Surgical treatment in a patient with multiple implanted intraperitoneal metastases after resection of ruptured large hepatocellular carcinoma

We report a case with multiple disseminated intraperitoneal metastases caused by the rupture of large hepatocellular carcinoma. A 52-year-old man was admitted due to sudden abdominal pain with progressive abdominal distention. Computed tomography showed a 5x7-cm low-attenuation mass in the right hepatic lobe and hemoperitoneum was demonstrated. He was taken for emergency hepatic angiography and hepatic arterial chemoembolization was done. Ten days after embolization, right lobectomy was performed successfully. Microscopic examination confirmed hepatocellular carcinoma. After 4 months, follow-up computed tomography showed a 2-cm-sized irregular-shaped mass at the right great omentum. A second surgery for omentectomy with mass excision was performed. Three months later, splenectomy and segmental resection of the colon was performed. Five months later, metastatic lymph node was detected around the head of the pancreas. Mass excision was then performed. Microscopically, all resected tumors were confirmed as metastatic hepatocellular carcinoma. The patient underwent resection of multiple intraperitoneal metastases three times for 1 year after resection of ruptured hepatocellular carcinoma and is currently disease free for 15 months. Hepatic resection is the treatment of choice for ruptured hepatocellular carcinoma but dissemination of tumor cells in the peritoneal cavity should be kept in mind. Even if intraperitoneal metastases develop, long-term survival could be possible with aggressive surgical treatment.     

Hepatic Angiomyolipoma Mimicking Hepatocellular Carcinoma: Magnetic Resonance Imaging and Clinical Pathological Characteristics in 9 Cases

Hepatic angiomyolipoma (HAML) is a rare mesenchymal tumor of the liver with marked histological diversity. The present study was to review the magnetic resonance imaging (MRI) and clinical pathological features of HAML resembling hepatocellular carcinoma (HCC).Nine patients who underwent surgical resection and had pathological diagnosis of HAML were retrospectively analyzed.All of 9 patients (5 males and 4 females) had a solitary hepatic mass with a median size of 4 cm (from 1.4 cm to 15.3 cm). Seven cases were identified as incidental liver tumors during health screening and 2 patients were diagnosed for hepatic mass when visited hospitals with unspecific abdominal discomfort. Before resection, 6 cases were diagnosed as HCC on MRI. MRI on chemical shift imagings showed a large amount of lipids in 5 cases. The enhancement pattern of MRI was classified into 2 types: in 2 cases, lesions with small or no vessels that demonstrated prolonged enhancement (1 mixed subtype and 1 myomatous subtype) and in 7 cases, lesions with abundant central vessels that show rapid washout (3 mixed subtypes and 4 myomatous subtypes) in the portal venous/delayed phase. All patients underwent resection of hepatic tumor and no recurrence was observed during follow-up (range: 2–24 months) of median 10 months. By immunohistochemistry, the tumor cells demonstrated positive immunostaining for human melanoma black-45, smooth muscle actin, and CD34.In conclusion, all of 9 patients with HAML presented with none or nonspecific clinical manifestations. The diagnosis of HAML relies on disease and immunohistochemistry, but not MRI due to its resemblance to HCC.     

Gastric gastrointestinal stromal tumor smaller than 20 mm with liver metastasis

There have been no reports of gastric gastrointestinal stromal tumors (GISTs) <20 mm with distant metastasis. We report a case of a 15-mm gastric GIST with liver metastasis 1 year after surgical resection of the primary lesion. A 35-year-old man underwent routine esophagogastroduodenoscopy in July 2009. A submucosal tumor (SMT) <20 mm was incidentally detected at the posterior wall of the gastric body. Endoscopic ultrasound (EUS) indicated that it was a gastrointestinal mesenchymal tumor, including GIST, leiomyoma or schwannoma. He did not accept regular follow-up for this gastric SMT, therefore local laparoscopic excision was carried out in October 2009. The final pathological diagnosis after surgery was GIST, 15 mm in size, and a mitotic rate of 7/50 high-power fields, which did not indicate a high metastatic risk. The patient was followed up regularly without adjuvant chemotherapy. At 1 year after surgery, a space-occupying lesion ~15 mm was detected in the left lobe of the liver by abdominal ultrasound, where no mass lesion had been observed before surgery. To make a definite diagnosis of the hepatic mass lesion, EUS-guided fine-needle aspiration was performed, which demonstrated a metastatic liver tumor from a gastric GIST. Although this was a rare case, we should keep in mind that gastric GISTs do have a chance of malignant behavior, even if <20 mm.     

