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A Child With An Abdominal Mass: Pediatric Rounds
A Child With An Abdominal Mass: Pediatric Rounds
Table 1. Differential Diagnosis of Abdominal Mass in Table 2. Suggested Initial Evaluation of Abdominal Mass
Children
Radiologic imaging
Neonates Plain abdominal radiograph
Renal Sonogram
Hydronephrosis* Computed tomography scan or magnetic resonance imaging*
Multidysplastic kidney* Laboratory studies
Mesoblastic nephroma* Complete blood count with differential
Renal vein thrombosis† Electrolytes (including calcium, phosphorus) blood urea nitrogen,
Polycystic kidney disease† creatinine
Wilms’ tumor† Uric acid and lactate dehydrogenase
Rhabdoid tumor† Urinalysis
Pelvic Urine homovanillic acid and vanillylmandelic acid*
Ovarian cyst Serum β chorionic gonadotropin and alpha-fetoprotein*
Hydrocolpos
*If clinically indicated.
Hydrometrocolpos
Gastrointestinal duplication
masses or, less commonly, with hematuria and may be signs include periorbital ecchymoses, exophthalmos,
detected prenatally. Associated paraneoplastic syn- and Horner’s syndrome (miosis, ptosis and anhidro-
dromes have been described, mainly hypercalcemia sis). Urinary catecholamines are elevated in 90% to
and hypertension.7,8 Mesoblastic nephroma most often 95% of cases. CT scanning or MR imaging is needed to
is a benign lesion that is successfully treated by surgical delineate the tumor size, extent of invasion of adjacent
resection. Several instances of metastasis have been structures, and presence of metastatic disease. Bone
described.9,10 Other rarely encountered masses in neo- scan and skeletal radiographs are helpful in defining
nates derived from the genitourinary system include bony involvement. Bone marrow invasion should be
polycystic kidney disease, renal vein thrombosis, Wilms’ evaluated by bilateral bone marrow biopsies. The out-
tumor, rhabdoid tumor, hydrocolpos, and hydrometro- come depends on the tumor stage and the patient’s
colpos.11,12 age at diagnosis, but the behavior of neuroblastoma is
Ovarian cysts commonly occur in neonates and may not always predictable. N-myc oncogene amplification
also be diagnosed prenatally. They are mostly functional occurs in approximately 20% of such tumors and is
cysts stimulated by fetal, placental, and maternal hor- strongly associated with poor prognosis.2,20 Chemo-
mones. A significant proportion of cysts undergo sponta- therapy and surgical resection followed by radiation
neous regression within the first few months of life, and therapy may be employed. A variant of neuroblastoma
the incidence of malignancy in these cysts is extremely affecting infants, with dissemination limited to bone
low. Large-sized cysts, measuring more than 5 cm, may marrow, skin, or liver, has an exceptionally good out-
be percutaneously aspirated to minimize the risk of tor- come with spontaneous tumor regression. Patients with
sion. Surgical intervention in neonates is generally dis- neuroblastoma should be immediately referred to a
couraged as a primary therapy and is reserved for persis- tertiary center with pediatric oncology and surgical
tent or recurrent cases.13,14 expertise.1,2
Abdominal masses in neonates may originate from Wilms’ tumor. Wilms’ tumor is the second most
the gastrointestinal tract. Duplications are cystic congen- common abdominal tumor in childhood and the most
ital abnormalities of the gastrointestinal tract that can common primary pediatric renal malignancy. It is an
occur at any level from mouth to anus but most com- embryonal renal neoplasm, with 450 new cases report-
monly involve the ileum, followed by the esophagus and ed annually in the United States.3 Presentations in-
duodenum. Duplications vary in size, have spherical or clude a flank or abdominal mass, left-sided varicocele,
tubular shapes, and may or may not communicate with hematuria, and hypertension. Such masses can be
the enteric lumen. They can present as asymptomatic quite large at diagnosis because they can go unnoticed
masses or with signs of obstruction, bleeding, and perfo- due to their retroperitoneal location and are usually
