Non-functional pancreatic endocrine carcinoma with multiple liver metastases--a case report

A 60-year-old woman was admitted to our hospital because of upper abdominal discomfort and body weight loss. Abdominal CT showed multiple liver tumors with early enhancement and delayed washout. There were no abnormal findings in other organs. IVR-CT showed hypervascular masses, but it is not a typical tumor staining of HCC. To obtain the diagnosis, we performed laparoscopic partial liver resection in the left lateral segment. Histological examination suggested a primary hepatic carcinoid a tumor. But primary hepatic carcinoid tumor is comparatively rare, so we underwent further examinations. Two months later after the liver resection, the tumor of pancreatic tail was detected by CT and MRI. We could obtain the diagnosis of pancreatic endocrine tumor by EUS-FNA. We conducted a distal pancreatectomy with splenectomy and partial colonic resection. She had no symptom related to neuroendocrinology. The final diagnosis was non-functional endocrine carcinoma of pancreas. After that, we added extended right hepatic lobectomy with radiofrequency ablation in left lobe. The woman remains alive without a recurrence after the surgery.     

食管间质瘤与平滑肌肿瘤的临床鉴别及治疗

背景与目的：食管间质瘤与平滑肌肿瘤虽然具有不同的病理学特征,但是临床上不易鉴别。本研究比较食管间质瘤和平滑肌肿瘤的临床特征,并探讨其治疗原则。方法：用免疫组织化学法检测原诊断为食管平滑肌（肉）瘤20例和间质瘤1例肿瘤组织中CD117和CD34等一组抗体表达。结合病理学形态表现和临床表现进行分析,并总结各自的治疗原则及疗效。结果：16例原诊断为食管平滑肌瘤有5例CD117（＋）,按照间质瘤评定标准,均为非高度侵袭危险性食管间质瘤,余11例CD117（-）;1例原间质瘤CD117（＋）和CD34（＋）,为高度侵袭危险性食管间质瘤;4例食管平滑肌肉瘤CD117（-）和CD34（-）。食管非高度侵袭危险性间质瘤和平滑肌瘤的病理学形态表现、临床表现、治疗方法和预后没有明显差异;食管高度侵袭危险性间质瘤和平滑肌肉瘤的病理学形态表现、临床表现、治疗方法和预后也没有明显差异。结论：病理学形态表现和临床表现无法区分食管间质瘤和平滑肌肿瘤,CD117等抗体的免疫组织化学法检测是区分食管间质瘤和平滑肌肿瘤必不可少的手段。食管非高度侵袭危险性间质瘤和平滑肌瘤局部切除术或部分食管切除术可以达到根治,预后良好;高度侵袭危险性食管间质瘤和平滑肌肉瘤需行食管部分切除术。     

Primary leiomyosarcoma of the fallopian tube

Primary sarcoma of the fallopian tube is a very rare neoplasm. We report the case of a 69-year-old woman affected with leiomyosarcoma of the left fallopian tube. Her chief complaint was lower abdominal pain. The preoperative diagnosis was a left adnexal malignant tumor based on pelvic examination, abdominal computed tomography, and magnetic resonance imaging. Following a laparotomy, she was ultimately diagnosed with a FIGO IIc fallopian tube leiomyosarcoma. She was treated with total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic lymph node dissection, partial omentectomy, and low anterior resection for rectal invasion. The patient subsequently received adjuvant chemotherapy with pirarubicin and ifosfamide. Thirty months after the first therapy, a computed tomography scan revealed metastasis of the liver, lung, and supraclavicular lymph node. The patient died of the disease 39 months after the initial treatment.     

