Extralobar pulmonary sequestration mimicking neuroblastoma

Extralobar pulmonary sequestration is a congenital pulmonary malformation, which rarely may be present in an intra-abdominal location. We describe a 1-week-old newborn with an intra-abdominal sequestration, which presented to us as an antenatally diagnosed suprarenal mass. Intra-abdominal extralobar sequestration should also be kept in differential diagnosis in cases of masses in the suprarenal location.     

Antenatal detection and management of suprarenal masses

The indications for, and timing of, surgical intervention for suprarenal masses detected prenatally are unclear. Also, the definitive diagnosis of suprarenal masses using imaging studies is difficult at best. We report 2 cases of antenatally detected suprarenal masses. One case represents an initial cystic mass expanding and becoming solid that had benign pathologic features. The second case was a stable solid mass that, on exploration, was malignant. Management options are discussed.     

Prenatal diagnosis of an intrathoracic gastric duplication: a case report

We report an intrathoracic gastric duplication cyst detected antenatally on obstetrical ultrasonography at 33 weeks of gestation. After birth, the newborn was directly intubated. At day 19, the intrathoracic cyst was removed, and the patient discharged on his 27th day of life. Histologic findings confirmed the diagnosis of a gastric duplication cyst. This appears to be the first reported case of an intrathoracic gastric duplication detected antenatally. Intrathoracic gastric duplication should be among the differential diagnosis of any mediastinal cyst discovered antenatally; its detection allows appropriate management at birth.     

Antenatally detected proximal ureteral diverticulum

We present what is to our knowledge the first antenatal detection of a proximal ureteral diverticulum, an extremely rare urologic abnormality, that was successfully reconstructed in the early postnatal period and should henceforth be included in the differential diagnosis of the antenatally detected cystic mass.     

Atrophic upper pole of a duplex collecting system masquerading as suprarenal mass: a case study and literature review

The growing use of maternal fetal ultrasound is leading to the discovery of an increasing number of suprarenal masses. Our experience with a cystic suprarenal mass detected on antenatal ultrasound is described. Location and radiographic features could not rule out the possibility of a cystic neuroblastoma; therefore, surgical resection of the mass was performed. Despite the absence of common radiologic characteristics, the pathology of the specimen revealed a non-functioning upper pole of a duplex kidney with complete duplication of the collecting system. Neonatal evaluation and management and the differential diagnosis are discussed.     

Laparoscopy in the management of antenatally detected liver masses

Antenatally detected liver masses that are not clearly benign on postnatal investigation pose a management dilemma. Unless the diagnosis is clear, observation alone is risky. Improvements in radiological diagnosis permit confirmation of the benign nature of these masses in some instances, but it is usually difficult to distinguish them from malignant lesions. Since recent advances in ultrasound facilitate identification of liver masses during prenatal life, differential diagnosis of these masses has become a recurring issue in recent years. Laparoscopy may play a major role in the surgical management of right upper quadrant masses detected antenatally. We describe its use in a patient with an antenatally detected liver mass. No clear diagnosis could be made with radiologic investigation in the neonatal period. Definitive diagnosis was made laparoscopically: focal nodular hyperplasia was confirmed with laparoscopy and biopsy. In cases where the etiology of a liver mass remains unclear after radiologic investigation, laparoscopic intervention may prove beneficial in neonates and infants. We present an algorithm for the management of similar antenatally detected right upper quadrant lesions.     

Diagnosis of adrenocortical tumor in a neonate by detection of elevated blood 17-hydroxyprogesterone measured as a routine neonatal screening for congenital adrenal hyperplasia: a case report

We report herein a case of prenatally detected neonatal adrenocortical tumor (ACT). The patient was an otherwise healthy newborn girl. No signs of Beckwith-Wiedemann syndrome were identified, and her family medical history did not suggest predisposition to cancer. Computed tomography and ultrasonography after birth revealed a round solid tumor 40 mm in diameter in the right suprarenal area. The precise diagnosis of ACT was unexpectedly obtained based on results from the Japanese neonatal mass screening program. Blood 17-hydroxyprogesterone is routinely measured as a part of this program for early detection of congenital adrenal hyperplasia in Japan. Abnormally elevated level of 17-hydroxyprogesterone was reported in the patient and, thus, led to the diagnosis of ACT. Surgical resection was safely performed with perioperative steroid replacement. Adrenocortical tumors are extremely rare in childhood, particularly in the neonatal period. Some of these tumors secrete abnormally high levels of cortisol, suppressing function of the contralateral adrenal gland and, thus, leading to life-threatening postoperative adrenal insufficiency. Scheduled steroid replacement enables safe perioperative management in such cases. Adrenocortical tumor should always be considered among the differential diagnoses for neonatal suprarenal mass because precise diagnosis will enable the physician to develop appropriate treatment strategies.     