ration. Malignant transformation has rarely been report- painless unless hemorrhage or rupture occurs. Wilms’
ed.15 Sonography is helpful in establishing the diagnosis. tumor may occur in association with other congenital
Meckel scan can be useful because of the frequent pres- anomalies or syndromes including sporadic aniridia,
ence of gastric mucosa. Occasionally, duplications are isolated hemihypertrophy, cryptorchidism, Beckwith-
only recognized at the time of surgery. Surgical resection Wiedemann syndrome, Denys-Drash syndrome, and
is usually curative.16,17 WAGR complex (Wilms’ tumor, aniridia, genitourinary
malformations, mental retardation). In such associa-
Infants and Children tions, Wilms’ tumor is more likely to be bilateral and
Malignant lesions are more commonly encountered may present at a younger age.21 Approximately 15% of
as the cause of abdominal masses in infants and chil- patients will have metastatic disease at diagnosis, most
dren than in neonates. The most common tumors are commonly affecting the lungs followed by the liver and
neuroblastomas, Wilms’ tumors, and lymphomas. regional lymph nodes.22 Sonography is the best initial
Neuroblastoma. Neuroblastoma is the most com- imaging technique to confirm the kidney as the organ
mon malignancy in infants and the most common of origin and to estimate the tumor size. Major blood
extracranial solid tumor in childhood.18 Neuroblasto- vessels should be assessed to determine the extent of
mas arise from neural crest cells within the sympathetic intravascular tumor thrombi if present. A CT scan with
chain or adrenal medulla, with 60% to 70% of cases contrast is helpful to determine the degree of kidney
originating within the abdomen.1,19 Approximately 550 invasion and evaluate for metastasis. The contralateral
new cases are diagnosed in the United States every kidney should be assessed carefully for possible involve-
year.19 Presenting symptoms include a palpable mass, ment. Treatment includes surgery, if possible, radia-
pain, weakness, and failure to thrive. Other associated tion, and chemotherapy. Four-year survival rates range
from 95% for patients with low stage and favorable his- choice, revealing a hyperechoic, solid intrahepatic
tology to less than 25% for advanced initial disease and mass. Sonography also permits assessment for vascular
unfavorable histology.21 Similar to neuroblastoma, such invasion. CT scanning may follow to determine disease
patients should be cared for in a specialized pediatric extent and look for metastasis. Evaluation by this radio-
center. logic technique often demonstrates a delineated hypo-
Lymphomas. Lymphomas are the third most common attentuated mass compared with the surrounding nor-
malignancies in childhood. Sixty percent are non- mal tissue. A biopsy is usually recommended for
Hodgkin lymphomas and one third of these have ab- diagnosis. Treatment consists of surgical resection and
dominal disease.1 Signs and symptoms include abdominal chemotherapy. Liver transplantation is occasionally
pain, gastrointestinal obstruction, or a palpable mass. performed in selected patients. Long-term survival
Intussusception, secondary to a lymphomatous lead varies depending on the stage of the disease and the
point, can occasionally be the presenting picture. Son- success of initial surgical resection.26,29
ography, often confirmed by CT scanning, can determine Embryonal sarcoma is a mesenchymal malignancy
the primary lesion and other organ involvement. These that accounts for approximately 10% of all hepatic
lesions tend to grow rapidly but are usually responsive to tumors in children.29 Presentations include an abdomi-
chemotherapy. Surgical excision and occasionally radia- nal mass, swelling, or pain. Serum alpha-fetoprotein
tion may also be employed. Overall survival rates range levels are usually normal. Sonographic evaluation
between 75% and 95%.23 Peripheral blood stem trans- reveals a large predominantly solid mass with hypo-
plantation may be an option for patients with advanced or echoic areas representing cystic areas. CT scan demon-
recurrent disease. strates a hypovascular low-attenuated mass with septa.
