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Clinicopathological study of sporadic Burkitt lymphoma in children.

Huang H, Liu ZL, Zeng H, Zhang SH, Huang CS, Xu HY, Wu Y, Zeng ST, Xiong F, Yang WP - Chin. Med. J. (2015)

Bottom Line: Polypoid BL showed a lower clinical stage (P = 0.002), and advanced clinical stage and low serum albumin level at diagnosis were associated with poor outcome (P = 0.024 and 0.053, respectively).The anatomical location, growth pattern and serum albumin level of BL were associated with biological behavior.MUM1 may be a potential adverse prognostic marker, and not associated with EBV infection status.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Jiangxi Children's Hospital, Nanchang, Jiangxi 330006, China.

ABSTRACT

Background: Non-Hodgkin lymphoma is the fourth most common malignant tumors in children, Burkitt lymphoma (BL) accounts for 30-50% of all pediatric lymphomas. The aim of this study was to investigate the clinicopathologic features, immunophenotype, Epstein-Barr virus (EBV) infection and c-myc gene rearrangement of sporadic BL in children.

Methods: Ninety-two cases of pediatric BL were retrospectively analyzed for clinical features, immunohistochemistry, EBV-encoded RNA (EBER) status by in situ hybridization and c-myc gene rearrangement by fluorescence in situ hybridization.

Results: In the 92 cases, male is predominant in sex distribution (M: F = 3.38:1). The average age at diagnosis was 4.97 years. Polypoid BL showed a lower clinical stage (P = 0.002), and advanced clinical stage and low serum albumin level at diagnosis were associated with poor outcome (P = 0.024 and 0.053, respectively). The positive expression of CDl0, B-cell lymphoma-6, MUMl and EBER were 95.7% (88 cases), 92.4% (85 cases), 22.8% (21 cases), 41.3% (38 cases), respectively. The expression of MUM1 were not associated with EBV infection status (P = 1.000). c-myc gene rearrangement was detected in 94.6% (87/92). Clinical treatment information for 54 cases was collected, 21 patients died of tumor after surgery alone, 33 patients received surgery and chemotherapy, and of which six patients died shortly afterwords (MUM1 positive expression in 3 cases, P = 0.076).

Conclusions: The anatomical location, growth pattern and serum albumin level of BL were associated with biological behavior. MUM1 may be a potential adverse prognostic marker, and not associated with EBV infection status.

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Macroscopic photograph of Burkitt lymphoma: A 4 years girl presented with a polypoid lesion in the ileum.
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Figure 1: Macroscopic photograph of Burkitt lymphoma: A 4 years girl presented with a polypoid lesion in the ileum.

Mentions: From 1984 to 2013, BL accounts for 38.98% of all lymphomas of children. Among the 92 cases, there was a male predominance in sex distribution (M:F = 3.38:1). The mean and median age at diagnosis was 4.97 and 5.50 years, respectively (ranged from 3 months to 14 years). Abdomen was the most common site (88 cases, 95.7%), followed by superficial lymph node (3 cases) and testis (1 case), 14 cases presented with a polypoid lesion in the ileum or cecum [Figure 1], the size of tumors ranged from 2 cm to 6.5 cm, 10 cases with symptoms of abdominal pain because of intussusception. Of 14 cases, eight patients were in Stage II, two patients in Stages III and IV, including one patient with liver lesions and one patient with bone marrow involvement, four patients with no stage. Polypoid BL has a lower clinical stage (P = 0.002). Among the remaining 74 abdominal patients, the mesentery (including mesenteric lymph node) with or without greater omentum involvement accounting for 71.6% (53/74), followed by ileocecum (18 cases, 24.3%) and 3 cases in colon. Clinical presentation varied according to the anatomic sites of involvement including abdominal masses, abdominal pain, distension, diarrhea, hematochezia, intussusception, as well as intestinal obstruction, were the main complains or signs. In all cases, 32 patients (34.8%) had constitutive B symptoms such as fever, night sweat, and weight loss. In the 33 patients received surgery and chemotherapy, serum albumin level <35 g/L 10 cases (four patients died of tumor), low serum albumin level at diagnosis were associated with poorer outcome (P = 0.053).


