Hepatocellular adenoma associated with familial adenomatous polyposis coli

Hepatocellular adenoma (HCA) is a benign liver tumor that most frequently occurs in young women using oral contraceptives. We report a rare case of HCA in a 29 years old female with familial adenomatous polyposis (FAP). The first proband was her sister, who under-went a total colectomy and was genetically diagnosed as FAP. A tumor, 3.0 cm in diameter, was detected in the right lobe of the liver during a screening study for FAP. A colonoscopy and gastroendoscopy revealed numerous adenomatous polyps without carcinoma. The patient underwent a total colectomy and ileoanal anastomosis and hepatic posterior sectoriectomy. The pathological findings of the liver tumor were compatible with HCA. The resected specimen of the colon revealed multiple colonic adenomatous polyps. Examination of genetic alteration revealed a germ-line mutation of the adenomatous polyposis coli (APC) gene. Inactivation of the second APC allele was not found. Other genetic alterations in the hepatocyte nuclear factor 1 alpha and β-catenin gene, which are reported to be associated with HCA, were not detected. Although FAP is reported to be complicated with various neoplasias in extracolic organs, only six cases of HCA associated with FAP, including the present case, have been reported. Additional reports will establish the precise mechanisms of HCA development in FAP patients.     

Renal cell carcinoma presenting as Henoch–Schönlein purpura with leukocytoclastic vasculitis, hematuria, proteinuria and abdominal pain

Some infectious, rheumatic, allergic diseases, and malignancies have been associated with leucocytoclastic vasculitis (LCV). LCV and cancer occur most frequently in patients with hematological malignancies such as lymphomas and leukemias. There have been a few prior cases reported of LCV associated with renal cell carcinoma (RCC). A 25-year-old male patient was referred from the department of dermatology and nephrology because of recurrent petechiae on both lower legs for several months; the patient also had a tumor of the left kidney. The findings on the skin biopsy were compatible with the diagnosis of LCV. The patient was diagnosed as having Henoch–Schönlein purpura (HSP) with LCV. A CT scan performed due to abdominal pain revealed a mass on the upper pole of the left kidney. A partial nephrectomy of the left kidney including the tumor was performed. The pathology report was consistent with a clear cell type of renal cell carcinoma, Fuhrman’s grade 2; the tumor measured 0.9 × 0.8 cm and focal segmental glomerulosclerosis was noted in non-neoplastic regions. Here, we report a case of LCV associated with RCC presenting as HSP. This case illustrates the importance of evaluating patients for an underlying malignancy when HSP or LCV is diagnosed.     

A mucinous cystadenoma in rectal cuff of a patient with familial adenomatous polyposis

Introduction

Familial adenomatous polyposis (FAP) may be associated with some extracolonic manifestations which in this vein, it is known as Gardner’s syndrome. To our knowledge, so far, there is no report of mucinous cystadenoma in association with FAP.

Case presentation

We report a 31-year-old woman with FAP who underwent total proctocolectomy with ileal pouch-anal anastomosis 5 years earlier. During endoscopic surveillance, she was found to have a submucosal lesion in rectal cuff.

Results

Endoscopic ultrasound (EUS) revealed a round submucosal anechoic lesion measuring about 3 cm originating from the second layer of the rectal cuff. Surgical resection was performed and a cystic tumor was removed. Histologic examination was consistent with mucinous cystadenoma.

Conclusion

FAP can be associated with mucinous cystadenoma.

     

Hepatocellular carcinoma with gastric metastasis treated by simultaneous hepatic and gastric resection: report of a case

Hepatocellular carcinoma (HCC) with gastric metastasis is extremely rare. There have been few reports on curative surgical resection for gastric metastasis of HCC. We herein report such a case successfully treated by simultaneous surgical resection. A 73-year-old male was admitted for evaluation and treatment of a liver tumor. Computed tomography showed an exophytic tumor of 170 mm in diameter located in the left lobe of the liver with poor delineation to the gastric wall. Upper gastrointestinal endoscopy revealed a submucosal tumor with ulceration in the antrum of the stomach. With a diagnosis of HCC with invasion to the gastric wall, an en bloc resection was planned, and the patient underwent laparotomy. The patients underwent left hemihepatectomy with partial resection of the stomach for adhesion and distal gastrectomy for the tumor. Pathological examination of the liver tumor revealed poorly differentiated HCC, and pathological diagnosis of the tumor in the submucosal and muscular layer of the stomach was compatible with metastasis from HCC, which was separate from the liver tumor. Therefore, we diagnosed the tumor as HCC with hematogenous gastric metastasis. The patient remains well with no evidence of tumor recurrence as of 13 months after resection.     

