A case of hepatoblastoma occurring in an adult

We present herein a rare case of hepatoblastoma occurring in an adult male. The patient was 22 years old and his laboratory investigations on admission showed a marked elevation of α-fetoprotein in the serum. CT scan and other examinations revealed a primary tumor, 6.5 cm in size, in the left hepatic lobe with metastasis in the head of the pancreas. Thus, left hepatic lobectomy and pancreaticoduodenectomy were performed, but metastasis to the right hepatic lobe, left lung and abdominal skin were found 2 months later. Despite repeated courses of chemotherapy with adriamycin and cisplatin, the patient died 9 months after his operation. Pathological findings revealed poorly differentiated type hepatoblastoma. A review of the literature revealed only twelve other such cases.     

Adult hepatoblastoma: a review of 47 cases

Background

Adult hepatoblastoma is a very rare and aggressive neoplasm. The aim of this study was to review the cases of adult hepatoblastoma patients.

Methods

A search of all case reports about adult hepatoblastoma from 1958 to 2016 by medical database was performed. Data about demographic information, clinical symptoms and signs, laboratory test and imaging examination, treatment and clinical outcomes were extracted and analysed.

Results

A total of 43 English articles including 47 cases of adult hepatoblastoma patients were collected. The median age was 39 years (range: 18–84 years), including 25 males and 22 females. Hepatitis B virus tests were positive in seven patients. Alpha‐fetoprotein was positive in 18 patients. Liver cirrhosis was confirmed in six cases. The median size of main tumour nodule was 15 cm (range: 6–42 cm) in 30 patients with details. Hepatectomy was undertaken in 28 cases, exploratory laparotomy in seven cases and chemoembolization and chemotherapy in two cases. Follow‐up data were available in 33 patients including 23 patients with liver resection and the median survival time was 8 months (range: 0–151 months) and the 1‐year survival rate was 39.2%. For 23 patients with liver resection, the median survival time was 15 months (range: 0.5–151 months) and the 1‐year survival rate was 54.2%. In Cox multivariate analysis, liver resection was indentified as an independent prognostic factor for prolonged survival (P = 0.001).

Conclusion

Curative liver resection can prolong survival of adult hepatoblastoma. More effective systemic therapies may need to provide a great survival benefit.     

Gastrointestinal stromal tumor of the lesser omentum in a young adult patient with a history of hepatoblastoma: Report of a case

A gastrointestinal stromal tumor (GIST) of the lesser omentum is extremely rare. This report presents a case of GIST of the lesser omentum in a 22-year-old man with a history of hepatoblastoma. Computed tomography showed an abdominal mass about 30 mm in diameter adjacent to the lesser wall of the stomach. A laparotomy showed a mass of about 27 × 24 × 20 mm in diameter originating from the lesser omentum but isolated from the stomach and the liver. Histopathology showed that the tumor was composed of spindle-shaped tumor cells with high cellularity. Immunohistochemically, the tumor was positive for KIT and CD34. An in-frame deletion was observed in the c-kit gene exon 11. Therefore, the tumor was diagnosed as a GIST originating from the lesser omentum.     

Successful resection of dermatomyositis associated with thymic carcinoma: Report of a case

We report a case of thymic carcinoma associated with dermatomyositis (DM) in a 53-year-old man. The patient presented with the characteristic features of a skin rash with Gottron’s papules, proximal muscle weakness, and increased serum levels of the muscle-associated enzymes. Comprehensive clinical examinations revealed an anterior mediastinal tumor. We resected the tumor and histological examination confirmed squamous cell carcinoma of the thymus. Thereafter, his clinical symptoms improved dramatically and his serum levels of muscleassociated enzymes dropped, indicating that the DM was a paraneoplastic phenomenon. Our search of the literature found only one other case report of DM accompanied by thymic carcinoma, and to our knowledge, this is the fi rst documented case of dramatic improvement of DM after resection of thymic carcinoma. We propose that thymic carcinoma should be added to the list of malignancies that can complicate DM as a paraneoplastic disease. The first two authors contributed equally to this study.     

Successful resection of a glomus tumor arising from the lower trachea: Report of a case

(Received for publication on Sept. 30, 1996; accepted on Mar. 4, 1997)     

Hepatoblastoma in neonates: Report of a case and review of the Japanese literature

A female infant who presented with abdominal distention and jaundice at the age of 2 days underwent resection of a large hepatoblastoma at the age of 8 days by a right trisegmentectomy. Although postoperative adjuvant chemotherapy was not given, the patient is now alive without disease 10 months after surgery. We were able to find only 10 other cases of hepatoblastoma occurring in the newborn period in the Japanese literature. Resection of the tumor was performed in seven of these patients, of whom there were five survivors, one operative death and one death due to tumor recurrence. However, none of the three patients treated conservatively survived. Thus, we suggest that in newborns with hepatoblastoma, resection of the tumor should be performed, if feasible, but believe that postoperative chemotherapy is not necessary for patients whose tumor has been completely resected.     