Hepatic angiomyolipoma growing to cause epigastric discomfort: a case report

We report herein a case of hepatic angiomyolipoma growing to cause epigastric discomfort. A 33-year-old man was referred for excisional operation of hepatic hypervascular tumor, complaining of epigastric discomfort. The tumor had enlarged from the time 3 years before when the tumor was diagnosed as hepatic hemangioma in the lateral segment. Partial hepatectomy with tumor resection was carried out. Pathological diagnosis was confirmed as hepatic angiomyolipoma with a finding of enriched vessels, extramedullary hematopoiesis, fatty tissue, and most epithelioid cells immunoreactive to HMB-45. Further analysis also revealed positive staining cells for granulocyte colony-stimulating factor. It was suggested that growth of hepatic angiomyolipoma in this case might be caused by extramedullary hematopoiesis induced by granulocyte colony-stimulating factor.     

Spontaneous Rupture of Hemangioma of the Liver: Treatment with Transcatheter Hepatic Arterial Embolization

A case of spontaneous rupture of a cavernous hemangioma of the liver with a successful hepatic resection after transcatheter hepatic arterial embolization is reported. Twenty-eight cases of spontaneous rupture of hepatic hemangioma have been reported in the medical literature. Each of the cases (19 adults and nine children) were reviewed, including the present one reported here. Surgical treatments were carried out on 20 patients, of whom only five survived. Ruptured hemangiomas ranged in size from 3.0 to 25.0 cm, and many were located on the inferior surface of the liver. Surgical resection should be considered for a large hepatic hemangioma located on the inferior surface of the liver if the tumor has a high probability of rupture. Once the hemangioma has ruptured, emergent hepatic resection is recommended for low-risk patients, whereas high-risk patients should receive transcatheter hepatic arterial embolization prior to elective hepatic resection.     

Hepatic carcinosarcoma with heterogeneous carcinomatous and sarcomatous elements: report of a case and review of the literature

We report a rare case of hepatic carcinosarcoma consisting of two carcinomatous components and four sarcomatous components. A 54-year-old Japanese man presented with sudden right upper abdominal pain. Computed tomography showed a hepatic tumor measuring 17 cm in the greatest dimension and intractable ascites, suggesting rupture of hepatocellular carcinoma (HCC). Transcatheter arterial embolization was repeated at our hospital, resulting in stabilization of the patient’s general condition. Right anterior and left median sectionectomies were performed. Histologically, the tumor consisted of carcinomatous and sarcomatous elements. The carcinomatous elements consisted of moderately differentiated HCC (positive for hepatocyte antibody, murine monoclonal anti-cytokeratin antibody, and alpha-fetoprotein and negative for S-100 and desmin) and adenocarcinoma (negative for cytokeratin 7 and positive for carcinoembryonic antigen). Sarcomatous elements consisted of undifferentiated spindle cell sarcoma, rhabdomyosarcoma with acidophil striated cells including striped cells (desmin-positive), chondrosarcoma with cartilage matrix (S-100-positive), and osteosarcoma with osteoid. The sarcomatous elements were negative for epithelial markers. The patient is alive at 34 months after the operation with peritoneal dissemination. A total of 23 cases, including the present case, of hepatic carcinosarcoma have been reported in the English language literature. This is the third reported case of hepatic carcinosarcoma with heterogeneous carcinomatous and sarcomatous elements.     

