This paper presents a case report of a primary lymphoma of the appendix as the underlying cause of acute appendicitis. As in previous reported cases, diagnosis can only be made intraoperatively followed by a proven histopathological picture as they present with an acute surgical abdomen.
Desmoid tumours are uncommon. They are locally invasive and incomplete excision leads to recurrence, which can pose a significant management challenge. Patients therefore require effective treatment, which essentially entails tumour excision with a clear surgical margin. The resulting wide defect may lead to difficulty in closure of the anterior abdominal wall. We report our experience in treating large desmoid tumours of the anterior abdominal wall. Between January 2000 and December 2001, three patients with large desmoid tumour of the anterior abdominal wall were treated with wide excision, which included a 3-cm margin of uninvolved tissues. This led to a considerable abdominal wall defect. The peritoneal defect was closed as a separate layer, though under considerable tension, while the abdominal wall musculature defect was closed with a polypropylene mesh. All three patients recovered well with no immediate or late postoperative morbidity. Follow-up until December 2006 has not revealed any tumour recurrence or hernia development. Wide excision of an anterior abdominal wall desmoid tumour with a clear margin of 3 cm including the peritoneum should be considered when managing such tumours. Closure under tension of the peritoneum did not seem to produce any morbidity.
We report a rare case of a giant extradural and intraabdominal ganglioneuroblastoma in a young Malay girl who presented to a paediatrician initially at 5 days of life with a palpable abdominal mass. Unfortunately, the parents refused any form of surgical intervention until the child was 3 years old. She subsequently underwent vascular embolisation followed by the removal of this large tumour both via the abdomen and through a laminectomy approach and subsequently refused chemotherapy. The c-myc amplication in this patient was absent and there were no chromosomal aberrations, During the 2 year folow-up the patient remained well, and ambulatory with no tumour recurrence.
Ultrasound examinations were done to evaluate clinically palpable abdominal masses in 125 children. The examinations were normal in 21 patients. In 15 patients, the clinically palpable masses were actually anterior abdominal wall abscesses or hematomas. Final diagnosis was available in 87 of 89 patients with intraabdominal masses detected on ultrasound. The majority (71%) were retroperitoneal masses where two-thirds were of renal origin. Ultrasound diagnosis was correct in 68 patients (78%). All cases of hydronephrosis were correctly diagnosed based on characteristic ultrasound appearances. Correct diagnoses of all cases of adrenal hematoma, psoas abscess, liver hematoma, liver abscess and one case of liver metastases were achieved with correlation of relevant clinical information.
Haemangiopericytoma (HPC) is a rare tumour with a predilection for the central nervous system. Though previously thought to originate from the meninges and ventricular walls, HPC's are currently accepted as distinct mesenchymal neoplasms unrelated to meningiomas. Haemangiopericytomas have been previously reported to be associated with the production of insulin like growth factor II (IGF-II) and hypoglycaemia. A case of a 61-year-old poorly controlled diabetic lady with a rare presentation of an abdominal haemangiopericytoma is discussed. A laparoscopic assisted resection of the massive lobulated tumour arising from the parietal peritoneum with dense attachment to the diaphragm and the liver was performed with an uncomplicated postoperative recovery. Control of the patient's diabetes improved dramatically following surgery and the rare association of hyperglycaemia and HPC, which has not been previously described, is elaborated upon in this report.
Most functional phaeochromocytomas/paragangliomas produce noradrenaline and/or adrenaline. Those that produce dopamine are rare. We describe the distinguishing clinical features of dopamine-secreting phaeochromocytomas and paragangliomas from those that secrete noradrenaline/adrenaline and the impact on their management. We present a case of a dopamine-secreting paraganglioma from our institution and review 14 case reports of dopamine-secreting phaeochromocytomas/paragangliomas published between 1984 and 2008. As observed in the literature, 80% of the tumours were extra-adrenal. Most patients presented with non-specific symptoms or mass effect without the classical presentation of catecholamine excess. The majority were diagnosed with urinary or plasma dopamine. Five patients had malignant tumours and 12 patients underwent surgical resection of the primary tumours. Unlike noradrenaline/adrenaline-secreting phaeochromocytomas/paragangliomas, dopamine-secreting tumours lack a classical presentation, are extra-adrenal and have a higher malignant potential. A routine inclusion of urinary or plasma dopamine as part of catecholamine screening in all suspected phaeochromocytomas and paragangliomas is recommended.
Extensive full thickness anterior abdominal wall defects pose a difficult challenge to the reconstructive surgeon. The objectives of reconstruction are the support of the intra-abdominal structures in order to preserve the functional integrity and achieve an aesthetically-acceptable appearance. Autologous tissues are versatile and provide the best reconstructive option in this type of defects. The tensor fascia latae myocutaneous flap provides identical abdominal wall musculofascial cover for full thickness defects. In extensive defects, the extended tensor fascia latae flap is a versatile option with a second microvascular anastomosis at the distal end of the flap. A total anterior abdominal wall soft tissue tumour resection defect was reconstructed with the use of the double pedicle extended free tensor fascia latae myocutaneous flap in a 60-year-old man. The patient however succumbed to the disease process six months post-reconstruction. During the follow-up period, there was no evidence of hernia at the anterior abdominal wall.
A ten-year review revealed a similarity in the incidence of teratoma in relation to other childhood tumors in Malaysian as compared with Caucasian children. The most common sites of origin were the sacropcoccygeal, gonadal, and retroperitoneal areas. The reason for the high incidence of retroperitoneal tumor in our series as compared with other countries is not clear. Late presentation and poor followup are associated with poor prognosis.