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Prevesical genetic paraganglioma and left adrenal mass: laparoscopic resection

Hereditary paraganglioma-pheochromocytoma syndromes are caused by genetic mutations, which lead to the development of multiple neuroendocrine tumors and paraganglioma tumors in the adrenal glands. We report the case of a young patient aged 13 who has been followed up routinely for a familial mutation of the SDHB gene. In this routine follow-up examination, an excessive plasma normetanephrine and norepinephrine secretion is evidenced. A genetic paraganglioma is diagnosed. Imaging studies are conducted to identify its location. A prevesical fixation is demonstrated by both the PET-scan and the MIBG scintigraphy. In this video, a laparoscopic resection of both lesions is demonstrated.

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Prevesical genetic paraganglioma and left adrenal mass: laparoscopic resection

作者群

D Mutter, J Marescaux

摘要

Hereditary paraganglioma-pheochromocytoma syndromes are caused by genetic mutations, which lead to the development of multiple neuroendocrine tumors and paraganglioma tumors in the adrenal glands. We report the case of a young patient aged 13 who has been followed up routinely for a familial mutation of the SDHB gene. In this routine follow-up examination, an excessive plasma normetanephrine and norepinephrine secretion is evidenced. A genetic paraganglioma is diagnosed. Imaging studies are conducted to identify its location. A prevesical fixation is demonstrated by both the PET-scan and the MIBG scintigraphy. In this video, a laparoscopic resection of both lesions is demonstrated.

關鍵字

Prevesical, genetic, paraganglioma, left, adrenal node, laparoscopic resection

媒體類型

手術影片

期間

10'06''

刊物

2010-09

普通的

最愛

Favorites Media

音訊

數位出版

WeBSurg.com, Sept 2010;10(09).
URL: http://www.websurg.com/doi-vd01en2968.htm

Prevesical genetic paraganglioma and left adrenal mass: laparoscopic resection

1. Clinical case presentation 00'08''

We report the case of a young patient aged 13 who has been followed up routinely for a familial mutation of the SDHB gene. In this routine follow-up examination, an excessive plasma normetanephrine and norepinephrine secretion is evidenced. A genetic paraganglioma is diagnosed. Imaging studies are conducted to identify its location. A prevesical fixation is demonstrated by both the PET-scan and the MIBG scintigraphy. Conventional imaging includes MRI and CT-scan studies. The MRI also demonstrates the 4cm prevesical tumor situated at the upper left horn of the bladder. The CT-scan corroborates the presence of this tumor localized at the upper left portion of the bladder. However, it also shows the presence of an adrenal or peri-adrenal tumor. Since this tumor is not marked on scintigraphy, its diagnosis in association with the paraganglioma cannot be firmly established. At this moment, its resection is decided upon during the same surgical intervention to make sure that there is no double localization of the paraganglioma. The first operative step is to approach the prevesical tumor. Considering the secretion, calcium channel blockers are administered to the patient preoperatively.

2. Laparoscopic exploration 01'35''

The intervention starts with a laparoscopic exploration, which very easily allows to identify the presence of this right lateral vesical tumor. It cannot be determined with certainty if this tumor invades the bladder or not.

3. Start of dissection 01'48''

The dissection is carried out progressively in order to search for a healthy cleavage plane and to ensure that there is no need for an associated bladder resection. The dissection is performed in a stepwise fashion using two instruments, a forceps and a vessel-sealing Ligasure® device. This allows to dissect tissues, to divide and to cauterize them. During the dissection, the limit between the tumor and the bladder is perfectly identified. The tumor is hyper-vascularized. Voluminous and diffuse draining veins are identified. They are easily and perfectly controlled with the 5mm Ligasure® device. The dissection is continued progressively in order to avoid any potential complications. It is essential to prevent any bladder injury and to prevent bleeding that might occur on an artery collateral to the epigastric artery.

4. Lower dissection and peritoneum opening 02'58''

When performing the lowermost dissection, attention must be paid to avoid any injury to the ureter. The peritoneum is incised in a stepwise manner and cauterization is achieved distally from the tumor in order to maintain a safety margin.

5. Tumor freeing and vascular pedicle division 03'22''

The tumor and its vascular pedicles can then be totally freed. Voluminous draining veins can be seen here again when the dissection is performed posteriorly. These veins are controlled. During the surgical procedure, a moderate release of catecholaminergic hormones occurs. These are responsible for a partial increase of the arterial blood pressure without ever reaching values close to malignancy. Blood pressure becomes stabilized immediately as soon as the dissection is completed and the tumor has been totally freed.

6. Peritoneal suture 04'04''

At the end of the procedure, the bladder is repositioned and the peritoneum is sutured in order to get back to a “normal” anatomy. In this young patient aged 13, a re-operation may be envisaged in the future. This peritoneal suture is achieved using absorbable threads and interrupted stitches. The specimen is introduced in a bag in order to avoid any lesion and any peritoneal contamination. The anatomy of the peritoneal cavity is restored. The surgical procedure is further completed by the resection of the left adrenal tumor. As a reminder, this tumor does not show any fixation, as demonstrated by the scintigraphic examination. However, the risk of a paraganglioma being situated in a second location cannot be ruled out.

7. Change in patient position for adrenal dissection 04'57''

Once the prevesical paraganglioma has been extracted through the umbilical opening, the incisions are closed and the patient’s set-up is changed into a conventional left adrenal approach position. The adrenal gland will be approached classically.

8. Dissection of adhesions 05'16''

First, a few adhesions between the splenic flexure and the flank must be taken down. The spleen must then be mobilized. The splenopancreatic block will be progressively tilted to the right and the dissection is continued in contact to Gerota’s fascia in a totally avascular plane.

9. Adrenal gland identification 05'42''

The left adrenal gland will be identified fairly rapidly in this young patient. Attention is turned directly to the tumor in order to assess its morphological aspect. Usually, a total left adrenalectomy should be performed removing both the tumoral lesion and the adrenal gland. However, the absence of fixation as demonstrated by the scintigraphic examination is suggestive of a benign tumor that could be unrelated to the patient’s pathology and that could also not be a paraganglioma.

10. Nodular lesion identification 06'26''

During the dissection, the 15mm diameter nodule is perfectly identified. However, it must be observed that there is a real cleavage plane between the lesion, the adrenal gland and its vascular elements. Considering the patient’s young age, the decision is made to preserve the left adrenal gland along with its entire vascularization.

11. Nodule dissection with preservation of adrenal gland 06'50''

The tumor is located posteriorly to the adrenal vein. It is therefore important to gently retract the gland and the vein and to progressively cauterize all the small vascular elements surrounding the tumoral lesion. Once again, the dissection is carried out using monopolar cautery and/or the Ligasure® device depending on the elements to be controlled. It is essential to identify the entire anatomy, and more specifically the main adrenal vein along with the renal vein.

12. Nodular lesion removal 07'37''

This lesion can be totally removed, preserving its entire vascularization. All in all, this surgery is performed to resect both a prevesical paraganglioma and a left posterior adrenal tumor in a patient with a genetic mutation. The pathological examination will confirm the presence of a moderately malignant PASS score III paraganglioma. The adrenal tumor is examined. The pathological finding demonstrates the presence of a benign adenopathy without any signs of malignancy. The resection of this lesion has therefore been achieved by preserving the left adrenal gland in this young patient aged 13 and by perfectly identifying the anatomy. The postoperative outcome is uneventful and the patient’s long-term follow-up does not show any recurrence of paraganglioma-related hormone hypersecretion.