| Ovarian
teratomas in children are the most common germ cell tumors. Can be
mature, immature and malignant. The vast majority of ovarian teratoma
are mature. Ovarian immature teratomas (OIT) represent 1% of ovarian
tumors graded according to the proportion of tissue containing immature
neural elements. More than 80% of immature teratoma has elevated levels
of alpha-fetoprotein. Tumors with higher AFP levels exhibit additional
foci of malignant germ cell components. Peak incidence occurs between
15 and 19 years of age presenting as pelvic mass, abnormal uterine
bleeding, abdominal pain or abdominal distension. US shows a complex
ovarian lesion (solid and cystic components) or a heterogenous lesion
in CT-Scan. Fat and scattered calcifications can also be present.
Staging represents findings at surgery whether the tumor is confined to
the ovary, peritoneum, pelvis, lymph node, adjacent organs, bilateral
or has malignant ascites. Grade refers to pathologic presence of
immature tissue in lower power field. Immature teratomas behave in a
malignant fashion only if foci of malignant germ cell elements (yolk
sac tumor) are present or if they are resected incompletely giving rise
to the growing teratoma syndrome. Grade at diagnosis is the most
important risk factor for relapse across all age groups. In children
with grade 1 and 2 tumors there are no relapse regardless of stage. The
majority of relapses (20%) occur in children with grade 3 tumors. Grade
3 with stage I/II disease have excellent free survival in comparison
with stage III/IV. Completeness of resection influences free survival.
Most children with OIT will not need chemotherapy. Grade, stage and
completeness of resection are important risk factors for relapse.
Recurrent disease occurs within the pelvis at the site of the original
tumor. Tumor size does not correlate with tumor grade. The management
of ovarian immature teratoma is unilateral salpingo-oophorectomy plus
comprehensive staging. Complete resection is a key factor in avoiding
tumor relapse. Routine biopsy of the unaffected ovary is
unnecessary because immature teratoma is almost always unilateral.
Lymphadenectomy does not provide any significant benefit to the
survival of patients affected by immature teratoma. The reason to
remove the tube with the tumor is to reduce an ectopic pregnancy risk.
Initial adjuvant chemotherapy does not reduce future relapse or
progression in OIT. Ovarian-sparing surgery during tumorectomy is an
option being studied and depends on the anatomic feasibility of each
case. Adjuvant chemotherapy is use for residual or recurrent disease
though it may cause growing teratoma syndrome. Children with OIT should
be follow-up with serial US and AFP levels. References: 1- Zhao T, Liu Y, Wang X, Zhang H, Lu Y: Ovarian cystectomy in the treatment of apparent early-stage immature teratoma. J Int Med Res. 45(2):771-780, 2017 2- Pashankar F, Hale JP, Dang H, et al: Is adjuvant chemotherapy indicated in ovarian immature teratomas? A combined data analysis from the Malignant Germ Cell Tumor International Collaborative. Cancer. 122(2):230-7, 2016 3- Luczak J, Baglaj M: Ovarian teratoma in children: a plea for collaborative clinical study. J Ovarian Res. 11(1):75, 2018 4- Frazer JL, Hook CE, Addley HC, et al: Recurrent ovarian immature teratoma in a 12-year-old girl: Implications for management. Gynecologic Oncology 154: 259-265, 2019 5- Shinkai T, Masumoto K, Chiba F, et al: Pediatric ovarian immature teratoma: histological grading and clinical characteristics. J Pediatr Surg 55: 707-710, 2020 6- Li S, Liu Z, Dong C, Long F, et al: Growing Teratoma Syndrome Secondary to Ovarian Giant Immature Teratoma in an Adolescent Girl: A Case Report and Literature Review. Medicine (Baltimore). 95(7):e2647, 2016 |
| Sinusoidal
obstruction syndrome (SOS) is a hepatic veno-occlusive disease
occurring as a frequent complication after high-dose chemotherapy in
the setting of hematopoietic stem cell transplantation. It can also be
seen after conventional chemotherapy in pediatric patients after
receiving dactinomycin-based chemotherapy for Wilms tumor,
rhabdomyosarcoma, medulloblastoma and malignant germ cell tumor.
