| 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 Percutaneous Liver Biopsy in a Patient With Peliosis Hepatis. Gastroenterology Res. 10(5):318-321, 2017 |
| 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
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 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 |
We are inviting all of you to submit contribution of your own to the newsletter. Credit will be given to the authors keeping his or her name, institution and E-mail address on their work.A list of contributors will appear in the internet homepage.All works should be send to Dr. H. Lugo-Vicente to the e-mail: titolugo@coqui.net |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|