Breast hibernomas: Questioning the embryological origin?

This paper aims to review the concept of hibernomas, with focus on their occurrence, in the breast. It will make reference to a specific case from the Helen Joseph Hospital's Breast Clinic situated in Johannesburg, South Africa. We describe the clinical, radiological and pathological findings in a patient as well as the final diagnosis and treatment (in the form of surgery). This report emphasizes the distinguishable features of hibernomas, and gives guidance as to the surgical approach in large hibernomas stressing the ease of achieving cosmesis without major oncoplastic techniques. More specifically, discussion as whether these rapidly growing, nonrecurring, usually encapsulated growths consisting of brown fat tissue similar to that found in hibernating animals arise from the breast or the underlying muscle is considered.

Diagnostic Imaging of Fetal and Neonatal Abdominal and Soft Tissue Tumors.

Imaging plays a key role in the diagnosis and staging of prenatal and neonatal tumors, and is essential in treatment planning. Though obstetrical ultrasound is the first choice prenatally, fetal MRI continues to play an increasing role as experience with this imaging modality increases. In the neonate, in addition to ultrasound and MRI, CT and nuclear medicine studies can also play an important role. We describe the prenatal and neonatal imaging findings of some of the most common congenital abdominal and soft tissue neoplasms including neuroblastoma, renal, liver and soft tissue tumors.

Urologic and anorectal complications of sacrococcygeal teratomas: prenatal and postnatal predictors.

PURPOSE: Anorectal and urologic sequelae are observed in long-term survivors of sacrococcygeal teratoma (SCT). In this study we evaluate the incidence and predictors of anorectal and urologic complications in SCT. METHODS: A retrospective review was performed for all SCT patients who underwent resection at a single institution between 2000 and 2012. Enrollment criteria included a minimum of 12months follow-up. Categorical variables were analyzed by Fisher's exact test and continuous variables by Mann Whitney test (p<0.05). RESULTS: Forty-five patients were studied. Anorectal complications occurred in 29%, including severe chronic constipation (n=13) and fecal incontinence (n=4). Urologic complications occurred in 33%, including neurogenic bladder (n=12), vesicoureteral reflux (n=5), and urinary incontinence (n=7). Prenatal imaging by fetal MRI demonstrated mass effect with obstruction of the bowel (n=4) or bladder and collecting system (n=7) in a subset of patients with postnatal complications (anorectal 4/4, PPV 100%; urologic 6/7, PPV 86%). Postnatal complications were associated with obstructive findings on prenatal imaging, prenatal therapeutic interventions, Altman classification, perineal reconstruction, and tumor recurrence. No anorectal or urologic complications occurred in patients with Altman type I tumors. CONCLUSIONS: Urologic and anorectal complications are common in patients with SCT. Higher Altman classification and prenatal imaging suggestive of intestinal or urologic obstruction should prompt focused prenatal counseling and postnatal screening for anorectal and urologic dysfunction.

Zebrafish models of rhabdomyosarcoma.

Rhabdomyosarcoma (RMS), an aggressive malignant neoplasm that shows features of skeletal muscle, is the most common soft tissue tumor of childhood. In children, the major subtypes are embryonal and alveolar. Although localized disease responds to a multimodal treatment, the prognosis for patients with high-risk features and metastasis remains dismal. Several in vivo models of RMS have been developed in mouse, human xenografts, zebrafish, and Drosophila to better understand the underlying mechanisms governing malignancy. The findings so far have indicated the potential role of skeletal muscle precursor cells in malignant transformation. To better understand histogenesis and different aspects of tumorigenesis in RMS, we have previously developed a robust zebrafish model of kRAS-induced RMS, which shares morphologic and immunophenotypic features with the human counterpart. Cross-species mircroarray comparisons confirm that conserved genetic pathways drive RMS growth. The ease for ex vivo manipulation allows the development of different transgenic and co-injection strategies to induce tumor formation in zebrafish. In contrast to other vertebrate model systems, the tumor onset in zebrafish is short, allowing for efficient study of different tumor processes including tumor growth, self-renewal, and maintenance.

Damage control operation for massive sacrococcygeal teratoma.

Surgical resections of massive sacrococcygeal teratomas (SCTs) carry significant risk due to baseline hemodynamic instability and the potential for significant hemorrhage. In this case report, a fetus with sacrococcygeal teratoma developed high-output cardiac instability at 32 weeks' gestation. After urgent cesarian delivery, a damage-control operation using Teflon-pledgeted mattress sutures allowed for hemodynamic control of bleeding into the tumor. One week later, after subsequent fluid resuscitation and stabilization, an elective staged resection of the complete mass including the pelvic portion was performed. The patient was discharged home on postoperative day 30 and was doing well at her most recent clinic visit at 30 months of age.

