Undifferentiated (embryonal) sarcoma of the liver complicated with rupture in a child

Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare hepatic tumor and mainly occurs in children. It usually has cystic changes on computed tomography scan, which represents areas of necrosis and hemorrhages. UESL is a rapidly growing tumor and tumor rupture does occur. We reported a girl with a large multicystic hepatic tumor who died of tumor rupture shortly after the diagnosis of UESL. It is mandatory to be aware of this potentially fatal complication especially in patients with UESL of large tumor volume.     

A single‐center experience with undifferentiated embryonal sarcoma of the liver

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare aggressive mesenchymal pediatric tumor. Previously, reported outcomes have been very poor. Here, we report a single‐center experience of five patients with UESL treated with upfront gross total resection and adjuvant chemotherapy. We have a median follow‐up of 8 years with a range from 5 to 19 years with 100% event‐free survival.     

Undifferentiated sarcoma of the thyroid in a child

The most common malignant tumor of the thyroid is papillary carcinoma. Sarcoma of the thyroid is encountered very rarely; its therapy is complex and poses significant problems due to the problematic location of the tumor. A 14‐year‐old female was diagnosed with undifferentiated sarcoma of the thyroid and received combined therapy comprising surgery, chemo‐ and radiotherapy without significant side effects. This case underlines the fact that undifferentiated thyroid sarcoma may be a diagnostic possibility in children with malignant masses of the neck and may be successfully treated with modern therapeutic strategies. Pediatr Blood Cancer 2010;54:1038–1040 © 2010 Wiley‐Liss, Inc.     

Favorable response to soft tissue sarcoma therapy in an adolescent with embryonal renal sarcoma

Embryonal renal sarcomas were first identified in 1995 among banked tumor samples originally classified as adult Wilms tumor. Few long-term remissions were observed when these rare tumors were treated with chemotherapy usually used for childhood Wilms. Data were collected from the medical record of an adolescent female with embryonal renal sarcoma and treated with sarcoma-directed chemotherapy and radiation. At 66 months following diagnosis, the patient has no evidence of tumor but has experienced severe renal dysfunction and ovarian failure. We believe there is a subset of patients with disseminated embryonal renal sarcoma that respond to intense sarcoma-directed therapy.     

儿童肝脏未分化胚胎性肉瘤8例临床分析及文献复习

目的分析儿童肝脏未分化胚胎性肉瘤(UESL)的临床特征、治疗方案及预后。方法回顾分析2012年11月至2019年6月收治的8例UESL患儿的临床资料,采用Kaplan-Meier生存分析法计算生存率。结果 8例患儿中,男性5例、女性3例,中位发病年龄7岁(8个月～10岁);8例患儿的肿瘤均位于肝右叶,中位最大径为13.5 cm(10～20 cm),超声检查均显示为混合回声为主的肝内囊实性肿块。所有患儿手术完整切除肿块,3例术前肿瘤破裂。术后均接受顺铂/长春新碱/阿霉素/环磷酰胺(AVCP)方案与异环磷酰胺/依托泊苷/长春新碱(IEV)交替化疗6个周期。患儿中位随访时间47(24～90)月,7例完全缓解,1例死于复发;5年无事件生存率(EFS)为(79.25±9.31)%。结论 UESL患儿术前可出现肿瘤破裂,完整切除手术联合化疗能获得长期无病生存,少数复发患儿预后不佳。     

Undifferentiated embryonal sarcoma of the liver: US and CT findings

Six cases of undifferentiated embryonal sarcoma (UES) were reviewed to determine their characteristic features on ultrasonography (US) (n=5) and computed tomography (CT) (n=6). US demonstrated a single large, echogenic mass with some anechoic spaces. Contrast-enhanced CT scan revealed a well-demarcated low-attenuation mass with hyperdense septations of variable shape and thickness. Discrepancy of internal architecture on US and CT was one of the important characteristics of UES. CT numbers were 25–47 HU in low-attenuation areas. Enhancing peripheral rim was found in four cases and some solid portions at the periphery or adjacent to the septa were found in all cases. Two patients who had follow-up US and CT without treatment showed enhancing solid portions, changing to hypodense as the tumor grew. When compared with the pathologic findings, US showed a more accurate representation of internal architecture than did CT. Familiarity with these US and CT findings of UES of the liver will be helpful in the differential diagnosis of primary hepatic tumors in childhood.     