Histoculture drug response assay guided concurrent chemoradiotherapy for lung metastasis from adrenocortical carcinoma--a case report

A 50-year-old woman underwent surgical resection of a left adrenocortical carcinoma in April 2000. Bilateral pulmonary metastases and abdominal lymph node metastasis were detected in June 2001. After radiation therapy for the abdominal lymph node metastasis, a pulmonary metastatic lesion was thoracoscopically resected. The specimen was subjected to histoculture drug response assay (HDRA), and results revealed that this tumor was sensitive for cisplatin. We therefore performed concurrent chemoradiotherapy including cisplatin for the residual pulmonary metastatic lesion; a complete response was then obtained. Standard protocols of chemotherapy are often absent for malignant tumors, such as in this case, with low incidences. HDRA seems useful for chemotherapy agent selection in cases of rare malignant tumors.     

A case of mucinous adenocarcinoma of descending colon with abscess formation and solitary metastasis at right side of the abdomen

A 59-year-old man realized a left lower abdominal pain and palpable mass about a month ago. He was admitted to our hospital because of the pain and high fever. Blood examinations revealed high levels of white blood cell count and CRP. Ultrasonography showed a wall thickness of the descending colon and 7 cm cystic mass with air around. Moreover, a 6 cm cystic mass was found an outside of the ascending colon. We first treated with antibiotics under the diagnosis of abscesses because of perforation of diverticulum. Colonoscopy revealed an obstructing tumor in descending colon and biopsy specimens of the tumor showed adenocarcinoma. The preoperative diagnosis of the right side tumor was unclear. We performed a partial resection of the descending colon, and the tumor outside of the ascending colon was resected. The pathological diagnosis of the tumors was both mucinous carcinoma of the colon. Mucinous carcinoma tends to be more found in the right side of the colon than in the left side. The rate of lymph node metastasis and dissemination is higher than that in differentiated adenocarcinoma. We report a rare case of mucinous carcinoma originated from the left side of the colon with a solitary dissemination in opposite side of the abdomen.     

Transdifferentiation of pancreatic stromal tumor into leiomyosarcoma with metastases to liver and peritoneum: a case report

Background

Primary pancreatic leiomyosarcoma is a rare pancreatic malignancy; the clinical presentation and treatment is not well-characterized. Further, the molecular mechanisms underlying its pathogenesis are not known. We report a patient with pancreatic stromal tumor that progressed to primary pancreatic leiomyosarcoma with hepatic and peritoneal metastases.

Case presentation

A 54-year-old woman was found to have pancreatic and hepatic tumor masses on routine health checkup. Owing to the difficulty in performing biopsy, this patient underwent open operation. Histopathological examination of pancreatic and liver biopsy specimen demonstrated spindle cells with nuclear mitoses. Immunohistochemical examination showed positive staining for Cluster of Differentiation117 (+) and negative staining for S-100 (-) and Smooth Muscle Actin (-). Thus, the patient was diagnosed as a case of advanced pancreatic stromal tumor with liver metastases. After surgery, treatment with oral imatinib mesylate combined with thymosin injection therapy was prescribed. Follow-up examination at 13-months revealed multiple nodular masses in liver and right peritoneum. The patient underwent a second surgery. Liver biopsy and the resected peritoneal specimen showed positive staining for Discovered On Gastrointestinal tumor-1(weak +), Actin (+), Smooth Muscle Actin (+) and negative staining for Cluster of Differentiation117 (-) Cluster of Differentiation34 (-) and S-100 (-). Histopathological examination showed spindle cells with nuclear mitoses. The final diagnosis was primary pancreatic leiomyosarcoma, transdifferentiating from pancreatic stromal tumor, with liver and peritoneal metastases.

Conclusions

Surgery is the first line treatment for primary pancreatic leiomyosarcoma and extra-gastrointestinal stromal tumors. In the present case, radical resection was not performed owing to hepatic metastases. Palliative treatment with radioactive ¹²⁵I ion implantation and microwave coagulation therapy was administered. However, the long-term therapeutic effect needs to be assessed in future.