Primary squamous cell carcinoma of the liver concomitant with primary colon cancer: report of a case

A 55-year-old Japanese female was admitted to our hospital to treat colon cancer. Computed tomography revealed a 2.6 × 2.0 cm liver mass considered to be liver metastasis. She synchronously underwent right colectomy with D3 lymph node dissection and subsegmentectomy 8 under the diagnosis of advanced colon cancer with liver metastasis. The pathology examination revealed the liver nodule was pure squamous cell carcinoma (SCC), whereas histology of colon cancer was a well differentiated tubular adenocarcinoma containing no squamous component. The patient underwent intensive checkup by imaging for a primary site of SCC. However, no lesion considered as possible primary site of SCC was found. Therefore, the liver nodule was finally diagnosed as a primary hepatic SCC. Primary SCC of the liver is a rare and high-grade malignant tumor. Recurrent multiple liver nodule was found at 13 months after surgery and the patient died of cancer 17 months after surgery.     

肝血管平滑肌脂肪瘤43例临床分析

目的 研究肝血管平滑肌脂肪瘤(HAML)临床、影像学、病理及免疫组织化学生表现,旨在提高其诊治水平.方法 回顾性分析2009年6月-2011年6月第二军医大学东方肝胆外科医院43例经手术病理证实HAML病例的临床、影像学、病理和免疫组织化学资料.结果 本组HAML患者,女性多见,大部分无HBV感染背景,肿瘤标志物[甲胎蛋白(AFP)、癌胚抗原(CEA)、CA-199]阴性,临床症状无特异性;影像学表现多样,术前诊断为HAML的8例,误诊为肝细胞癌17例,误诊为局灶性结节样增生(FNH)的2例,误诊为肝腺瘤2例,误诊为血管瘤2例,诊断不明确的12例;明确诊断依赖于术后病理及免疫组织化学结果,HMB45阳性率97.6％,SMA阳性率88.1％,CD34阳性率70.7％.结论 HAML术前容易误诊,误诊为肝细胞癌多见,结合临床与影像学检查,可能提高其术前诊断率;该肿瘤术后预后良好.     

Clinical characteristics and outcomes of patients with hepatic angiomyolipoma: A literature review

First reported in 1976, hepatic angiomyolipoma (HAML) is a rare mesenchymal liver tumor occurring mostly in middle-aged women. Diagnosis of the liver mass is often incidental on abdominal imaging due to the frequent absence of specific symptoms. Nearly 10% of HAMLs are associated with tuberous sclerosis complex. HAML contains variable proportions of blood vessels, smooth muscle cells and adipose tissue, which renders radiological diagnosis hazardous. Cells express positivity for HMB-45 and actin, thus these tumors are integrated into the group of perivascular epithelioid cell tumors. Typically, a HAML appears on magnetic resonance imaging (or computed tomography scan) as a hypervascular solid tumor with fatty areas and with washout, and can easily be misdiagnosed as other liver tumors, particularly hepatocellular carcinoma. The therapeutic strategy is not clearly defined, but surgical resection is indicated for symptomatic patients, for tumors showing an aggressive pattern (i.e., changes in size on imaging or high proliferation activity and atypical epithelioid pattern on liver biopsy), for large (> 5 cm) biopsy-proven HAML, and if doubts remain on imaging or histology. Conservative management may be justified in other conditions, since most cases follow a benign clinical course. In summary, the correct diagnosis of HAML is challenging on imaging and relies mainly on pathological findings.     