Ovarian masses. Masses of ovarian origin, mostly Treatment with multiple modalities including adjunct
ovarian cysts, are also encountered in childhood. Such chemotherapy, radiation, and surgical resection pro-
cysts are occasionally associated with sexual precocity. vides a 70% to 80% survival rate at 4 years.26,29
Sonography can help establish the diagnosis. Manage- Nonhepatic gastrointestinal abdominal masses in
ment of ovarian cysts is based on the cyst size and com- children also include duplications, Meckel’s diverticu-
position and the patient’s symptoms. Ovarian malig- lum, fecal masses caused by severe constipation, and
nancies may be present in approximately 30% of cases, omental or mesenteric cysts.
especially when solid or complex structures are noted
within the mass.24 The most common of these malig- Key Point
nant tumors are teratomas. Ovarian malignancies in Abdominal masses in neonates are most often benign and of
children are frequently found at an early stage and genitourinary origin. Malignant abdominal masses are more
likely to be encountered beyond the neonatal age and mainly
tend to respond favorably to chemotherapy.13,14,25
include neuroblastomas, Wilms’ tumors, and lymphomas.
Hepatic masses. Abdominal masses arising from the
liver are often malignant and include hepatoblastomas,
embryonal sarcoma, hepatocellular carcinoma, or CASE PATIENT: DIAGNOSIS AND MANAGEMENT
metastatic disease.26 Less frequently occurring benign The exact origin of the mass could not be deter-
masses of hepatic vascular origin include hemangioen- mined radiologically. The etiology was not evident
dotheliomas and hamartomas.27 Hepatoblastomas are from other noninvasive tests. The preoperative diagno-
the most common liver malignancies in this age- sis was possible gastrointestinal tract duplication. Jaun-
group, accounting for at least 75% of cases.28 They may dice was thought to be caused by common bile duct
be congenital or familial and usually present with a obstruction. At exploratory laparotomy, a large mass
rapidly enlarging abdomen in an otherwise asympto- adherent to the posterior gastric wall was observed. A
matic child. They are derived from undifferentiated large blood clot was present within the mass. The mass
embryonal tissue and have been associated with pre- was completely resected and did not communicate
maturity, Beckwith-Wiedemann syndrome, and familial with the gastric lumen. A small perforation was found
polyposis. Serum alpha-fetoprotein levels are almost in the common bile duct, probably from the mass
always elevated, which aids in diagnosis and in moni- effect causing ischemia. The perforation was repaired.
toring response to therapy. Metastasis is present in 20% Histologic examination showed a gastric duplication
of cases at diagnosis with the lungs being the most cyst. The patient had an uncomplicated postoperative
common site, followed by the brain, bone, or bone course. She has continued well over several months of
marrow. Sonography is the initial imaging modality of follow-up.
CONCLUSION 12. Rizk D, Chapman AB. Cystic and inherited kidney dis-
The neonate, infant, or child with an abdominal eases. Am J Kidney Dis 2003;42:1305–17.
13. Templeman C. Ovarian cysts. J Pediatr Adolesc Gynecol
mass needs rapid clinical evaluation. Age, history, and
2004;17:297–8.
physical examination provide initial guideposts to diag- 14. Strickland JL. Ovarian cysts in neonates, children and
nosis. Imaging studies, particularly sonography, may adolescents. Curr Opin Obstet Gynecol 2002;14:459–65.
provide a specific diagnosis. If the initial evaluation 15. Coit DG, Mies C. Adenocarcinoma arising within a gas-
indicates possible malignancy, more complex testing of tric duplication cyst. J Surg Oncol 1992;50:274–7.
blood, bone marrow, serum chemistries, and urine 16. Berrocal T, Lamas M, Gutieerrez J, et al. Congenital
may be required. CT scanning may be also be useful in anomalies of the small intestine, colon, and rectum. Ra-
identifying the type and extent of abdominal masses in diographics 1999;19:1219–36.
children. Outcome varies widely depending on the 17. Bissler JJ, Klein RL. Alimentary tract duplications in chil-
malignant or benign nature of the existing mass but is dren: case and literature review. Clin Pediatr (Phila)
1988;27:152–7.
generally more favorable in neonates. HP
18. Goodman MT, Gurney JG, Smith MA, Olshan AF. Sym-
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