was observed in 93% of all ganglioneuroma tumors. None of the 22 tumors analyzed exhibited MYCN gene alterations. Although 12 patients had macroscopic residuals, no tumor progression or recurrence was observed in a median follow-up of 25 months. Ganglioneuroma may present with metabolic activity such as increased secretion of catecholamines and/or mIBG uptake. There are no specific diagnostic signs or symptoms discriminating ganglioneuroma and neuroblastoma tumors. Therefore, ganglioneuroma requires tissue investigation for diagnosis. Prognosis after surgical resection without further therapy seems to be excellent. Copyright 2001 American Cancer Society.

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Author and article information

Contributors

Third Department of Surgery, Athens General Hospital, “G. Gennimatas,” Greece.

Department of Endocrinology, Athens General Hospital, “G. Gennimatas,” Greece.

Department of Pathology, Athens General Hospital, “G. Gennimatas,” Greece.

Department of Endocrinology, Athens General Hospital, “G. Gennimatas,” Greece.

Department of Internal Medicine, Athens General Hospital, “G. Gennimatas,” Greece.

Third Department of Surgery, Athens General Hospital, “G. Gennimatas,” Greece.

Journal

Journal ID (nlm-ta): JSLS

Journal ID (iso-abbrev): JSLS

Journal ID (hwp): jsls

Journal ID (pmc): jsls

Journal ID (publisher-id): JSLS

Title: JSLS : Journal of the Society of Laparoendoscopic Surgeons

Publisher: Society of Laparoendoscopic Surgeons (Miami, FL )

ISSN (Print): 1086-8089

ISSN (Electronic): 1938-3797

Publication date (Print): Oct-Dec 2007

Publication date PMC-release: Oct-Dec 2007

Volume: 11

Issue: 4

Pages: 487-492

Affiliations

Third Department of Surgery, Athens General Hospital, “G. Gennimatas,” Greece.

Department of Endocrinology, Athens General Hospital, “G. Gennimatas,” Greece.

Department of Pathology, Athens General Hospital, “G. Gennimatas,” Greece.

Department of Endocrinology, Athens General Hospital, “G. Gennimatas,” Greece.

Department of Internal Medicine, Athens General Hospital, “G. Gennimatas,” Greece.

Third Department of Surgery, Athens General Hospital, “G. Gennimatas,” Greece.

Author notes

Address reprint requests to: G. N. Zografos, MD, 10 K. OURANI str., Athens, 15237, Greece. Telephone: +302106801360, E-mail: gnzografos@ 123456yahoo.com

Article

PMC ID: 3015852

PubMed ID: 18237516

SO-VID: da17925e-d92b-45b1-b11c-e315fdaa8a82

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This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives License ( http://creativecommons.org/licenses/by-nc-nd/3.0/), which permits for noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited and is not altered in any way.

Laparoscopic Resection of Large Adrenal Ganglioneuroma

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Most cited references 29

Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation.

Neurogenic tumors in the abdomen: tumor types and imaging characteristics.

Metabolic activity and clinical features of primary ganglioneuromas.

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