Unusual presentation of right-side accessory spleen mimicking a retroperitoneal tumor

Abstract:   An unusual case of right-side retroperitoneal accessory spleen is presented. A 68-year-old man visited our hospital for the management of incidentally detected retroperitoneal mass. The computed tomography scan of the abdomen revealed the presence of a retroperitoneal tumor (4.0 × 3.8 cm) at the right suprarenal space. Laparoscopic excision was carried out with excellent results. On histological examination, the tumor exhibited a structure typical of splenic tissue. This accessory spleen was unusual in its size and location. Though it existed at the right side, surgeons should be aware of the possible existence of accessory spleens for the differential diagnosis of retroperitoneal tumors.     

Intestinal duplications--experience in 6 cases

Intestinal duplications are rare congenital anomalies, and most of them are detected in the first 2 years of life or antenatally. The clinical presentation depends on location, size, and the presence of ectopic gastric mucosa. Ultrasound scans during pregnancy result in a higher rate of antenatally detected duplications which allows early treatment and avoidance of possible complications. Resection of the duplication without adjacent bowel should be the treatment of choice. En bloc resection or partial excision with mucosectomy is advised in some cases. We present our experience with 6 patients treated in our institution from February 2002 to October 2005.     

Laceration of a benign adrenal adenoma mimicking a splenic rupture

Lacerations of adrenal tumors are very rare events and have been described for myelolipoma, pheochromocytoma, and cortisol-producing adenoma. We report on a patient who was admitted with suspected splenic rupture. Computed tomography showed a mass 14 cm in diameter adjacent to the spleen, but selective angiography revealed blood supply by the left suprarenal artery. A ruptured adrenal tumor was therefore diagnosed and resected. No hormone production was detected. Histologically a benign adrenal adenoma was found. Frequency, diagnosis and therapy of adrenal masses are discussed. Received: 17 June 1997     

Neuroblastoma in an adult

Neuroblastoma is extremely rare in adults. Advances in classification and therapy have been recently made by an analysis of pediatric tumors. We present the case of a 39-year-old man who had Stage 1 neuroblastoma with unfavorable histologic features that was treated with surgery only. Neuroblastoma should be considered as a diagnosis for adult patients with a suprarenal mass and, if confirmed, neuroblastoma in adults should be classified and treated according to pediatric guidelines.     

Bladder evisceration in a child with antenatally detected gastroschisis: Outcome of bladder function

In patients with gastroschisis, evisceration of the bladder is rare. To date, little is known about functional outcome of the antenatally eviscerated bladder. We present a case of antenatally detected gastroschisis associated with entire bladder evisceration and fetal hydronephrosis. Full urological evaluation was carried out after primary closure of the abdominal wall defect. The postoperative period was uneventful and there were no abnormal findings in the kidney or urinary tract. This indicated good prognosis of the antenatally eviscerated bladder.     

Retroperitoneal ganglioneuroma in the infancy

Specific management guides for suprarenal incidentalomas detected after birth do not exist for children, but due to the high proportion of malignant tumors, surgical resection is recommended. We present the case of a 7-year-old patient with a non-functioning left suprarenal incidentaloma. Surgical resection and subsequent anatomopathologic analysis of the mass confirmed the diagnosis of ganglioneuroma in process of maturation. Most ganglioneuromas are incidental and have a good prognosis due to their benign behaviour, even though malignant transformation has also been described, for which reason postoperative controls to detect local recurrence and occurrence of new foci are recommended.     

Adrenal cytomegaly: two cases detected by prenatal diagnosis

We report our experience with two cases of adrenal cytomegaly, both of which were detected as cystic adrenal masses during prenatal ultrasonographic examinations. In Case 1, a left suprarenal cystic mass was detected in the fetus at 25 weeks of gestation. The mass, measuring 7 cm in diameter, did not show any change in size and was resected 26 days after birth. In Case 2, a right suprarenal lesion was found at 30 weeks of gestation. The cystic lesion, measuring 2 cm x 1.5 cm, did not change in size and was resected 3 months after birth. Adrenal cytomegaly is still not well known. It is characterized by the presence of large polyhedral cells with eosinophilic granular cytoplasm and enlarged nuclei in the adrenal cortex. This condition is thought to be a degenerative process but not a malignancy. Adrenal cytomegaly rarely forms cysts. It seemed to be impossible to diagnose preoperatively in our cases. Because of the difficulty of differentiating between cystic adrenal cytomegaly and other cystic diseases such as neuroblastoma, operative intervention is required in cases where the cysts do not decrease in size. Further study of a larger number of cases is needed to establish an optimal treatment protocol for these tumours.     

