Mini-Invasive Surgery for Adrenocortical Carcinoma in Children: Is It Safe?
Thomas Gelas,1 Didier Frappaz,2 Pascale Berlier,3 Pierre D. Mouriquand,1 and Pierre-Yves Mure1
1 Pediatric Surgery Department, Hôpital Femme Mère Enfant, Hospices Civils de Lyon and Claude Bernard University, France; 2 Institute of Pediatric Hematology-Oncology, Lyon, France; 3 Pediatric Endocrinology Department, Hôpital Femme Mère Enfant, Hospices Civils de Lyon and Claude Bernard University, France
Mini-invasive surgery for adrenocortical carcinoma in children: is it safe? Laparoscopy in adreno- cortical carcinoma is controversial due to high risk of local recurrence in case of per-operative spillage. We wanted to report the case of a successful laparoscopic transperitoneal adrenalectomy performed in a 3-year-old girl presenting a 5.5-cm adrenocortical carcinoma.
Keywords endocrinology, pediatric oncology, surgery
DEAR EDITORS
We want to report a case of laparoscopic adrenalectomy (LA) performed in a 3-year-old girl who presented a 5-cm adrenocortical carcinoma (ACC), associated with clinical signs of virilization. Adrenalectomy by minimal invasive surgery is used worldwide since the 1990s but experience in children remains limited. However, in a large multicentric study [1], it has been showed that LA can be used regardless of age and should be considered as the preferred approach for the majority of adrenal lesions in children.
CASE REPORT
The patient was a 35-month-old girl with signs of virilization (clitoral hypertro- phy, pubic hair growth, and acne). The laboratory tests showed a high DHEAS (>10,000 µg/dL), normal cortisolemia (289 nmol/L), and ACTH (18.2 ng/L). The radiological examination revealed a right-sided adrenal mass measuring 53 mm. Sted. There were no signs of local or general invasion. The father’s family was known to have genetically proven Li-Fraumeni syndrome (p53 mutation) with early malignancies
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tribution
Received 27 January 2013; accepted 27 January 2013; published online 27 February 2013. Presented at the International Pediatric Endosurgery Group’s 21st Annual Congress, San Diego, California, March 6-10, 2012.
Address correspondence to Dr Thomas Gelas, Pediatric Surgery Department, Hôpital Femme Mère Enfant, Hospices Civils de Lyon and Claude Bernard University, Boulevard Pinel, Lyon-Bron 69500, France. E-mail: thomas.gelas@chu-lyon.fr
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developed in siblings. The diagnosis of ACC was suspected and a surgical treatment was proposed. Initial laparoscopic access was through the umbilicus using an open technique. No local invasion or peritoneal carcinomatosis was detected. The right adrenal vein was controlled and divided. Then, the adrenal gland was dissected peripherally. Duration of the surgery was 155 minutes and postoperative course was uneventful. Histopathological examination revealed a 55 x 50 mm ACC weighing 53 g. The surgical margins were free of tumor and it was classified T2, stage I. The patient is doing well 56 months after surgery without any signs of residual tumor or metastasis.
DISCUSSION
ACC are representing 0.2% of all carcinomas in children [2]. The median age at presen- tation is around 4 years old with a predominance of girls presenting signs of virilization in more than 90% of the cases. The 5-year event-free survival is around 54% but some factors are associated with better survival: age <4 years old, virilization alone and stage I (see Table 1) [3]. Patients of this group have 5-year survival estimates greater than 85% [4]. International Pediatric Endosurgery Group guidelines [5] states that LA is techni- cally feasible in children and can be performed in selected patients for benign and malignant tumors. Indeed, in large reviews [1,6], safety of LA has been demonstrated with a low conversion rate. In 2004, Porpiglia et al. [7] reported that LA was a feasible option for ACC, if the principles of oncological surgery are respected. Conversely, rup- ture of tumor’s capsule with resultant tumor spillage is frequent (approximately 20% of initial resections), and therefore, laparoscopy in patients suspected to have ACC is controversial. Some adult series have demonstrated an increased rate of peritoneal carcinomatosis [8] or positive surgical margins [9] after laparoscopic approach.
Mini-invasive surgery for ACC is feasible and allows a complete visual inspection of the abdomen and radical treatment with minimal morbidity, short hospital stay, and excellent cosmetic result. However, cautious preoperative radiological and complete excision is mandatory. Experienced pediatric laparoscopic surgeon in adrenalectomy field seems to be mandatory and conversion to open surgery is necessary in case of local invasion, vascular thrombosis, or when the dissection cannot be as accurate as in conventional operation.
| Stage | Definition | Treatment |
|---|---|---|
| I | Completely resected, small tumors (<100g and <200cm3) with normal postoperative hormone levels | Surgery alone |
| II | Completely resected, large tumors (≥100g and ≥200cm3) with normal postoperative hormone levels | Surgery RPLN dissection |
| III | Unresectable, gross or microscopic residual disease Tumor spillage Patients with Stage I and II tumors who fail to normalize hormone levels after surgery Patient with retroperitoneal lymph node involvement | Chemotherapy Surgery + RPLN dissection |
| IV | Presence of distant metastases | Chemotherapy surgery + RPLN dissection |
Abbreviations: RPLN, retroperitoneal lymph node; chemotherapy, mitotane, cisplatin, etoposide, and doxorubicin.
REFERENCES
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[3] Ribeiro RC, Pinto EM, Zambetti GP, Rodriguez-Galindo C. l. The international pediatric adrenocorti- cal tumor registry initiative: contributions to clinical, biological, and treatment advances in pediatric adrenocortical tumors. Mol Cell Endocrinol. 2012:31;351(1):37-43.
[4] Michalkiewicz E, Sandrini R, Figueiredo B, et al. Clinical and outcome characteristics of children with adrenocortical tumors: a report from the international pediatric adrenocortical tumor registry. J Clin Oncol. 2004:22;838-845.
[5] International Pediatric Endosurgery Group. IPEG guidelines for the surgical treatment of adrenal masses in children. J Laparoendosc Adv Surg Tech A. 2010 Mar;20(2):vii-ix.
[6] Pampaloni F, Valeri A, Mattei R, et al. Experience with laparoscopic adrenalectomy in children. Chir Ital. 2006;58(1):45-54.
[7] Porpiglia F, Fiori C, Daffara F, et al. Retrospective evaluation of the outcome of open versus laparo- scopic adrenalectomy for stage I and II adrenocortical cancer. Eur Urol. 2010 May;57(5):873-878.
[8] Leboulleux S, Deandreis D, Al Ghuzlan A, et al. Adrenocortical carcinoma: is the surgical approach a risk factor of peritoneal carcinomatosis? Eur J Endocrinol. 2010 Jun;162(6):1147-1153.
[9] Miller BS, Ammori JB, Gauger PG, et al. Laparoscopic resection is inappropriate in patients with known or suspected adrenocortical carcinoma. World J Surg. 2010 Jun;34(6):1380-1385.
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