Laparoscopic-assisted limited liver resection: technique, indications and results

Background/Purpose

The purpose of this work was to evaluate the short-term results of laparoscopic-assisted limited liver resection.

Methods

We analyzed the clinical outcome in 17 patients (mean age 70 ± 8 years) who had undergone laparoscopic-assisted limited liver resection from March 2006 to December 2008. Preoperative diagnoses were HCC in 13 patients and metastasis of colon cancer in 4. The operation consisted of laparoscopic mobilization of the target liver lobe, followed by open liver resection through a 7- to 10-cm extraction site.

Results

Mean tumor size was 3.0 ± 1.1 cm (range 1.2–5 cm). The mean operative time was 362 ± 85 min. The mean blood loss was 451 ± 413 ml, and no blood transfusion was required in any patient. There were no intraoperative complications, and conversion to laparotomy was needed in one case. Postoperative complications developed in 4 cases (4 infections, 24%), all of which were improved by conservative management. However, there was no postoperative mortality. None of the patients had any peritoneal carcinomatosis or port-site or resection site recurrence during a mean follow-up of 18 ± 9.6 months. According to the analysis of the tumor location, the criterion for an adequate tumor location in the right lobe for this operation was set with the tumor at a distance of more than 5 cm from the inferior vena cava and the root of the hepatic vein (5 cm rule).

Conclusion

Laparoscopic-assisted limited liver resection is feasible and well tolerated. Accumulation of more data may be needed for evaluation of long-term outcome.     

Co-existence of hepatocellular adenoma and focal nodular hyperplasia in a young female

Focal nodular hyperplasia (FNH) and hepatocellular adenoma (HA) are both benign hepatocellular lesions, presenting mainly in women of childbearing age in non-cirrhotic, non-fibrotic livers. Simultaneous occur-rence of these two lesions is extremely rare. We herein report a case of a young female without any predis-posing risk factors who presented to our emergency department complaining of acute abdominal pain. Imaging studies revealed a 6 cm lesion in the right hepatic lobe and a 2.5 cm lesion in the left hepatic lobe, respectively. In view of the patient’s symptoms and lack of a confirmed diagnosis based on imaging, we performed a bisegmentectomy Ⅴ-Ⅵ and a wedge resection of the lesion in segment Ⅲ by laparotomy. Postoperative course was uneventful and the patient was discharged on the fourth postoperative day. The pathology report demonstrated an HA in segments Ⅴ-Ⅵ and FNH in segment Ⅲ, respectively. Six months later, the patient remains asymptomatic with normal liver function tests, ultrasound and magnetic reso-nance imaging follow-up. To our best knowledge, this is the first case to describe simultaneous occurrence of HA and FNH without the presence of any known risk factors for these entities. The uncertainty in diagnosis and acuteness of presenting symptoms were estab-lished criteria for prompt surgical intervention.     

A rare case of advanced esophageal adenocarcinoma with exceedingly long-segment Barrett’s esophagus

A 71-year-old male patient was admitted to our hospital with dysphagia. Upper gastrointestinal endoscopy revealed severe stenosis between 26 and 36 cm from the incisors, and biopsy specimens taken from the stenotic lesion showed well-differentiated adenocarcinoma. A long-segment Barrett’s esophagus (LSBE) between 25 and 40 cm from the incisors was diagnosed. Subtotal esophagectomy with right thoracotomy was performed. Pleural tumor dissemination was seen 7 months later and was treated with chemotherapy. However, the patient died 14 months postoperatively. In Western countries, the incidence of adenocarcinoma in Barrett’s esophagus is high, but in Japan, the incidence is very low. In Japan, the incidence of short-segment Barrett’s esophagus is high, but that of LSBE is very low. We herein report esophageal adenocarcinoma in an exceedingly long LSBE of 15 cm.     

