Pathology of pediatric liver tumors, a single center experience from south of Iran.

Background: Pediatric hepatic malignancies are rare, accounting for 1-4% of all solid childhood tumors. The histopathology of childhood hepatic tumors guides the treatment and prognosis, and is the cornerstone for precise diagnosis. Until now, there has been no documented study on pediatric liver tumor cases from this center; in this report, we show our experience about the common types of childhood hepatic tumors during five years (2002-2007) and compare them with other studies. Materials and Methods: During five years (2002-2007), all the hepatic tumors of childhood (under 18 year-old) from the pathology file of Namazi Hospital of Shiraz University of Medical Sciences are recorded. This includes both resected specimens and biopsies. All the slides were reviewed and the pathologic diagnosis was confirmed. Results: We detected 53 liver tumor cases in children (below 18 years of age). Among these tumors, 36 (67.9%) were malignant. Male to female ratio was 1.5 to 1. Hepatoblastoma was the most common liver tumor in this age group accounting for 22 patients (41.5%). The second most common primary tumor was hepatocellular carcinoma (HCC), with five patients. Another malignant tumor was embryonal sarcoma. Benign tumors included adenoma, mesenchymal hamartoma, vascular tumors, focal nodular hyperplasia, and inflammatory pseudo tumor. There were also seven metastatic tumors during these five years. Conclusions: The spectrum of hepatic tumors in children is different from that found in the older age group (adults) and also different in different populations.

Early Postoperative Complications of Pediatric Liver Transplantation.

We reviewed records of 35 pediatric liver transplant recipients who were operated at the Shiraz Organ Transplant Center between April 1998 and April 2005 to gather demographic data, primary diagnosis, duration of hospital stay, source of graft, mortality, and surgical (vascular, biliary, fluid collection) and medical (infection, respiratory, neurological, cardiovascular, and gastrointestinal) complications. Among 23 male and 12 female pediatric liver transplant recipients (mean age: 11.8±4.9 years) with a mean hospital stay duration of 23.3±20.3 days, the postoperative complications included biliary leakage (7.20%), biliary stricture (3.10%), biliary obstruction (3.10%), pleural effusion (9.26%), lung collapse (n=1) pulmonary hemorrhage (n=1), and vascular complications of portal and hepatic vasculature (n=10, 28.6%); and infections of the peritoneum, lung, wound site, and urinary tract (n=10; 28.6%). Acute cellular rejection was documented in 6 (17.1%) recipients. Overall, 13 (37.1%) children died.

Inflammatory pseudotumor of the liver: two case reports and a review of the literature.

Rare cases of inflammatory pseudotumor (IPT) in two adolescents are reported. Both of them presented with significant weight loss and were operated upon with the impression of liver abscess and malignant liver tumor. These two cases are reported to emphasize IPT of the liver as a differential diagnosis of hepatic masses in children because recognition of this condition before operation can avoid unnecessary surgery.

Role of rectal biopsy in predicting response to intrasphincteric botulinum toxin injection for obstructive symptoms after a pullthrough operation.

OBJECTIVE: Our aim was to correlate the pathological results and clinical response in patients who underwent botulinum toxin (BT) injection for obstructive symptoms (OS) after a pullthrough operation for Hirschsprung's disease (HD). METHODS: Between August 2002 and February 2006, 16 of 107 HD patients (15%) were referred with persistent OS after pull-through (PT) operation in this center. They underwent rectal biopsy and BT injection in the internal sphincter. Their responses to BT injection were evaluated by the constipation score before, and at 1, 3 and 8 months after the injection, and anorectal manometry (ARM) before and at 2 weeks, and 1 and 8 months after the injection. The association between response to BT and acetylcholinesterase (AChE) staining of rectal biopsy was also assessed. RESULTS: Fourteen of 16 patients (87%) had improvement in bowel function after 2 weeks, and two patients did not respond at all. Six of the 14 patients with early response had recurrence of symptoms after 2-3 months. Eight patients with normal ganglia and negative AChE had good response with no recurrence on follow-up. However, 4 of 6 recurrences were neurogenic dysfunctions and 2 were intestinal neuronal dysplasia (2-4+AChE). Two patients with no response had an aganglionic segment (4+AChE). Four of 6 patients with recurrence showed improvement with BT re-injection and only 2 did not improve. CONCLUSION: A higher degree of AChE staining is associated with lack of response to BT injection. This is also a test for predicting the severity of neurogenic dysfunction in the intestinal wall.

Comparison of botulinium toxin injection and posterior anorectal myectomy in treatment of internal anal sphincter achalasia.

OBJECTIVE: We compared the efficacy of intra-sphincteric botulinum toxin (BT) injection and posterior anorectal myectomy (PARM) for the treatment of internal anal sphincter achalasia (IASA). METHODS: Twenty eight of 120 patients (23%) with chronic constipation, who were referred to our clinic from September 2005 to December 2006, were evaluated. Patients had an absence of rectoanal inhibitory reflex on anorectal manometry (ARM) and showed no transitional region on barium enema. Fourteen patients each underwent rectal biopsy, and were treated with either intrasphincteric BT injection (Group I) or PARM (Group II). Nine patients were excluded because of absent ganglion cells on histology or positive acetylcholinesterase staining (AChE). The remaining 19 patients were followed up. All patients underwent ARM and constipation severity score (CSS) assessment 2 weeks before, and 1 and 6 months after the treatment. Patients were followed up telephonically at 12 months after treatment. RESULTS: Clinically good response was seen after 12 months in 3 patients each in Groups I and II. The median values of resting rectal pressure in Group I before and 6 months after BT injection were 60 mmHg and 40 mmHg (p< 0.0001), respectively, while in Group II the corresponding values were 60 mmHg and 45 mmHg (p< 0.0001), respectively. Compared to pre-treatment, median CSS improved in both Group I (14 to 13) and Group II (16 to 14) at 6 months after treatment (p< 0.0001 for both). However, there was no difference in resting rectal pressure and CSS between the groups. Three patients in Group II developed local abscess, postoperatively. CONCLUSIONS: BT injection has a similar efficacy as compared with PARM for the treatment of IASA, is less invasive and, is also associated with fewer complications.

Analysis of patients listed for liver transplantation in Shiraz, Iran.

AIM: To analyze records of patients listed for liver transplantation (LT) at our hospital, the first and the largest LT center in Iran. METHODS: We analyzed medical records of patients aged 14 years or older, who were listed for LT for chronic liver disease between 1994 and 2004. Outcome was determined from records or follow-up data. RESULTS: Among the 480 patients (mean age 39 [SD 13] years; 327 [68.1%] men) listed for LT, common causes of cirrhosis were cryptogenic (143; 29.9%) and hepatitis B (127; 26.5%). Child-Turcott-Pugh (CTP) class distribution of these patients was: A - 37 (7.7%), B - 258 (53.7%) and C - 185 (38.6%). Mean (SD) follow-up duration was 11.4 (11.8) months (range 1-108). One hundred and four (21.7%) patients received LT and 173 (36%) died while awaiting LT. CTP class influenced 1-year (90%, 73% and 55% in class A, B and C, respectively) and 2-year (84%, 48% and 25%, respectively) survival rates. MELD score also influenced survival. Survival was better in patients who underwent LT than in those who continued on the waiting list (p< 0.01). CONCLUSION: Only about one-fifth of patients listed for LT in Iran received LT and a large proportion died without LT.