PEDIATRIC SURGERY Update

Vol. 50 No. 03 MARCH 2018

Peliosis

Peliosis hepatis (PH) is a very rare benign disease characterized by multiple small
blood-filled cysts of various sizes and shape within the liver parenchyma. Peliosis
comes from the Greek word pelios that means reddish or bluish (extravasated blood).
What triggers PH is unknown, but dilation of sinusoids might be due to an altered
outflow damaging the sinusoid wall and creating dilation of the central vein of the
hepatic lobules. Peliosis can be focal or widespread with most cases involving the right
hepatic lobe. Peliosis can also occur on other organs such as the spleen, bone marrow,
lymph nodes, etc. Etiologic factors associated with PH include drugs, autoimmune
mechanisms and infectious causes. Drugs associated with PH include steroids, oral
contraceptives, tamoxifen, methotrexate, thiopurine, azathioprine and iron chelators.
Alcohol consumption can trigger PH. Imaging studies such as CT-Scan and MRI
angiography suggest the diagnosis, but cannot be precised enough since PH cannot be
differentiated from adenomas, hemangiomas, focal nodular hyperplasia, Caroli disease
or multiple abscess. Lesions are from few millimeters in diameter to 4 cm. Ultrasound
may show a pseudocystic lesion of the hepatic parenchyma with intra- or perilesional
vascularity. Angiography demonstrates multiple hypervascularized nodules during the
late arterial phase with enhancement more pronounced during the parenchymal phase
which persists during the venous phase. MRI when combined with hepatospecific
contrast material represents the gold standard for radiological diagnosis of PH. Due to
the high risk of bleeding an open biopsy using intraoperative US is needed to establish
the diagnosis of PH. The disease can cause stenosis of the vena cava when developed
in young age. Though mainly asymptomatic, PH can rupture and produce spontaneous
hemoperitoneum. Management is usually in the acute setting due to bleeding and
consists of either hepatic lobectomy, transplantation or percutaneous embolization.

References:
1- Crocetti D, Palmieri A, Pedulla G, Pasta V, D'Orazi V, Grazi GL: Peliosis hepatis: Personal experience
and literature review. World J Gastroenterol. 21(46):13188-94, 2015
2- Hong GS, Kim KW, An J, Shim JH, Kim J, Yu ES: Focal type of peliosis hepatis. Clin Mol Hepatol.
21(4):398-401, 2015
3- Iwata T, Adachi K, Takahashi M: Peliosis Hepatis Mimicking Malignant Hypervascular Tumors. J
Gastrointest Surg. 21(6):1095-1098, 2017
4- Dai YN, Ren ZZ, Song WY, Huang HJ, Yang DH, Wang MS, Huang YC, Chen MJ, Zhang JJ, Tong YX,
Pan HY: Peliosis hepatis: 2 case reports of a rare liver disorder and its differential
diagnosis. Medicine (Baltimore). 96(13):e6471, 2017
5- Tan CHN, Soon GST, Kow WCA: Liver lesions detected in a hepatitis B core total antibody-positive
patient masquerading as hepatocellular carcinoma: a rare case of peliosis hepatis and a review of the
literature. Ann Hepatobiliary Pancreat Surg. 21(3):157-162, 2017
6- Biswas S, Gogna S, Patel P: A Fatal Case of Intra-Abdominal Hemorrhage Following Diagnostic Blind
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Percutaneous Liver Biopsy in a Patient With Peliosis Hepatis. Gastroenterology Res. 10(5):318-321, 2017

Renal Clear Cell Sarcoma

Childhood renal tumors account for 7% of all pediatric cancers. Most cases (90%) are
Wilms tumor. Renal Clear Cell Sarcoma (RCCS) is a rare and very aggressive pediatric
tumor characterized for its tendency to metastasize to bone often called the bone
metastasis renal tumor of children. The term clear cell sarcoma relates to the presence
of numerous intracytoplasmic vesicles present in the tumor histology. Other features
that differentiate RCCS from Wilms tumor are that they are unicentric in the medullary
region of the kidney with foci of necrosis and cyst formation. RCCS is the second most
common malignant kidney tumor in children after Wilms tumor comprising
approximately 4-6% of all pediatric renal tumors. RCCS is not associated with genetic
predisposition syndrome and familial cases have not been reported. RCCS is rare
below the age of one year, have a peak incidence between three and five years of age
and are more common in males patients. Metastasis from RCCS can also occur to
lymph nodes, lungs, liver and brain. RCCS occurs in the same age range as Wilms
tumor with no specific radiological features to help distinguish one from the other.
Grossly RCCS include large tumor size (more than 10 cm in diameter), mucoid texture,
foci of necrosis and prominent cyst formation. Nine histologic different patterns of
RCCS have been described. The four important prognostic factors associated with
RCCS include treatment with doxorubin, beyond stage I, age at diagnosis greater than
two years and tumor necrosis. Management of RCCS at all stages requires aggressive
surgical approach followed by chemotherapy and radiotherapy as per NWTS-5
protocols. Overall survival after treatment is 69%. Relapse rates are high and often
occur late. Adverse prognostic factors identified are young age, advanced stage IV
disease and those with relapse disease.

