Alveolar soft part sarcoma: clinical, histopathological, molecular, and ultrastructural aspects

Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor occurring mainly in the adolescents and young adults. Multimodality treatment has not been effective, and excision remains the mainstay of treatment. Histopathologically, it varies little from case to case. It is composed of organoid aggregates of large polygonal cells with vesicular nuclei and abundant granular, eosinophilic cytoplasm, separated by delicate vascular channels. The line of differentiation of this unique tumor is yet undetermined, although recent advances have led to a better understanding of the genetic events underlying the pathogenesis of this tumor. The histopathological, ultrastructural, immunohistochemical, and genetic aspects of ASPS are discussed.     

Paediatric and adolescent alveolar soft part sarcoma: A joint series from European cooperative groups

Background

Alveolar soft part sarcomas (ASPS) are generally chemo‐ and radio‐resistant mesenchymal tumours, with no standardized treatment guidelines. We describe the clinical behaviour of paediatric ASPS and compare these features to previously reported adult series.

Patients and Methods

The clinical data of 51 children and adolescents with ASPS, prospectively enrolled in or treated according to seven European Paediatric trials were analysed.

Results

Median age was 13 years [range: 2–21]. Primary sites included mostly limbs (63%). IRS post‐surgical staging was: IRS‐I (complete resection) 35%, II (microscopic residual disease) 20%, III (gross residual disease) 18% and IV (metastases) 27%. Only 3 of the 18 evaluable patients (17%) obtained a response to conventional chemotherapy. After a median follow‐up of 126 months (range: 9–240), 14/18 patients with IRS‐I tumour, 10/10 IRS‐II, 7/9 IRS‐III and 2/14 IRS‐IV were alive in remission. Sunitinib treatment achieved two very good partial responses in four patients. Ten‐year overall survival (OS) and event free survival (EFS) was 78.0 ± 7% and 62.8 ± 7% respectively. Stage IV, size >5 cm and T2 tumours had a poorer outcome, but only IRS staging was an independent prognostic factor.

Conclusions

ASPS is a very rare tumour frequently arising in adolescents and in the extremities, and chemo resistant. Local surgical control is critical. ASPS is a poorly chemo sensitive tumour. For IRS‐III/IV tumours, delayed radical local therapies including surgery are essential. Metastatic patients had a poor prognosis but targeted therapies showed promising results. Pediatr Blood Cancer 2013;60:1826–1832. © 2013 Wiley Periodicals, Inc.     

Laparoscopic cholecystectomy with abdominal wall traction in pediatric patients

Laparoscopic cholecystectomy using abdominal wall traction with a winch retractor instead of a pneumoperitoneum in pediatric patients is reported. Lifting the abdominal wall by the falciform ligament is useful for laparoscopic procedures, especially in small children. Because of the elasticity of the abdominal wall in children, traction on the right upper quadrant alone allows sufficient access to perform a cholecystectomy without maintenance of a pneumoperitoneum.     

Antiangiogenic treatment as a pre-operative management of alveolar soft-part sarcoma

Alveolar soft-part sarcoma (ASPS) is a rare tumor. Cure is based solely on radical surgery. The general prognosis is poor. The tongue is an unusual site in adults, but not in children. Tumor removal can cause a severe impact on quality of life, even if reconstruction is possible. ASPS is a highly vascularized tumor and antiangiogenic therapy may have a role. We describe the use of the antiangiogenic combination bevacizumab and celecoxib in the preoperative management of a patient with an ASPS of the tongue.     

Cutaneous gonococcal abscess of the abdomen in a child

Virtually all pediatric cases of Neisseria gonorrhoeae originate from contact with an infected adult. A cutaneous abscess caused by N. gonorrhoeae in a child is extremely rare, especially outside the genital area. We report a case of a 22-month-old boy with a gonococcal cutaneous abscess on the abdominal wall and suggest that N. gonorrhoeae should be included in the differential diagnosis of skin and soft tissue infections in children.     