Benign cervical teratoma in an adult: Report of a case

Cervical teratomas are rarely encountered in adults. We report herein the case of a 21-year-old woman who was admitted to our hospital for surgical treatment of a neck tumor, 7.5×4.5×2.7 cm in size, located in the left lower pole of the thyroid. Ultrasonography (US) and computed tomography (CT) revealed a multicystic tumor. The levels of carcinoembryonic antigen (CEA), carbohydrate antigen 19–9 (CA 19-9), and squamous cell carcinoma-related antigen (SCC) in the cystic fluid were extremely elevated in contrast to the normal levels found in the serum. The tumor was completely excised and histological examination revealed it to be composed of elements derived from the three germ layers, confirming a diagnosis of benign cystic teratoma.     

Focal thymic hyperplasia in an adult: Report of a case

We report herein an unusual case of a 35-year-old Japanese man who underwent thoracotomy for a mass in the mediastinum which was found to be a well circumscribed localized tumor within the thymus. Focal thymic hyperplasia in adults is extremely rare and the clinicopathological features of this case were particularly interesting and posed great difficulty in establishing a diagnosis.     

Symptomatic perineal hernia after an abdominoperineal resection following a transsacral resection of the middle rectum: Report of a case

We herein report the unusual case of a 75-year-old woman who developed a perineal hernia of the small bowel after undergoing abdominoperineal resection following a transsacral resection of the middle rectum for rectal cancer.     

Multicystic kidney in association with hepatoblastoma —A case report—

In the absence of functional renal tissue on the right side, differentiating between a liver tumor and a renal tumor, both clinically and on routine imaging, is difficult. This clinical dilemma is highlighted here in a case report of a 2 year old girl with hepatoblastoma occurring in association with multicystic kidney. To our knowledge no other such case has been reported so far.     

Successful Resection of a Ruptured Hepatoblastoma Prior to Chemotherapy: Report of a Case

A 12-year-old boy was referred to our hospital suffering from severe anemia and liver dysfunction. The laboratory data on admission confirmed severe anemia and an elevated -fetoprotein level. Abdominal ultrasonography revealed a mass measuring 51 × 49mm in size, and abdominal computed tomography showed a low-density mass in S8 of Couinauds segment and a low-density area in S7, thus suggesting bleeding in the tumor. Right subphrenic fluid collection and perirectal fluid collection were also observed. Celiac arteriography showed a faint tumor stain fed by A5–8 but no evidence of any extravasation. A diagnosis of pediatric liver carcinoma was made, and the case was classified as T2 C3 V0 N0 M0 Stage IIIA. Although there was no evidence of bleeding during angiography, because of the high risk of rebleeding, a laparotomy was performed before chemotherapy. At operation, the tumor rupture site and hematoma appeared to be in S7, and a right lobectomy was thus performed. Ascitic fluid cytology was class V. The cut surface of the resected specimen showed a tumor measuring 51 × 49mm located in S8 and a hematoma located in S7. Histologically, the tumor was a well-differentiated hepatoblastoma. The patient was transferred to the pediatric department and treated with six courses of intravenous chemotherapy followed by peripheral blood stem cell transplantation. The outcome has been favorable, with no recurrence as of 25 months after the operation.     

Laparoscopic resection of a primary retroperitoneal mucinous cystadenoma: Report of a case

(Received for publication on July 4, 1996; accepted on Mar. 4, 1997)     

Testosterone-producing hepatoblastoma in a 3-year-old boy with precocious puberty

The syndrome of isosexual precocious puberty (PP) associated with a primary malignant hepatic tumor is rare and previously reported in only 17 cases with poor prognosis. Twelve cases are well-documented gonadotropin-producing tumors. We here describe a new case of virilizing hepatoblastoma in a 2-year-10-month-old boy with evidence of testosterone (T) production by the tumor itself, and survival with a 3 1/2-year follow-up after an extended right hepatic lobectomy. Preoperative laboratory findings showed high levels of serum alpha-fetoprotein (AFP) and T:350,000 ng/mL and 4.92 ng/mL, respectively, which normalized after surgery. There was no circulating gonadotropin nor stimulation of the hypothalamic-pituitary axis. Testicular biopsy showed neither interstitial-cell maturation nor Leydig-cell hyperplasia. Moreover, demonstration of T secretion by tumor cells and T synthesis in presence of C14 progesterone was performed in an in vitro culture system. These data seem to provide supportive evidence of a T-producing hepatoblastoma.     