A child with undifferentiated sarcoma of the liver complicated with bronchobiliary fistula and detected by hepatobiliary scintigraphy

Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignant mesenchymal tumor principally affecting patients of pediatric age. Bronchobiliary fistula is a very rare complication in patients with liver tumor. To the authors' knowledge, this is the first report of a bronchobiliary fistula resulting from tumor invasion in a child with liver sarcoma. A 12-year-old boy was diagnosed to have USL of the right liver lobe, invading the diaphragm. An extended right hepatectomy and total resection of the mass was performed, leaving patchy tumoral invasion of the anterior diaphragmatic surface followed by combined chemotherapy regimen. Six months after the operation, the presence of bilious sputum suggested a bronchobiliary fistula, which was confirmed by hepatobiliary scintigraphy. The patient underwent a right thoracotomy and fistula division. Although bronchobiliary fistula is a very rare complication in patients with hepatic tumors, suspicion in the appropriate clinical setting is necessary to recognize this problem. Hepatobiliary scintigraphy is the useful diagnostic procedure to define bronchobiliary fistula in children with liver tumor and clinical suspicion of bronchobiliary fistula.     

Undifferentiated Embryonal Sarcoma with Unusual Features Arising within Mesenchymal Hamartoma of the Liver: Report of a Case and Review of the Literature

Undifferentiated embryonal sarcoma (UES) is a rare and highly malignant hepatic neoplasm, affecting almost exclusively the pediatric population. It has replaced malignant mesenchymoma, under which diagnostic term the first three cases were described. A link between embryonal sarcoma and mesenchymal hamartoma of the liver (MHL) has long been proposed, because of clinicopathologic overlaps of these entities; however, until recently, this association remained tenuous. Cases of UES arising in a background of mesenchymal hamartoma of the liver have previously been reported in two teenage girls. Discovery of a similar genetic abnormality in MHL and UES has clinched the supposed link between them. There have also been two reports of UES with prominent cystification, one associated with peripheral eosinophilia, and thereby masquerading as hydatid cyst of the liver. We report a case of UES arising in a young boy with MHL, with unusual histologic features, including large mesothelial-lined cysts and ectopic adrenal cortical tissue under Glisson's capsule. Received May 19, 2000; accepted January 31, 2001.     

Outcome of patients with undifferentiated embryonal sarcoma of the liver treated according to European soft tissue sarcoma protocols

Background

To assess the outcomes of pediatric patients with undifferentiated embryonal sarcoma of the liver (UESL) and treatment including at least surgery and systemic chemotherapy.

Methods

This study included patients aged up to 21 years with a pathological diagnosis of UESL prospectively enrolled from 1995 to 2016 in three European trials focusing on the effects of surgical margins, preoperative chemotherapy, use of radiotherapy (RT), and chemotherapy.

Results

Out of 65 patients with a median age at diagnosis of 8.7 years (0.6–20.8), 15 had T2 tumors, and one had lymph node spread, 14 were Intergroup Rhabdomyosarcoma Study (IRS) I, nine IRS II, 38 IRS III, and four IRS IV. Twenty-eight upfront surgeries resulted in five operative spillages and 11 infiltrated surgical margins, whereas 37 delayed surgeries resulted in no spillages (p = .0119) and three infiltrated margins (p = .0238). All patients received chemotherapy, including anthracyclines in 47. RT was administered in 15 patients. With a median follow-up of 78.6 months, 5-year overall and event-free survivals (EFS) were 90.1% (95% confidence interval [CI]: 79.2–95.5) and 89.1% (95% CI: 78.4–94.6), respectively. Two out four local relapses had previous infiltrated margins and two out of three patients with metastatic relapses received reduced doses of alkylating agents. Infiltrated margins (p = .1607), T2 stage (p = .3870), use of RT (p = .8731), and anthracycline-based chemotherapy (p = .1181) were not correlated with EFS.

Conclusions

Multimodal therapy improved the outcome of UESL. Neoadjuvant chemotherapy for pediatric patients increases the probability of complete surgical resection. The role of anthracyclines and RT for localized disease remains unclear.     

Undifferentiated (Embryonal) Sarcoma of the Liver: Ultrastructure, Immunohistochemistry, and DNA Ploidy Analysis of Two Cases

We describe the histopathologic, immunohistochemical, electronmicroscopic features, and DNA flow cytometric analysis of 2 cases of undifferentiated (embryonal) sarcoma of the liver. The tumor cells were found to be uniformly nonreactive to antibodies that identify cells belonging to the macrophagic-monocytic system. Focal expression of cytokeratin was an unexpected finding. We conclude that the observations are compatible with origin of this sarcoma from primitive mesenchyme. Uncertainty still exists about histogenesis, but the morphologic features of this neoplasm are quite characteristic. Both tumors demonstrate aneuploid stem lines with high S phase. Whether these findings are prognostically significant remains to be seen.     