Clinicopathological study of sporadic Burkitt lymphoma in children.

Huang H, Liu ZL, Zeng H, Zhang SH, Huang CS, Xu HY, Wu Y, Zeng ST, Xiong F, Yang WP - Chin. Med. J. (2015)

Macroscopic photograph of Burkitt lymphoma: A 4 years girl presented with a polypoid lesion in the ileum.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4836256&req=5

Figure 1: Macroscopic photograph of Burkitt lymphoma: A 4 years girl presented with a polypoid lesion in the ileum.
Mentions: From 1984 to 2013, BL accounts for 38.98% of all lymphomas of children. Among the 92 cases, there was a male predominance in sex distribution (M:F = 3.38:1). The mean and median age at diagnosis was 4.97 and 5.50 years, respectively (ranged from 3 months to 14 years). Abdomen was the most common site (88 cases, 95.7%), followed by superficial lymph node (3 cases) and testis (1 case), 14 cases presented with a polypoid lesion in the ileum or cecum [Figure 1], the size of tumors ranged from 2 cm to 6.5 cm, 10 cases with symptoms of abdominal pain because of intussusception. Of 14 cases, eight patients were in Stage II, two patients in Stages III and IV, including one patient with liver lesions and one patient with bone marrow involvement, four patients with no stage. Polypoid BL has a lower clinical stage (P = 0.002). Among the remaining 74 abdominal patients, the mesentery (including mesenteric lymph node) with or without greater omentum involvement accounting for 71.6% (53/74), followed by ileocecum (18 cases, 24.3%) and 3 cases in colon. Clinical presentation varied according to the anatomic sites of involvement including abdominal masses, abdominal pain, distension, diarrhea, hematochezia, intussusception, as well as intestinal obstruction, were the main complains or signs. In all cases, 32 patients (34.8%) had constitutive B symptoms such as fever, night sweat, and weight loss. In the 33 patients received surgery and chemotherapy, serum albumin level <35 g/L 10 cases (four patients died of tumor), low serum albumin level at diagnosis were associated with poorer outcome (P = 0.053).

Bottom Line: Polypoid BL showed a lower clinical stage (P = 0.002), and advanced clinical stage and low serum albumin level at diagnosis were associated with poor outcome (P = 0.024 and 0.053, respectively).The anatomical location, growth pattern and serum albumin level of BL were associated with biological behavior.MUM1 may be a potential adverse prognostic marker, and not associated with EBV infection status.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Jiangxi Children's Hospital, Nanchang, Jiangxi 330006, China.

ABSTRACT

Background: Non-Hodgkin lymphoma is the fourth most common malignant tumors in children, Burkitt lymphoma (BL) accounts for 30-50% of all pediatric lymphomas. The aim of this study was to investigate the clinicopathologic features, immunophenotype, Epstein-Barr virus (EBV) infection and c-myc gene rearrangement of sporadic BL in children.

Methods: Ninety-two cases of pediatric BL were retrospectively analyzed for clinical features, immunohistochemistry, EBV-encoded RNA (EBER) status by in situ hybridization and c-myc gene rearrangement by fluorescence in situ hybridization.

Results: In the 92 cases, male is predominant in sex distribution (M: F = 3.38:1). The average age at diagnosis was 4.97 years. Polypoid BL showed a lower clinical stage (P = 0.002), and advanced clinical stage and low serum albumin level at diagnosis were associated with poor outcome (P = 0.024 and 0.053, respectively). The positive expression of CDl0, B-cell lymphoma-6, MUMl and EBER were 95.7% (88 cases), 92.4% (85 cases), 22.8% (21 cases), 41.3% (38 cases), respectively. The expression of MUM1 were not associated with EBV infection status (P = 1.000). c-myc gene rearrangement was detected in 94.6% (87/92). Clinical treatment information for 54 cases was collected, 21 patients died of tumor after surgery alone, 33 patients received surgery and chemotherapy, and of which six patients died shortly afterwords (MUM1 positive expression in 3 cases, P = 0.076).

Conclusions: The anatomical location, growth pattern and serum albumin level of BL were associated with biological behavior. MUM1 may be a potential adverse prognostic marker, and not associated with EBV infection status.

Show MeSH
Related in: MedlinePlus