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• W2313362952 abstract "A term newborn presents with respiratory distress and significant abdominal distention after birth (Figure 1).The infant responded well to blow by oxygen, and respiratory distress improved without significant further interventions. Abdominal radiography (Figure 1) was performed immediately to assess level of abdominal distention and revealed left upper quadrant calcifications and a nonobstructive gas pattern with paucity of bowel gas in the left abdomen. Given these unusual findings in an otherwise healthy term infant, the patient then underwent complete abdominal ultrasonography, which revealed a sizeable 9-cm mass that extended from the left kidney and adrenal gland to past the midline in the upper abdomen and ascites. The outside hospital neonatologist then scheduled an urgent transfer of this patient to a children’s hospital for further evaluation and assessment.On arrival at the children’s hospital, the following were observed:On admission to the neonatal intensive care unit, the patient was stable on room air and in no acute distress. After prompt discussion with the patient’s father regarding urgency of diagnostic imaging, the patient was sent for magnetic resonance imaging (MRI) of the abdomen (Figure 2). This study revealed a large circumscribed mixed cystic and solid intra-abdominal mass centered in the left upper quadrant, which was displacing the left kidney inferiorly. Although the organ of origin could not be clearly determined, given the displacement of the adjacent kidney, the source was interpreted as most likely suprarenal and thus potentially the adrenal gland. Fluid distention of the stomach and esophagus was present and thought to represent duodenal or small bowel obstruction due to extrinsic compression from the large mass. Abdominal radiography was performed again to reassess bowel gas patterns for obstruction, but this revealed identical findings to radiography performed before transfer.After diagnostic studies, pediatric oncology and surgical services were notified for immediate consultation. Both consulting services agreed that gross total excision of the mass would likely be curative. After further communication with the family, the decision was made for the patient to undergo surgery. Before surgery, the patient received nothing by mouth with an orogastric tube in place to release pressure from duodenal obstruction because the patient was unable to tolerate even small volume feeds. With direction by the oncology service, laboratory evaluation revealed no abnormalities in vanillylmandelic acid, homovanillic acid, α-fetoprotein, β-human chorionic gonadotropin, uric acid, or lactic acid. Four days after delivery, the patient was taken to the operating room, where complete excision of the mass was performed along with removal of the left adrenal gland.Term infant with a large intra-abdominal mass of cystic and solid nature with calcifications noted on abdominal radiography in the left upper quadrant. The differential diagnoses are listed below:The infant was diagnosed with benign mature teratoma.Teratomas are benign germ cell neoplasms composed of more than one cell line (ectoderm, endoderm, and/or mesoderm). It is hypothesized that incomplete differentiation or migration of these precursor cells may lead to the formation of teratomas. (1)(2) Retroperitoneal teratomas are rare, account for only 2% to 5% of all extragonadal teratomas, and are notable in their 2:1 female predominance. Although most of these tumors are benign, 7% become malignant, although this percentage is elevated to 25% when diagnosed in the first month after birth. (3)Mature teratomas, as seen in this patient, are by classification grade 0 and are not considered malignant, although they possess the risk to undergo malignant transformation when left untreated. (3) Although generally considered to be slow growing, several studies have found teratomas with advanced rates of growth in neonates, making gross total surgical resection critical from an oncologic and mass effect standpoint. (4)Although there are no known genetic syndromes associated with teratoma development, the heterogeneity of the tumor’s multiple cell lines makes differentiation from conditions such as fetus in fetu challenging even after surgical resection and pathologic analysis. (5)A large portion of these tumors have both immature and mature segments that increase risk of malignant transformation when left untreated or not detected (Table 1).Teratomas (both benign and malignant) carry a wide range of histologic variance because they are formed from 3 types of precursor cell lines. Osseous, neural, dermal, adnexal, lymphoid, muscular, and cartilaginous tissues are all commonly found in teratomas to varying degrees. Retroperitoneal teratomas tend to be more cystic in nature but also have a variety of tissues present when analyzed.The diagnosis of benign retroperitoneal teratoma is typically made within the first 5 years after birth based on careful physical examination given the low percentage of total teratomas that present in this region. (6) Although abdominal masses are a common presentation, other signs and symptoms of large tumors in the retroperitoneum can be observed secondary to mass effect, such as compression of the gastrointestinal system causing pseudo-obstruction, genitourinary abnormalities such as changes in urine output or urine retention, or high-output heart failure in cases of well-vascularized teratomas.Even with appropriate imaging and laboratory assessment, it is difficult to differentiate a retroperitoneal teratoma from other more treacherous types of solid masses. This places significant stress on immediate assessment by oncologic and surgical services because time is critical in assessment and eventual resection of these masses.The evaluation of an infant with abdominal distention requires careful physical examination and immediate imaging studies. Although magnetic resonance imaging is the preferred modality for definitive assessment of the anatomy of any retroperitoneal mass, abdominal radiography is also critical for assessment of calcifications that are not easily visualized by MRI.Exploratory surgical laparotomy with the goal of gross total resection is the curative measure for the most common forms of retroperitoneal tumors, including teratomas, neuroblastomas, and germinomas. These procedures should be performed at tertiary children’s hospitals given the need for pediatric surgeons, pediatric oncologists, and intra-operative pathology assessment.For benign teratomas, such as that found in this patient, gross total resection is curative, and there is no need for chemotherapy or radiation therapy after gross total resection. Postsurgical monitoring with serial abdominal MRI by a pediatric oncologist is recommended for up to 1 year because regrowth of this type of tumor is extraordinarily rare beyond that timeframe.Prepared by JoDee M. Anderson, MD, MEd, Assistant Editor, Visual Diagnosis, Video Corner; Associate Professor, Oregon Health & Science University, Portland, OR." @default.
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• W2313362952 date "2014-12-01" @default.
• W2313362952 modified "2023-09-27" @default.
• W2313362952 title "Newborn With Respiratory Distress and Abdominal Distention" @default.
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• W2313362952 doi "https://doi.org/10.1542/neo.15-12-e570" @default.
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