Perineal hernia as a rare complication after laparoscopic abdominoperineal resection: Report of a case

We report what seems to be the second documented case of perineal hernia after laparoscopic abdominoperineal resection (APR) and describe its successful repair with transperineal intraperitoneal mesh. An 89-year-old woman complained of a large, painful perineal swelling 4 months after APR for rectal cancer. Computed tomography (CT) showed small intestine protruding through the pelvic floor into the perineal area. However, opening of the hernia sac revealed no intra-abdominal adhesions. An oval, 8 × 12 cm Bard Composix Kugel Patch (Davol, Cranston, RI, USA) was inserted into the intraperitoneal space and secured over the defect in the pelvic floor; then firmly attached to the pelvic wall with 16 interrupted nonabsorbable sutures. There has been no sign of hernia recurrence in 10 months of follow-up. We speculate that because laparoscopic surgery is minimally invasive, fewer postoperative adhesions in the abdominal cavity can result in the small bowel sliding more readily into the perineal area. Based on our experience, perineal hernia after laparoscopic APR can be repaired easily and effectively with a Composix Kugel Patch.     

Successful laparoscopic resection of a sacrococcygeal teratoma in an adult: report of a case

Sacrococcygeal teratoma is a relatively rare congenital retroperitoneal tumor in adults. The standard treatment is a complete tumor resection. This report describes the successful laparoscopic resection of a sacrococcygeal teratoma. The patient was a 27-year-old woman with a well-demarcated cystic mass, 6 cm in diameter, in the retroperitoneum overlying the anterior surface of the sacrum. The mass was resected laparoscopically. A histopathological examination showed a mature teratoma. The magnifying function of the laparoscope allowed an en bloc resection in the narrow pelvic cavity, without damaging the tumor. The aesthetic outcome was excellent. The patient remains relapse-free at 1 year 6 months after surgery.     

Laparoscopic resection for ectopic gastric mucosa of the duodenum: Report of a case

(Received for publication on Jan. 22, 1998; accepted on July 7, 1998)     

Successful resection of a liver metastasis from gastric leiomyoblastoma: Report of a case

A 20-year-old woman was referred to our hospital for detailed investigation of a gastric submucosal tumor. A leiomyoma was preoperatively diagnosed and laparoscopic-assisted enucleation was performed. The resected tumor was 4 × 3 × 1.5 cm in size and postoperative histological examination identified it as a gastric leiomyoblastoma. Therefore, a secondary resection in the form of a distal gastrectomy was carried out. No tumor cells were found in the gastric specimen or in the lymph nodes; however, 5 months after the operation, an abdominal computed tomography scan revealed a recurrence in the liver, and she was readmitted for further examinations. The lesion was diagnosed as a single liver metastasis from the gastric leiomyoblastoma and successfully resected. The histopathological findings of the liver tumor resembled those of the primary gastric tumor. Her postoperative course was uneventful and she has been well, without any evidence of recurrence, to date. Only 12 other cases of leiomyoblastoma of the stomach with liver metastasis have been reported in Japan, all of which were associated with a very poor prognosis. Therefore, patients with this unusual disease entity should be carefully followed up after resection of the primary tumor. Received: July 14, 1999 / Accepted: May 30, 2000     

Hirschsprung’s disease in an adult patient with familial occurrence: Report of a case

(Received for publication on Sept. 30, 1996; accepted on Nov. 6, 1997)     

Combination chemotherapy for unresectable hepatoblastoma in children

Combination chemotherapy (adriamycin, vincristine, cyclophosphamide, etc.) was prescribed for two patients with initially unresectable hepatoblastoma. A significant reduction in tumor size occurred, and subsequent successful resection was feasible in one, although in the other the regreesed tumor remained inoperable due to the link to the hepatic veins. The survival time for these patients was 9 months after operation and 41 months from diagnosis, respectively. Preoperative combination chemotherapy for initially unresectable hepatoblastoma facilitates surgical removal under more favorable conditions of a decreased tumor size.     

Successful management of a large bronchopleural fistula after lobectomy: Report of a case

The development of bronchopleural fistula after pulmonary resection is a well-known complication associated with a high mortality rate. We herein describe the successful management of a bronchopleural fistula using a rib and intercostal muscle in a patient with a large stump opening of 25 mm in diameter. A flap with rib and intercostal muscle is useful for large bronchopleural fistulas to avoid airway stenosis.