A case of adrenal rest tumor of the liver: Radiological imaging and immunohistochemical study of steroidogenic enzymes

A case of adrenal rest tumor in the liver of a 67‐year‐old female was reported. The tumor appeared on diagnostic imaging as a hypervascular and fat‐containing mass at the periphery of right hepatic lobe and required differential diagnosis from hepatocellular carcinoma (HCC). Pathological diagnosis of the intraoperative frozen section taken under laparoscopic liver biopsy could not exclude fat‐containing HCC. Therefore, she underwent partial resection of the right hepatic lobe. A yellowish‐brown mass, 17 × 14 × 11 mm in a diameter, was located in the subcapsular portion. Histologically, the mass presented features similar to those of the adrenal cortex and was composed of pale cells. Immunohistochemically, the mass expressed the adrenal 4‐binding protein and several enzymes involved in the synthesis of adrenocortical steroids. Based on these findings, the hepatic mass was finally diagnosed as an adrenal rest tumor of the liver.     

Staged hepatectomy following arterial embolization for ruptured hepatocellular carcinoma

BACKGROUND/AIMS: While hemostasis by transcatheter arterial embolization is often the first choice in the initial treatment of ruptured hepatocellular carcinoma, post-transcatheter arterial embolization treatment has not fully been established. We studied the prognoses of ruptured hepatocellular carcinoma cases where hepatectomy was possible after transcatheter arterial embolization. METHODOLOGY: We retrospectively reviewed 10 cases of ruptured hepatocellular carcinoma which had been treated in our institution between 1989 and 1998. In all the 10 cases, emergency transcatheter arterial embolization was performed, which successfully achieved hemostasis. RESULTS: Following the achievement of hemostasis by transcatheter arterial embolization, hepatectomy was carried out in 5 cases after evaluation of general condition, functional liver reserve and extent of tumor spread. There was neither operative nor hospital death. One-year and 3-year survival rates were 100% and 40%, respectively, and 50% survival time was 36 months. In the other 5 patients, hepatectomy was decided to be impossible after evaluation of general condition, functional liver reserve and extent of tumor spread; all of them died within 0.5-10 months after transcatheter arterial embolization. CONCLUSIONS: Among the patients with ruptured hepatocellular carcinoma, those in which hepatectomy was decided to be possible after evaluation of general condition, functional liver reserve and extent of tumor spread, following successful hemostasis by transcatheter arterial embolization, had fairly good prognoses.     

Adult-onset diffuse nesidioblastosis causing hypoglycemia

We report the case of a 32-year-old male with adult-onset diffuse nesidioblastosis causing hypoglycemia. Under the tentative diagnosis of insulinoma, localization procedures were carried out but no tumor was found. The presence of an insulinoma in the tail of the pancreas was suggested by selective intra-arterial calcium stimulation with hepatic venous sampling (ASVS). A distal pancreatectomy was performed under the assumed diagnosis of insulinoma in the tail based upon the ASVS. Diffuse nesidioblastosis was diagnosed by histopathological evaluation. During the post-operative course, the patient’s glucose and insulin levels were well controlled and uneventful without any medications or insulin for 7 months.     

A survival case of hepatocellular carcinoma with splenic metastasis: metachronous rupture at the liver primary and splenic metastatic lesion]

A 78-year-old man was referred to our hospital in March 2003 for rupture of hepatocellular carcinoma (HCC). Hemostasis was obtained by emergency angiography. In December 2004, metastasis to the right lung appeared and right lower lobectomy was carried out. In October 2005, a splenic metastatic lesion ruptured and hemostasis was obtained by emergency partial splenic embolization (PSE). Since viable remnants of the splenic tumor were suspected by CT, splenectomy was subsequently performed. He has been followed up in the outpatient clinic without recurrence. This is a markedly rare case of HCC in which, metachronous rupture primary and metastatic lesions, the patient was saved.     