A case of a duodenal gastrointestinal stromal tumor diagnosed with the aid of diffusion-weighted magnetic resonance imaging

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumor of gastrointestinal tract, and bleeding is among the primary symptoms. If the tumor is located in the small intestine, diagnosis might be difficult because of its inaccessibility by endoscope. This report presents a case of duodenal GIST diagnosed with the aid of diffusion-weighted (DW) magnetic resonance imaging (MRI). A 66-year-old male was admitted to the hospital because of shortness of breath and melena. The patient demonstrated marked anemia. Esophagogastroduodenoscopy and colonoscopy revealed no involvement of the mucosa of the gastrointestinal tract in regard to the observed bleeding. Thereafter, computed tomography was done and showed a tumorous lesion 4.2 cm in diameter located around the ligament of Treitz. The area showed significant high intensity by DW-MRI, strongly suggesting malignant character. Examination with a long endoscope demonstrated a large submucosal tumor with wide ulceration located at the third portion of the duodenum. The biopsy sample contained spindle-shaped cells that were positive for CD34 and c-kit on histochemistry, and the tumor was diagnosed as GIST. The patient underwent surgery and survived for 16 months without any recurrence.     

Esophageal schwannoma of the upper mediastinum: report of a case

Esophageal schwannoma is extremely rare, and it is difficult to confirm a definitive diagnosis preoperatively. We report the case of a 67-year-old male with an asymptomatic esophageal schwannoma with a major axis of 6 cm. Computed tomographic angiography was very useful for determining a safe and efficient route of access to the tumor. Although high 18F-fluorodeoxyglucose uptake and high signal were revealed by diffusion-weighted images, a malignant potential was denied by low-grade clinical symptoms and surgical findings. The patient underwent tumor excision via a cervical collar incision and median sternotomy. This procedure was very useful and was shown to be safe for tumor exfoliation from the main blood vessel and nerve.     

Giant primary liposarcoma of the stomach: a case report

Liposarcoma is the most common soft tissue sarcoma and accounts for 15%–20% of all mesenchymal malignancies. The tumor occurs most frequently in the limbs, retroperitoneum and rarely has a visceral location. We report a case of a gastric liposarcoma in a male patient who was admitted to hospital for abdominal discomfort and feeling fullness lasting for a month. The CT examination revealed a giant epigastric mass (36 cm × 34 cm) involving the wall of the stomach. A subtotal gastrectomy together was performed. The histopathological diagnosis was a myxoïd liposarcoma. The patient did not undergo any adjuvant treatment. He was under close follow up and nine months after surgery he represented with tumor recurrence. We report this case due to the rarity of this tumor in the stomach (fifteen cases reported in the literature).     

Mixed acinar-endocrine carcinoma of the pancreas treated with S-1

The case of a 63-year-old male with a large mass in the pancreatic tail and multiple liver metastases, diagnosed as acinar cell carcinoma of the pancreas with a few scattered endocrine cells by liver biopsy is presented. The S-1 chemotherapy was effective, and partial response was obtained with decreased levels of serum CA19.9 and NSE. Ten months after starting chemotherapy, the tumor began to grow accompanied by marked elevation of serum NSE levels (266 ng/ml). The patient died of liver failure due to multiple liver metastasis 18 months after the initiation of the S-1 chemotherapy. Histological findings at autopsy were acinar cell carcinoma with an endocrine component of more than 30 %; the final diagnosis was mixed acinar-endocrine carcinoma of the pancreas. This pathological change and clinical course may imply that S-1 was effective against the acinar component but less effective against the neuroendocrine component caused by tumor differentiation.     

Spontaneous rupture of a hepatic angiomyolipoma: Report of a case

A 70-year-old female experienced sudden onset of back pain on the right side and was admitted to our hospital in December 2010. Abdominal computed tomography revealed an S7 hepatic mass measuring 7 cm in diameter accompanied by a subcapsular hematoma. Emergency angiography confirmed the diagnosis of a ruptured hepatic mass, and hemostasis was carried out by embolization of A8 and A7 of the liver. A right hepatic lobectomy was carried out 39 days following transarterial embolization. Although almost all aspects of the tumor were necrotic, residual tumor cells stained positive for HMB-45, and negative for α-SMA, S-100, CD 34, c-kit, CAM 5.2, and hepatocytes. The MIB-1 index was 2 %. Pathological diagnosis was hepatic angiomyolipoma (HAML). The patient has shown no signs of recurrence at 42 months following surgery. Here, we report on this case of spontaneous HAML rupture and discuss therapeutic strategies for HAML and ruptured hepatic tumors.     