Laparoscopic-assisted surgical reconstruction of a rare congenital abdominal wall defect in two children misdiagnosed with prune-belly syndrome

PurposeAbdominal wall laxity is typically associated with prune-belly syndrome (PBS). Incomplete forms of PBS have been rarely reported with only the abdominal wall laxity. Herein, we describe a rare congenital abdominal wall defect that has been confused with PBS and illustrate the laparoscopic-assisted surgical technique used for reconstruction.Materials and methodsTwo boys with symmetrical, bilateral absence or hypoplasia of the internal and external oblique muscles and no genitourinary abnormalities underwent a laparoscopic-assisted abdominal wall reconstruction utilizing the technique previously described by Firlit. Each patient had a Ct scan which confirmed the absence of the oblique muscles. In one patient EMG data confirmed no electrical activity of the obliques. Radiologic evaluation of the urinary tracts revealed no abnormalities. The abdominal wall was plicated utilizing bilateral subcostal incisions.ResultsBoth patients had excellent cosmetic and functional results with no weakness or bulging of the lateral abdominal wall and improvement of associated symptoms.ConclusionsWe believe these two cases and their congenital abdominal wall defects are a rare and often misdiagnosed muscular deficiency separate from PBS. The novel laparoscopic-assisted surgical technique illustrated is feasible and highly successful for these and possible other patients with similar rare congenital abdominal wall defects.     

Monitoring for central apnoea in infancy--limitations of single channel recordings.

Apparent central apnoea (absent breathing movements) detected by monitoring movement of the thoracic wall was compared with simultaneous detection by abdominal wall movement. Eighteen infants provided one or more 24 hour recording of heart rate (electrocardiography), thoracic respiration (transthoracic impedance), and abdominal wall movement (pressure sensitive capsule distortion). Detection of true apnoea, recognition of artefact, and measurement of the duration of true apnoea were all improved when two channels of respiratory monitoring were used in combination. We recommend that any study purporting to observe breathing patterns by indirect recording of respiratory movement will be more reliable if more than one channel of respiratory movements is recorded simultaneously. Further, in infants no estimation of duration of central apnoea can be made on the basis of either a transthoracic impedance record alone or an abdominal wall movement sensor alone. Comparison of findings among studies using different single channel recordings are unlikely to be meaningful.     

Alveolar soft-part sarcoma: a rare soft-tissue malignancy with distinctive clinical and radiological features

Alveolar soft-part sarcoma (ASPS) is a rare tumour. Certain distinctive clinical and radiological features suggest the correct diagnosis. There is moderate predilection for young women. ASPS almost always arises in skeletal muscle and occurs most frequently in the lower limbs. There is often a long clinical history and a large mass at presentation. Two young females with ASPS presented with very vascular tumours in the thigh, with prominent intra- and extra-tumoural blood vessels. The imaging findings and the existing literature are reviewed. Received: 10 February 1999 Accepted: 24 April 2000     

Major abdominal wall defects in the low- and middle-income setting: current status and priorities

Major congenital abdominal wall defects (gastroschisis and omphalocele) may account for up to 21% of emergency neonatal interventions in low- and middle-income countries. In many low- and middle-income countries, the reported mortality of these malformations is 30–100%, while in high-income countries, mortality in infants with major abdominal wall reaches less than 5%. This review highlights the challenges faced in the management of newborns with major congenital abdominal wall defects in the resource-limited setting. Current high-income country best practice is assessed and opportunities for appropriate priority setting and collaborations to improve outcomes are discussed.     

Separation of the umbilical cord--histological findings

Even though the separation of the umbilical cord receives a great deal of interest, the process of cord separation has not been reported in the literature. A histological study of the umbilical area was carried out on 25 neonates who died within 7 days of birth (mean age at death 3 days). The umbilical cord itself became dried and mummified. Polymorphonuclear leucocytes infiltrated the areas between the mummifying cord stump and the vital tissues of the abdominal wall, forming a demarcation zone. No other leucocytes were present, and no bacteria were seen in any of the preparations studied.     

Abdominal mucinous cystic neoplasm in a male child.