Intensive antileukemic regimens that use 6-thioguanine also predispose
to hepatic SOS. The initiating event is injury to the endothelial cells
at the sinusoidal surface of the hepatocyte by the chemotherapeutic
agent. This causes obliteration of the hepatic venules as a result of
subendothelial edema and microthrombosis leading to hepatic congestion,
sinusoidal dilatation and portal hypertension with hepatocyte injury
and death. A cascade of hypercoagulable and proinflammatory pathways
causes further damage resulting in obstruction of hepatic venous
outflow, portal hypertension and multi-organ failure. Diagnosis is
clinical. This usually occurs in the third week after chemotherapy with
symptoms such as fever, jaundice, tender hepatomegaly, vomiting, fluid
retention, ascites, hypoalbuminemia, elevated serum transaminases,
thrombocytopenia and prolonged PT and PTT. In children there is no
limitation for time of onset of SOS and two or more of the symptoms
described are diagnostic. Imaging could demonstrate signs of hepatic
portal hypertension in late stages. The incidence of developing SOS in
Wilms tumors receiving chemotherapy is 1.5 to 8%. Liver SOS appears
after the 2nd to 6th dose of Dactinomycin, seven to 14 days after the
last dose. Special vulnerability occurs in children less than one year
of age, right sided Wilms tumor and those receiving abdominal
radiotherapy. Management of hepatic SOS is supportive with antibiotics,
fluid restriction, diuretics, plasma, albumin and platelets
transfusions. Pharmacologic intervention includes anticoagulants such
as defibrotide, antithrombin III, heparin, protein C concentrate,
N-acetylcysteine and gabexate mesylate. High dose methylprednisolone
and aspirin has been utilized in other patients. Most children recover
after approximately ten days of medical management. References: 1- Lee AC, Goh PY: Dactinomycin-induced Hepatic Sinusoidal Obstruction Syndrome Responding to Treatment with N-acetylcysteine. Journal of Cancer 2: 527-531, 2011 2- Totadri S, Trehan A, Bansai D, Jain R: Sinusoidal Obstruction Syndrome during Treatment for Wilms' Tumor: A Life-threatening Complication. Indian J Med Paediatr Oncol 38(4): 447-451, 2017 3- Corbacioglu S, Carreras E, Ansari M, et al: Diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in pediatric patients: a new classification from the European society for blood and marrow transplantation. Bone Marrow Transplant. 53(2):138-145, 2018 4- Corbacioglu S, Jabbour EJ, Mohty M: Risk Factors for Development of and Progression of Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome. Biol Blood Marrow Transplant 25: 1271-1280, 2019 5- Richardson PG, Grupp SA, Pagliuca A, Krishnan A, Ho VT, Corbacioglu S: Defibrotide for the treatment of hepatic veno-occlusive disease/sinusoidal obstruction syndrome with multiorgan failure. Int J Hematol Oncol. 6(3):75-93, 2017 6- Faraci M, Bertaina A, Luksch R, et al: Sinusoidal Obstruction Syndrome/Veno-Occlusive Disease after Autologous or Allogeneic Hematopoietic Stem Cell Transplantation in Children: a retrospective study of the Italian Hematology-Oncology Association-Hematopoietic Stem Cell Transplantation Group. Biol Blood Marrow Transplant. 25(2):313-320, 2019 |
| Infantile
hepatic hemangioendothelioma (IHH) is a very rare, benign vascular
tumor that appears during the first six-months of life with the
potential to regress spontaneously. Considered the most common vascular
tumor of the liver in children is associated with a high mortality
rate. IHH can be focal, multifocal or diffuse in the liver. Focal IHH
are fully grown tumors at birth that rapidly involutes with time.
Multifocal IHH are individual lesions separated by normal liver
parenchyma. Diffuse IHH is characterized by extensive replacement of
hepatic parenchyma with multiple lesions. IHH can be associated with
high output congestive heart failure, anemia, hypothyroidism,
consumption coagulopathy, thrombocytopenia (Kasabach-Merritt syndrome),
hepatomegaly (causing abdominal compartment syndrome), and
cutaneous hemangiomas. Prenatal diagnosis has been associated with
hydrops fetalis. Postnatal diagnosis is established with US, CT-Scan
and MRI. More than five cutaneous hemangiomas lesions are indication
for liver US in search of IHH. Alpha-fetoprotein levels should be
obtained to differentiate from hepatoblastoma. IHH can be associated
with consumptive hypothyroidism due to overproduction of type 3
iodothyronine deiodinase which deactivates thyroid hormones. Mortality
results from high-output cardiac failure secondary to arteriovenous
shunting within the tumor (up to 50% of the cardiac output can be
diverted), respiratory compromise, hepatic failure, intraperitoneal
hemorrhage and consumptive coagulopathy. The arteriovenous shunting can
result in a decreased of systemic blood volume and increase of
pulmonary blood volume thus the cardiac output increase. The younger
the age at diagnosis, the more severe the cardiac symptoms. Natural
history of asymptomatic IHH is spontaneous involution. Symptomatic
lesions need aggressive management. Radiotherapy and chemotherapy have
not shown consistently good results. Steroid and alpha-interferon are
used as initial treatment to inhibit proliferation of endothelial cells
with mixed results. Propranolol, a beta blocker, is now the preferred
systemic therapy for problematic IHH due to promotion of
pericyte-mediated vasoconstriction, and decrease and cease of growth
with more rapid involution of the lesion. Severe bilobar disease might
need percutaneous hepatic artery embolization or transplantation. Early
embolization is recommended for children with focal or multifocal
lesions presenting as shunts or those unresponsive to medication.
Hepatic artery ligation or embolization should not be done in patients
with shunting from the portal vein to the hepatic vein and minimal
systemic arterial collateral circulation since it might result in
hepatic necrosis. References: 1- Mhanna A, Franklin WH, Mancini AJ: Hepatic infantile hemangiomas treated with oral propranolol--a case series. Pediatr Dermatol. 28(1):39-45, 2011 2- Wang T, Wang Y, Liang Y, Lu G: Infantile Hepatic Hemangioendothelioma Associated With Congestive Heart Failure: Two Case Reports With Different Outcomes. Medicine (Baltimore). 94(52):e2344, 2015 3- Ji Y, Chen S, Xiang B, et al: Clinical features and management of multifocal hepatic hemangiomas in children: a retrospective study. Sci Rep. 2016 Aug 17;6:31744. doi: 10.1038/srep31744. 4- Rodrigues A, Forno A, Costa E, et al: Diffuse infantile hepatic haemangioma-how to manage an incidental but potentially lethal finding. Oxf Med Case Reports. 8: 268-270, 2918 5- Zhang XT, Ren WD, Song G, Xiao YJ, Sun FF, Wang N: Infantile hepatic hemangiomas associated with high-output cardiac failure and pulmonary hypertension. BMC Cardiovasc Disord. 19(1):216, 2019 6- Kassarjian A, Zurakowski D, Dubois J, Paltiel HJ, Fishman SJ, Burrows PE: Infantile hepatic hemangiomas: clinical and imaging findings and their correlation with therapy. AJR Am J Roentgenol. 182(3):785-95, 2004 |
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