Jejunal tubulovillous adenocarcinoma in adenoma presenting with entero-enteric intussusception

A 73-year-old male was admitted to our institution with complaints of nausea, vomiting, and abdominal distension. Plain abdominal computed tomography (CT) suggested intussusception in the jejunum. Enhanced abdominal CT revealed the ‘target-like’ sign and ultrasonography revealed the ‘multiple concentric ring’ sign; therefore, a diagnosis of entero-enteric intussusception was made. The small intestinal obstruction and cause of the intussusception were not evident. The patient was treated conservatively with fasting and transfusion therapy to prevent intestinal obstruction. However, with no spontaneous resolution of intussusception, surgical treatment was decided. The operative findings revealed a jejunal tumor about 30 cm from the Treiz ligament, and the jejunum including the tumor with a 5 cm margin were partially resected. The resected tumor was a 35 × 50 mm soft mass spreading laterally with nodules. The pathological examinations revealed tubulovillous adenocarcinoma in the adenoma. Intussusception is rare in adults compared to children. About 45 % of cases of intussusception in adults are due to small intestinal tumors such as malignant lymphoma or lipoma, but a tubulovillous adenocarcinoma with adenoma is a rare cause of intussusception. We present a rare case of jejunal tubulovillous adenocarcinoma in adenoma presenting with entero-enteric intussusception.     

Cholangiolocellular carcinoma with rapid progression initially showing abnormally elevated serum alfa-fetoprotein

Cholangiolocellular carcinoma (CoCC) is a rare malignant liver tumor derived from hepatic progenitor cells, which exist in the canals of Hering. We encountered a case of CoCC with an extremely poor clinical course, initially showing abnormally elevated serum alfa-fetoprotein (AFP). A 72-year-old male presented with a liver tumor and abnormally elevated serum AFP levels (16,399 ng/ml). We preoperatively diagnosed hepatocellular carcinoma and performed extended right hepatectomy, after which the serum AFP levels remarkably decreased to 97 ng/ml. Postoperatively, the disease was pathologically diagnosed as CoCC. Furthermore, immunohistochemical pathological findings were alcian blue negative, cytokeratin (CK) 7 partially positive, CK19 positive, hepatocyte paraffin-1 negative, membranous negative for epithelial membrane antigen, and AFP negative. Fifty-five days later, intra- and extrahepatic recurrence developed, and the patient died 65 days after surgery. Although CoCCs show favorable outcomes, these characteristics of our case were not previously reported. It is necessary to accumulate more information on CoCC.     

Multiple hepatic adenomas caused by long-term administration of androgenic steroids for aplastic anemia in association with familial adenomatous polyposis

We report a rare case of hepatic adenomas (HA), in a 20-year-old Japanese girl treated for 6 years with anabolic androgens for aplastic anemia. In a review of the world literature using computer MEDLINE search, we found only 17 cases of androgen-induced HA published between 1975 and 1998 in the English-language literature. The patient was referred to us because of liver lesions detected during a follow-up examination for familial adenomatous polyposis. After being diagnosed with aplastic anemia at 14 years of age, she had been treated with oxymetholone (30 mg/day) for 6 years. Laboratory evaluation revealed normal liver function. Ultrasonography (US) and computed tomography (CT) demonstrated multiple liver lesions. Histopathological examinations of biopsied specimens from the liver tumor showed HA. After the patient was diagnosed with HA, oxymetholone was tapered off. Patients taking androgenic-anabolic steroids should be carefully monitored with US and CT and tumor markers should be measured. This report may be helpful in identifying the population who is at risk of developing hepatic sex hormone-related tumors. Received: June 21, 1999 / Accepted: November 26, 1999     

Rapidly growing hepatocellular carcinoma recurrence during direct-acting antiviral treatment for chronic hepatitis C