Hepatocellular adenoma in a woman who was undergoing testosterone treatment for gender identity disorder

A 32-year-old Japanese woman was admitted to our hospital for the diagnosis and treatment of multiple liver tumors. She had been receiving 125 mg testosterone enanthate every 2 weeks following female-to-male gender identity disorder (GID) diagnosis at 20 years of age. Ultrasonography, computed tomography, and magnetic resonance imaging showed 11 hepatic nodular tumors with a maximum diameter of 28 mm. Liver tumors with hepatocellular adenoma (HCA) were diagnosed with needle biopsy. Segmentectomy of the left lateral lobe including two lesions, subsegmentectomy of S6 including two lesions, enucleation of each tumor in S5 and S7, and open surgical radiofrequency ablation for each tumor in S4 and S7 were performed. Immunohistochemical specimens showed that the tumor cells were diffusely and strongly positive for glutamine synthetase and that the nuclei were ectopically positive for β-catenin. Thus, the tumors were diagnosed as β-catenin-activated HCA (b-HCA). Transcatheter arterial chemoembolization plus subsequent radiofrequency ablation was performed for the 3 residual lesions in S4 and S8. Although testosterone enanthate was being continued for GID, no recurrence was observed until at least 22 months after the intensive treatments. HCA development in such patients receiving testosterone should be closely monitored using image inspection.     

Resection of a locally advanced hilar tumor and the hepatic artery after stepwise hepatic arterial embolization： A case report

We herein report a case of a hilar tumor with extensive invasion to the proper hepatic artery, which was successfully treated with a radical resection in a 57-year-old female patient after a stepwise hepatic arterial embolization. She underwent right colectomy and partial hepatectomy for advanced colon cancer two years ago and radiofrequency ablation therapy for a liver metastasis one year ago, respectively. A recurrent tumor was noted around the proper hepatic artery with invasion to the left hepatic duct and right hepatic artery 7 mo previously. We planned a radical resection for the patient 5 mo after the absence of tumor progression was confirmed while he was undergoing chemotherapy. To avoid surgery-related liver failure, we tried to promote the formation of collateral hepatic arteries after stepwise arterial embolization of the posterior and anterior hepatic arteries two weeks apart. Finally, the proper hepatic artery was occluded after formation of collateral flow from the inferior phrenic and superior mesenteric arteries was confirmed. One month later, a left hepatectomy with hepatic arterial resection was successfully performed without any major complications.     

Five years and 4 months of recurrence-free survival in hepatic angiosarcoma

A 54-year-old woman was referred with the diagnosis of hepatic angiosarcoma, made by percutaneous biopsy under ultrasonographic guidance. Ultrasonography (US) had revealed a 48 × 42 × 35?mm mass in the right lobe. Standard biochemical tests and whole blood count had yielded normal results. At our institution, magnetic resonance imaging demonstrated a hypervascular mass in the right lobe. Alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 levels were normal. Serological tests were negative for hepatitis B and C viruses. There was no evidence of metastasis. A right hepatectomy was performed. Histopathological examination confirmed the diagnosis of angiosarcoma. However, there was a suspicion of microscopically positive margins. Relaparotomy and resection of a 1-cm-thick slice of hepatic parenchyma was performed. Histopathological examination revealed necrotic tumor cells at the previous margin. The new surgical margin was tumor free. Due to the expected poor prognosis, prophylactic chemoembolization of the remnant liver (lipiodol + adriamycin + mitomycin) was performed at 3 and 7 months postoperatively. She has been alive without recurrence for 5 years and 4 months. Hepatic angiosarcoma has two distinct presentations: multiple tumors and a solitary tumor. The reported poor results largely stem from the predominance of the multiple tumors and consequent unresectability. Long-term survival is possible in solitary resectable hepatic angiosarcomas.     

Early venous return in hepatic angiomyolipoma due to an intratumoral structure resembling an arteriovenous fistula