References:
1- Walke VA, Shende NY, Kumbhalkar DT: Renal Clear Cell Sarcoma - Anaplastic Variant: A Rare Entity.
J Clin Diagn Res. 11(1):ED10-ED11, 2017
2- Sinha S, Khurana N, Sarin YK: Clear cell sarcoma of the kidney: report of two cases. APSP J Case
Rep. 5(3):32, 2014
3- Brok J, Treger TD, Gooskens SL, van den Heuvel-Eibrink MM, Pritchard-Jones K: Biology and
treatment of renal tumours in childhood. Eur J Cancer. 68:179-195, 2016
4- Mandal KC, Mukhopadhyay M, Barman S, Halder P, Mukhopadhyay B, Kumar R: Uncommon renal
tumors in children: A single center experience. J Indian Assoc Pediatr Surg. 21(2):61-5, 2016
5- Weaver J, Ho T, Lang A, Koenig JF, Coplen DE, Dehner L, Traxel EJ: Bladder Recurrence of Clear Cell
Sarcoma of the Kidney Seven Years After Initial Presentation. Urology. 106:193-195, 2017
6- Wong MK, Ng CCY, Kuick CH, et al: Clear cell sarcomas of the kidney are characterised by BCOR
gene abnormalities, including exon 15 internal tandem duplications and BCOR-CCNB3 gene fusion.
Histopathology. 72(2):320-329, 2018

Prophylactic Thyroidectomy

Prophylactic removal of all the thyroid gland (total thyroidectomy) is curative treatment
for children at risk of developing medullary thyroid cancer (MTC) caused by mutation in
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the RET proto-oncogene. Medullary thyroid cancer arises from the parafollicular cells
which are responsible for secreting calcitonin. Calcitonin is a sensitive and specific
tumor marker for MTC. MTC has an early and high penetrance in hereditary syndrome
caused by the RET mutations including multiple endocrine neoplasia (MEN) type 2A
and type 2B, and familial MTC which progress to regional lymph nodes and distant
metastasis if left unmanageable. The vast majority of MTC in children is hereditary.
Children with MEN 2A, MEN 2B and familial MTC can be followed after total
prophylactic thyroidectomy with calcitonin levels to monitor for recurrence or
development of MTC. Thyroglobulin, a protein precursor of thyroxine produced by the
thyroid follicular cells can be a useful test following prophylactic thyroidectomy since
many times surgeons leave behind thyroid tissue specially in the nearby region of the
recurrent laryngeal nerve (Zuckerkandl tubercle or ligament of Berry). If thyroglobulin is
high, an US should be performed to quantify how much residual thyroid tissue was left
behind since it has parafollicular cells that can become MTC. Children with MEN2B
should go RET genetic analysis and genophenotype ranking or risk level at birth and
those with MEN2A and familial MTC before age of five years. Age of prophylactic
thyroidectomy recommended include before the first year of age for those MEN children
with RET gene mutation and highest genophenotype risk (ATA-D), before age five
years for RET mutation and lower risk (ATA-C) and before 10 of age with the minimal
risk (ATA-B and ATA-A) or familial MTC. During prophylactic thyroidectomy central
lymph node removal is not warranted unless the child has elevated calcitonin level (> 40
pg/mL) with clinical MTC. Annual calcitonin level is needed in all cases and if abnormal
thyroidectomy should be performed immediately. The high rate of postoperative
hypocalcemia in very young children undergoing prophylactic thyroidectomy has
hampered others not to recommend it before the age of three years.

References:
1- Lifante JC, Blanchard C, Mirallia E, David A, Peix JL: Role of preoperative basal calcitonin levels in the
timing of prophylactic thyroidectomy in patients with germline RET mutations. World J Surg. 38(3):576-81,
2014
2- Seib CD, Harari A, Conte FA, Duh QY, Clark OH, Gosnell JE: Utility of serum thyroglobulin
measurements after prophylactic thyroidectomy in patients with hereditary medullary thyroid cancer.
Surgery. 156(2):394-8, 2014
3- Starenki D, Park JI: Pediatric Medullary Thyroid Carcinoma. J Pediatr Oncol. 3(2):29-37, 2015
4- Kluijfhout WP, van Beek DJ, Verrijn Stuart AA, Lodewijk L, Valk GD, van der Zee DC, Vriens MR, Borel
Rinkes IH: Postoperative Complications After Prophylactic Thyroidectomy for Very Young Patients With
Multiple Endocrine Neoplasia Type 2: Retrospective Cohort Analysis. Medicine (Baltimore). 94(29):e1108,
2015
5- Boybeyi-Turer O, Vuralli D, Karnak I, Gonc N, Yalcin E, Orhan D, Kandemir N, Tanyel FC: Surgical and
clinical strategies in the management of thyroid medullary carcinoma in children with and without ret
proto-oncogene mutations. Turk J Pediatr. 58(4):436-441, 2016
6- Bussieres V, Roy S, Deladoey J, Rousseau E, St-Vil D, Piche N: Prophylactic thyroidectomies in MEN2
syndrome: Management and Outcome. J Pediatr Surg http://dx.doi.org/10.1016/j.jpedsurg.2017.11.015

*Edited by: Humberto Lugo-Vicente, MD, FACS, FAAP

Rio Piedras, Puerto Rico. Director - Pediatric Surgery, San Jorge Childrens
Hospital.
Address: P.O. Box 10426, Caparra Heights Station, San Juan, Puerto Rico USA
00922-0426.
Tel (787)-999-9450 E-mail: titolugo@coqui.net
Internet: http://home.coqui.net/titolugo
PSU 1993-2017
ISSN 1089-7739