Upside-down spleen as cause of suprarenal mass

An upside-down configuration of the spleen can be mistaken for a left suprarenal mass. A radioisotopic liver-spleen scan will demonstrate a characteristic appearance of the spleen with its hilus superiorly and its convex border medially and adjacent to the kidney. When a left suprarenal mass is identified, we advocate the liver-spleen scan as the first step in the diagnostic workup. If the typical configuration is found, no further studies are necessary.     

"Coral reef" atherosclerosis of the suprarenal aorta: a unique clinical entity

Localized obstruction in a suprarenal aorta of normal diameter is rare. Between 1970 and 1983, nine patients (all women, mean age 51 years) required aortic reconstruction to relieve severe lower extremity ischemia (nine patients), hypertension (nine), visceral ischemia (two), and congestive heart failure (three) caused by an eccentric, heavily calcified polypoid lesion originating from the posterior surface of the suprarenal aorta. This mass typically began at the level of the diaphragm and extended to the level of the renal arteries, almost totally occluding the aortic lumen. The rock-hard, irregular, gritty, whitish surface strongly resembled a coral reef. Elective revascularization was carried out in eight patients, and an emergency procedure was necessary in one patient who had acute aortic thrombosis with catastrophic visceral, renal, and lower extremity ischemia. The suprarenal atheroma was removed en bloc through a retroperitoneal thoracoabdominal aortic endarterectomy. Concomitant aortoiliofemoral revascularization was necessary in seven patients (five prosthetic grafts, two endarterectomies). Two patients died postoperatively. The seven long-term survivors remain asymptomatic at a mean follow-up interval of 4 years after revascularization, without evidence of recurrence of this lesion. Suprarenal "coral reef" atherosclerosis should be considered if visceral, renal, and limb ischemia is not adequately explained by the arteriographic pattern of conventional atherosclerosis. This unusual atheroma exhibited extensive calcification and metaplastic bone formation, although its precise pathophysiology remains to be defined.     

Non-functioning tumor of the suprarenal gland. Compatible with metastasis from a melanoma

A case of metastatic melanoma in the right suprarenal gland is presented. It manifested as a mass and paraneoplastic syndrome. We put forward a set of considerations on the differential diagnosis of suprarenal masses, and the diagnostic use of several tests taken in conjunction (echography, computer tomography, percutaneous punctures).     

The effect of suprarenal cross-clamping on abdominal aortic aneurysm repair.

Two hundred five patients who underwent elective abdominal aortic aneurysm repair were divided into two groups: those who underwent infrarenal cross-clamping alone (n = 166) and those who underwent suprarenal cross-clamping alone or combined with infrarenal cross-clamping (n = 39). Mortality was comparable between groups (1.2% for infrarenal cross-clamping vs 2.6% for suprarenal cross-clamping). Transient renal insufficiency was more frequent in the suprarenal group than in the infrarenal group (28% vs 10%), but dialysis rates (3% for suprarenal vs 2% for infrarenal) were similar. Cardiac morbidity was comparable between groups as well. Operating room data reflected the technical challenge of complex aneurysm repairs. The retroperitoneal approach was the preferred exposure in the suprarenal group since better access to the suprarenal aorta may be achieved with this technique. While abdominal aortic aneurysm repairs requiring suprarenal cross-clamping remain a technical challenge, the risks are not formidable and suprarenal cross-clamping should be considered when confronted with difficult periaortic dissection.     

Castleman's disease in the left suprarenal region, mimicking an adrenal neoplasm

We present a rare case of a 16-year-old male patient with Castleman's disease (CD) manifest by a suspicious mass within the left suprarenal region abutting the left adrenal gland, mimicking an adrenal neoplasm. CD is an uncommon and poorly understood lymphoproliferative disorder that, when observed, typically occurs as a mediastinal mass. Two percent of cases reportedly occur in the pararenal region. Although rare, Castleman's disease should be considered in the differential diagnosis of a retroperitoneal mass.     

Laparoscopic excision of a gastric duplication cyst detected on antenatal ultrasound scan

The authors report a gastric duplication cyst detected antenatally and its subsequent laparoscopic removal at 2 months of age. Before birth, a cystic mass was detected behind the stomach on fetal ultrasound scan (US). After birth, an US, barium meal, nuclide scan, and cervical and thoracic vertebral x-rays suggested that the most probably diagnosis was a gastric duplication cyst. At 2 months of age, laparoscopic removal of the cyst was performed with closure of the muscle defect in the stomach wall. The patient was discharged 4 days later, feeding normally. Histology findings confirmed the diagnosis of a gastric duplication cyst. After the antenatal detection of a cyst behind the stomach, the laparoscopic removal of this gastric duplication in a 2 month old was accomplished without complications. This appears to be the first reported case using this type of procedure in an infant.