We herein report the case of a woman in her 80s with a recurrent hepatocellular carcinoma (HCC) tumor that rapidly increased in size during direct-acting antiviral (DAA) treatment. She suffered from HCC at her initial visit to our department and underwent hepatectomy. Thereafter, she underwent DAA treatment for chronic hepatitis C; however, her alpha-fetoprotein (AFP) level rapidly increased, and a liver tumor of >?1 cm in diameter was observed that had not been seen immediately before DAA treatment. She underwent hepatectomy again and moderate to poorly differentiated HCC was diagnosed. The patient’s AFP level showed a rapid increase immediately after the start of DAA treatment; however, the increase ceased after the first month, and the influence from the surrounding environment of the tumor was considered to be temporary.     

Gastric adenocarcinoma associated with fundic gland polyps in a patient with attenuated familial adenomatous polyposis

Familial adenomatous polyposis (FAP) is a rare autosomal dominant precancerous condition of the colon caused by mutations in the adenomatous polyposis coli (APC) tumor suppressor gene. FAP is characterized by the appearance of innumerable adenomatous polyps throughout the large bowel. Fundic gland polyps are the most common gastric lesion in FAP. It is generally believed that fundic gland polyps have little or no potential for malignant transformation in the population at large, and only a few case reports describe the development of high grade dysplasia or gastric adenocarcinoma associated with diffuse fundic gland polyposis in patients with FAP. We report the second case of gastric adenocarcinoma intimately associated with fundic gland polyposis in a family with an attenuated form of FAP. The patient had undergone routine screening per current guidelines because of his known mutation in the APC gene. This suggests that malignant transformation of fundic gland polyps in patients with FAP occur more frequently than previously believed. Current screening recommendations may not be sufficient for patients with FAP or its attenuated forms.     

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.     

Primary adenocarcinoma arising in an interposed colon

We herein report a rare case of adenocarcinoma arising in an interposed colon. A 71-year-old male underwent total thoracic esophagectomy for thoracic esophageal cancer followed by antesternal reconstruction using the right colon. Seven months later, ¹⁸F-fluorodeoxy-glucose positron emission tomography revealed increased uptake in the interposed colon. Endoscopy detected a mass 25 mm in diameter in the cecum of the interposed colon approximately 10 cm from the distal anastomosis. An endoscopic biopsy showed well-differentiated adenocarcinoma. Partial resection of the interposed colon was performed, and the patient’s postoperative course was uneventful. The patient is currently alive with no signs of recurrence three years after the final surgery. We herein report a case of primary adenocarcinoma arising from an interposed colon after esophagectomy and review the literature.     

Conversion surgery after S-1 plus oxaliplatin combination chemotherapy for advanced gastric cancer with multiple liver metastases

Conversion therapy for gastric cancer is a new therapeutic concept. We report a case of a patient with advanced gastric cancer who underwent conversion surgery due to a remarkable regression of multiple liver metastases following chemotherapy. A 71-year-old man was referred to our hospital with gastric cancer. Esophagogastroduodenoscopy (EGD) revealed an irregular, nodular, ulcerated lesion in the lower third of the stomach. Analysis of biopsy specimens revealed a poorly differentiated adenocarcinoma. Abdominal contrast-enhanced computed tomography (CT) showed multiple liver mass lesions. The patient was clinically diagnosed with advanced gastric cancer with liver metastases and received S-1 plus oxaliplatin chemotherapy. After 6 cycles of chemotherapy, CT and magnetic resonance imaging showed complete resolution of the liver metastases, and EGD detected mucosal irregularities only. Since there was no evidence of further metastatic lesions in other organs, the patient underwent distal gastrectomy with D2 lymphadenectomy. The gross appearance of the surgically resected specimen showed a slightly elevated tumor measuring 4.5?×?3.5 cm. Pathological examination confirmed the diagnosis of a moderately differentiated gastric adenocarcinoma invading the muscularis propria with no lymph node metastases. The postoperative course was uneventful. The patient has continued to receive S-1 and oxaliplatin chemotherapy, and there has been no evidence of recurrence for 3 months following the operation. We propose that conversion therapy might be an effective treatment for patients with advanced gastric cancer; however, further studies and assessments are needed to confirm and establish this treatment strategy.