Mucinous cystic neoplasms (MCNs) make up a morphologic family of similar appearing tumors arising in the ovary and various extraovarian sites, including the pancreas, hepatobiliary tract, paratesticular soft tissues, and mesentery. Other than the uncommon mucinous cystadenoma of the ovary presenting in adolescence, MCNs are rarely seen by the pediatric pathologist. The present case is a 5-year-old boy with an abdominal mass appearing to arise in the mesentery of the small intestine. Because of its unresectability, a generous biopsy was performed and disclosed a MCN with focal complex papillary architecture in the absence of appreciable cytologic atypia or invasion into the wall. Like other MCNs, this tumor had an inhibin-positive, ovarian-like stroma that was nonreactive for estrogen and progesterone receptors. Only 1 other case of a mesenteric MCN has been reported to date in a child and none in a male. The MCN of the mesentery joins other, somewhat more common cystic lesions of the omentum and mesentery presenting in childhood.     

Cystic lymphangioma of the abdominal wall in childhood: case report

Intra-abdominal lymphangiomas are rare benign tumours that can cause various symptoms, mainly during childhood. They are diagnosed by ultrasonography, CT scanning or at laparotomy; ultrasonographic examination often shows a voluminous tumoral cystic formation with septa. The location of the cyst may be determined either by ultrasonography alone or by CT scanning. The definitive histological diagnosis is confirmed by immunohistochemical staining techniques. Rarely intra-abdominal lymphangiomas can occur in the abdominal wall. Complete resection is the treatment of choice. The case of a 2-year-old-boy with a large lymphangioma involving the lower half of the abdominal wall is reported. The patient underwent the complete removal of lymphangioma with good cosmetic result.     

Extrathoracic Liver Hamartoma

Mesenchymal hamartoma is a benign tumor of the liver that often presents because of a palpable abdominal mass or respiratory distress. An unusual protrusion of this tumor through the chest wall of a neonate with Poland’s syndrome is reported.     

Prenatally detected gastroschisis presenting as jejunal atresia due to vanishing bowel

A case of gastroschisis complicated by vanishing bowel and presenting as jejunal atresia is reported that is uniquely different from previously reported cases. Following delivery, complete closure of the abdominal wall with a small fascial defect was observed. Complete healing of this fascial defect was observed at 1 month of age. Accepted: 5 January 1999     

The isolated bowel segment: angiographic visualization of the developing vascularity

An isolated bowel segment (IBS) is created by a two-stage procedure: (1) initial coaptation of the bowel segment to host organs such as abdominal wall muscle, liver, or intestine; and (2) division of its mesentery several weeks later. Between operations, vascular collaterals develop across the coaptation to preserve viability of the IBS. IBS motility and absorption have been observed in previous studies; this experimental study was designed to angio-graphically observe the vascular collaterals. An IBS was created in four dogs by myoenteropexy between the undersurface of the rectus abdominis muscle and a segment of jejunum or colon. Both IBS ends were exteriorized and intestinal continuity was restored. Four weeks later, the IBS mesentery was divided in three dogs and a sham operation was performed in the fourth. Three weeks thereafter, the animals were killed and appropriate arteries related to the IBS were injected with Microfil radiopaque silicone yellow rubber compound at a pressure of 150 mmHg. After the dye had polymerized, soft tissue X-ray was employed to identify vascular collaterals to the IBS. With ipsilateral deep inferior epigastric artery injection in the dogs with IBS mesentery division, the intramural vessels of the IBS filled via collaterals over the myoenteropexy. Dye injected into the IBS mesenteric artery in the fourth dog outlined the abdominal wall vessels via collaterals that had developed across the myoenteropexy. These observations suggest that the IBS is nourished by the abdominal wall vessels through vascular collaterals that have developed across the myoenteropexy during the interval between the staged procedures, and that viability of the IBS is preserved after IBS mesenteric division.     

A new case of omphalocele with absence of thumb

Omphalocele, or exomphalos, is a rare defect of the periumbilical abdominal wall in which the coelomic cavity is covered with peritoneum only, resulting in congenital eventration of abdominal contents at the base of the umbilical cord. The etiology is still unknown. In this paper a new case of omphalocele with absence of the right thumb in a five-year-old boy is reported. A common etiology and a new terminology for this combination are suggested.