Early venous return (EVR) is an important radiological feature of hepatic angiomyolipoma (HAML) that can aid in differential diagnosis, but the pathogenic mechanisms of EVR have yet to be elucidated. We present the first HAML case for which a probable mechanism for EVR is described. The patient was a 46‐year‐old woman, who had a growing 6‐cm tumor with EVR in segment 3 of the liver as revealed by dynamic contrast‐enhanced computed tomography. Left hepatic lobectomy was performed to prevent tumor rupture. Histopathological and immunohistochemical analyses of the excised tumor indicated HAML. Successive microsections of the tumor were stained with hematoxylin–eosin and Victoria blue to visualize the vascular structure within and around the tumor. These analyses led to three major findings. First, many well‐defined thick‐walled vessels, such as arteries, were found entering the tumor. Second, many thick‐walled vessels within the tumor were connected directly to thin‐walled vessels, resembling arteriovenous fistulae. Finally, thin‐walled intratumoral vessels were connected directly to the hepatic vein. These histological findings suggested that the rich arterial flow into the tumor was being rapidly drained into the hepatic vein through intratumoral arteriovenous connections. We also detected these same anomalous circulatory pathways in tissue sections from three of four additional HAML cases with EVR. Aberrant arteriovenous fistulae within the tumor may account for many cases of EVR in HAML patients.     

Long-term surviving patient with inferior vena cava tumor thrombus and extrahepatic metastasis after spontaneous ruptured hepatocellular carcinoma

We present a case of long-term survival in a patient with inferior vena cava tumor thrombus (IVCTT) and extrahepatic metastasis after resection for spontaneous ruptured hepatocellular carcinoma (HCC). The patient was a 73-year-old Japanese man previously diagnosed with chronic hepatitis B. He was referred to our emergency room and diagnosed with spontaneous ruptured HCC. The patient was immediately treated with transcatheter arterial embolization, and we then performed second-stage hepatic resection 50 days later. Although des-gamma-carboxy prothrombin was reduced to a normal level after hepatectomy, it gradually increased and computed tomography showed a disseminated tumor in the diaphragm near S2 of the liver with IVCTT and right atrium tumor thrombus. Recurrent HCC was treated with monthly transcatheter arterial infusion chemotherapy (TAI) and conformal radiotherapy (RT) of 40 Gy. After TAI and RT procedures, the disseminated tumor and IVCTT completely disappeared. Four years after TAI and RT procedures, the tumors were well controlled with no local recurrence. About 6−7 years after spontaneous ruptured HCC, lung metastasis and spleen metastasis were detected and resected, respectively. The patient is still alive and doing well over 7 years after spontaneous ruptured HCC.     

Presumed primary malignant melanoma of the liver: a case report

A 55-year-old woman was hospitalized with a palpable and painful mass in her upper abdomen. Abdominal computed tomography revealed a 10 × 7-cm tumor extending into the abdominal cavity from the left hepatic lobe and multiple metastatic lesions in the right hepatic lobe. A left hepatic lobectomy was performed for debulking and palliative resection. Histopathological examination of the resected specimen diagnosed the large hepatic tumor in the left hepatic lobe as a malignant melanoma. Physical and radiological examinations were performed on dermatological, ophthalmic, gynecological, and central nervous system areas, and endoscopic examinations were performed on the upper digestive tract and colon. No other lesions were disclosed as possible primary tumors for the disease. This result suggested that the tumor might arise from the left hepatic lobe.     

A case of a ruptured pheochromocytoma with an intratumoral aneurysm managed by coil embolization

Although the spontaneous rupture of adrenal pheochromocytoma is rare, it can be lethal because it can induce serious changes in the circulation. We describe a 32 year old man with bilateral pheochromocyroma presenting as abdominal pain. In the emergency room, an abdominal MRI showed an aneurysmal vessel in the right adrenal mass and accompanying hemorrhage around the tumor capsule. The bleeding site was found by transfemoral abdominal angiography. Coil embolization was done in the bleeding vessels, specifically branches of the right adrenal artery. The hemorrhage was successfully controlled and vital signs of the patient were restored. Following emergency care, biochemical and imaging studies showed compatible findings of a bilateral adrenal pheochromocytoma. Postoperative histologic findings confirmed these observations. A ruptured pheochromocytoma should be considered as a cause of acute abdomen in cases of a concomitant adrenal mass. Intratumoral aneurysmal bleeding may be a cause of ruptured tumor, and careful angiographic intervention will help to ensure safe control of bleeding in such an emergency situation, even in cases of bilateral tumor.