Rapidly involuting congenital hemangioma: a case of complete prenatal involution.

We report a case of rapidly involuting congenital hemangioma of the flank, which was diagnosed in the 2(nd) trimester of gestation and showed complete involution before term. In our case sonography revealed a highly vascular soft tissue mass with smooth contours, which was isointense with the placenta on T2-weighted MR images. The fetus was born with scar tissue at the site of the lesion. To our knowledge this is the 1(st) reported case of rapidly involuting congenital hemangioma showing complete involution before term.

Laparoscopic ligation of the median sacral artery before resection of a sacrococcygeal teratoma.

To minimize the risk of exsanguinating hemorrhage, the authors describe a technique of laparoscopic ligation of the median sacral artery before posterior sagittal resection of type I sacrococcygeal teratoma (SCT). Two female infants with antenatally diagnosed SCT underwent postnatal evaluation and preoperative imaging and were taken to the operating room. In both patients, pneumoperitoneum was established via an epigastic 5-mm trocar. Two additional trocars were inserted in the right and left lower quadrants. The peritoneal reflection was opened to the right of the sigmoid colon, and the presacral space was explored. A large median sacral artery was identified easily, isolated, and divided. The children then were placed in a prone position, and the tumors underwent en bloc resection via a Chevron incision with minimal blood loss. The laparoscopic portion of the procedure was performed in an average of 15 minutes. This is the first report of laparoscopic ligation of the median sacral artery before posterior resection of a sacrococcygeal tumor in an infant. This technique can be performed easily with minimal morbidity. Division of this artery is a logical preventative measure and may reduce the risk of hemorrhage during operative resection.

Newborn with an open posterior hip dislocation and sciatic nerve injury after intrauterine radiofrequency ablation of a sacrococcygeal teratoma.

Advanced prenatal ultrasonography techniques have allowed for better understanding of the natural history, treatment, and prognosis of sacrococcygeal teratomas. Several intrauterine surgical techniques to debulk the tumor when fetal and maternal life are in jeopardy have been described. Orthopaedic impairment, such as lower extremity weakness and swelling, also has been described in association with sacrococcygeal teratomas. The authors report on a newborn in whom a large soft tissue defect overlying the posterior hip region with direct exposure of the disarticulated hip joint existed at the time of birth, which resulted from intrauterine radiofrequency ablation of a sacrococcygeal teratoma. This unexpected complication has resulted in a loss of sciatic nerve function, malformation of the acetabulum and femoral head, and loss of the left ischium, coccyx, inferior sacrum, gluteal, adductor and piriformis muscles, and posterior hip capsule. At 16 months of age, the patient has a flaccid left lower extremity with a hypoplastic hip joint.

[A bizarre excrescence of the umbilicus in a 1-month-old child]. / Une excroissance bizarre de l'ombilic chez un enfant de un mois.

We report the case of an umbilical polyp, derived from omphalo-mesenteric remnants in an one-month-old female child. This rare abnormality results from a closure defect of the vitelline duct. The vitelline duct normally closes between the 5th and the 7th weeks of intra embryonic development but can lead to several pathologies in case of closure defects, giving rise to abdominal (Meckel diverticulum, vitelline cyst) or umbilical symptoms (umbilical fistula, umbilical sinus and umbilical polyp). These disorders have a 2% incidence, and may induce clinical symptoms of varied gravity ranging from clinical silence to acute abdomen. We seized the opportunity of this rare clinical observation to review the nosology of vitelline duct defects at the light of embryologic data.

[Embryonal rhabdomyosarcoma diagnosed antenatally]. / Rhabdomyosarcome embryonnaire de découverte anténatale.

A case of congenital embryonal rhabdomyosarcoma of the right shoulder is described. The patient was the first child born to a 24 year-old woman who had previously been treated for sterility. The diagnosis was made by echography during the 36th week of gestation. This full term white boy was born by cesarean section because of the tumor size. Surgical treatment was completed by chemotherapy. Despite 4 courses of VAC, local recurrence was noted that led to a second surgical excision followed by a new cyclic chemotherapy (IVA). The treatment was fairly well tolerated. The child is well 24 months later.

Ectopic vestigial lesions of the neck and shoulders.

A series of five vestigial lesions of the shoulder and back is reported. Their derivation is discussed and in four cases a branchial rather than a bronchial origin is favoured. The fifth case is held to represent skin involvement by thyroglossal duct elements.