A sewing needle migrating into the liver in a child: case report and review of the literature

Swallowing foreign bodies is a common problem in children. Although most objects pass through the gastrointestinal tract with no untoward effect; long, sharp-pointed, or slender objects can perforate the gut. Migration of an object to the liver is extremely rare and very few cases have been reported in the literature. The aim of this study is to draw attention to this subject once again by contributing a case report of a child with a hepatic sewing needle. A survey of the literature over the period from 1971 to 2006 revealed a total of five cases of childhood hepatic sewing needle together with our case. The practical lesson illustrated by this report is that the surgeon must carefully evaluate the liver when foreign body was not found in gastrointestinal system and also perforation site was found anywhere.     

Multiple malignant and benign lesions in the liver in a child with adrenocortical carcinoma

We report 4-year-old girl who was diagnosed with adrenocortical carcinoma when she was 2 years old. At the time of diagnosis there were no metastases, but 6 months later multiple liver metastases appeared. Following intensive chemotherapy the metastases resolved completely. Multifocal lesions were detected in the liver by US 16 months later. Their morphology on US and MRI differed from the previous metastases. Histopathological examination confirmed focal nodular hyperplasia. We discuss the origin and the uncommon appearance of multifocal nodular hyperplasia in hormone-active tumours such as adrenocortical carcinoma in children.     

Fibromyxoid sarcoma in a four-year-old child: Case report and review of the literature

We present a child with a rare and chemotherapy-resistant form of soft-tissue cancer, low-grade fibromyxoid sarcoma, first noted when he was 4 years old. He is the youngest patient reported to date. An 11-year-old white male presented to The University of Texas M. D. Anderson Cancer Center's Department of Pediatrics with a 7-year history of right thigh mass and pulmonary nodules, confirmed on examination. He had undergone extensive prior chemotherapy and surgery. He received chemotherapy with high-dose cyclophosphamide (7 g/m²) and later etoposide (150 mg/m²/day × 5), with only slight shrinkage of the thigh mass and none in the lungs. Subsequently the tumor in his proximal thigh and his lung metastases were resected, and radiation therapy was administered to the thigh. His disease remained stable for 12 months, but he then developed a pleural-based metastasis on the left side and new, bilateral lung metastases also. The tumors on the left side were removed; residual disease is stable after treatment for 6 months with subcutaneous alpha-interferon-2b. Low-grade fibromyxoid sarcoma is very uncommon in children. It grows slowly and metastasizes to distant organs, chiefly to the lungs. It is resistant to conventional chemotherapy, and thus far only surgery seems to have a life-prolonging effect. Newer chemotherapeutic and possibly biologic agents should be tried in future patients, in order to find an effective way to control the disease. © 1996 Wiley-Liss, Inc.     

Undifferentiated sarcoma of the liver: a rare tumor of childhood]

Undifferentiated sarcoma of the liver is a rare primary tumor of childhood: only about 150 cases have been reported in the literature. CASE-REPORT: A 10 year-old girl was admitted because of diarrhea and weight loss. Sonography, then CT-scan and MRI showed a large tumor of the liver. COMMENTS: In the differential diagnosis of primary liver tumors in children, one should think about undifferentiated sarcoma of the liver, especially if imaging shows haemorrhagic foci and if sonography and CT/MRI display a discordant appearance. Survival has improved in the last decade due to agressive surgery and intensive chemotherapy.     

Undifferentiated (embryonal) sarcoma of the liver: ultrastructure, immunohistochemistry, and DNA ploidy analysis of two cases

We describe the histopathologic, immunohistochemical, electronmicroscopic features, and DNA flow cytometric analysis of 2 cases of undifferentiated (embryonal) sarcoma of the liver. The tumor cells were found to be uniformly nonreactive to antibodies that identify cells belonging to the macrophagic-monocytic system. Focal expression of cytokeratin was an unexpected finding. We conclude that the observations are compatible with origin of this sarcoma from primitive mesenchyme. Uncertainty still exists about histogenesis, but the morphologic features of this neoplasm are quite characteristic. Both tumors demonstrate aneuploid stem lines with high S phase. Whether these findings are prognostically significant remains to be seen.     

Coincidental occurrence of actinomycosis and Ewing's sarcoma in a child

Pulmonary consolidation, rarefaction of adjacent ribs and pleural effusion, a triad frequently considered characteristic of actinomycosis, seemed in the present case to support this serologically entertained diagnosis. However, the subsequent course of disease disclosed the most uncommon association of actinomycosis and metastatic Ewing's sarcoma of the lung. The possibility of combined occurrence of an opportunistic infection by actinomyces and malignancy, calls for open biopsy in cases not responding to penicillin treatment or with an unexpected evolution of the clinical manifestations.