     

胃平滑肌肿瘤的诊断与治疗（附21例临床分析）

目的:研究旨在评价胃平滑肌肿瘤的生物学行为及其诊治。方法:收集1986～1995年间本科治疗患者,检出平滑肌起源胃肿瘤21例,复习病史、实验室检查、手术及病理报告,以比较良、恶性平滑肌肿瘤的临床特点、生物学活性及处置。结果:病理证实11例胃平滑肌瘤,9例肉瘤及1例子滑肌母细胞瘤。临床特点以腹痛(16/21)、呕血及血便(12/21),以及腹部包块(5/12),腹块主要见于恶性肿瘤中。肿瘤好发于胃体及胃底;肿瘤直径范围自0.5～15cm,肌瘤平均3.9cm,肉瘤平均7.8cm(P<0.05)。外科治疗的原则是局部切除肿瘤及周边2—3cm胃壁组织。在4例有转移的恶性肿瘤采用了姑息治疗措施。结论:区分平滑肌瘤及肉瘤主要标准是临床上局部浸润及转移以及组织学上的有丝分裂指数。肿瘤大于8cm恶性可能大,手术切除范围宜扩大。     

Primary leiomyosarcoma of the breast: a case report

Primary leiomyosarcoma of the breast is an extremely rare tumor, accounting for less than 1% of all breast tumors and only 24 cases have been reported in the English medical journals. It is quite difficult to diagnose leiomyosarcoma preoperatively. Establishing an accurate diagnosis is very important in planning treatment. When preoperative diagnosis can be achieved before or during the operation, wide resection should be performed. There is no need for axillary lymph node dissection. In this case report, we present a case involving primary leiomysarcoma of the breast in a 48-year-old woman and we discuss optimal treatment options.     

Dedifferentiated chondrosarcoma of the lung: case report and review of the literature

Primary malignant chondromatoid tumors of the lungs are rare. We report on a case of a 49-year-old woman who presented with a round focus in the upper lobe of the left lung. The performed biopsy showed features of a leiomyosarcoma. After chemotherapy and consecutive tumor resection, the histologic investigation of the entire tumor mass revealed a dedifferentiated chondrosarcoma. Careful clinical and radiologic examinations showed no evidence of further pulmonary and extra-pulmonary tumor lesions. The final diagnosis of a primary dedifferentiated chondrosarcoma of the lung was based on the morphologic criteria and review of the literature. The differential diagnosis of malignant hamartoma was discussed. Follow-up investigation after 2.5 years showed no evidence of another primary tumor site.     

Multiple Retroperitoneal Schwannomas Mimicking Adrenal Tumors

Schwannomas are benign tumors mostly arising from the head and neck. Retroperitoneal schwannomas are rare. Here we report the case of a 68-year-old woman with multiple retroperitoneal schwannomas. An abdominal computed tomography (CT) scan showed 1 non-enhancing mass with a low-density area, 4.6 cm in diameter, in the left adrenal region and another non-enhancing mass with a low-density area, 2.9 cm in diameter, in the para-aortic region. The patient had regularly undergone CT scans for malignant tumor screening in maintenance dialysis patients. Retrospectively, the 2 tumors had existed in the same regions in previous CT scans and had gradually increased in size. The tumors were completely resected. Pathological diagnosis of both tumors was schwannoma. When a CT scan shows a slowly growing retroperitoneal tumor with a low-density area, schwannoma should be considered. If the tumor increases in size, complete resection should be performed for pathological diagnosis.Key Words: Retroperitoneal schwannoma, Computed tomography scanning, Adrenal tumor     

A case of liver metastasis from gastric cancer responding to S-1/CDDP chemotherapy, allowing partial gastrectomy and left hepatectomy

A 63-year-old man was found to have an upper abdominal mass, and was referred to our hospital. Endoscopic examination and abdominal CT showed gastric cancer with liver metastasis. A large metastatic tumor of the liver invaded the hepatic hilus, making curative resection impossible. We started chemotherapy using S-1(120 mg/body/day), orally administered for three weeks followed by 2-week rest period, and cisplatin(100mg/body), administered intravenously on day 8 as 1 course. After 5 courses of chemotherapy, the liver tumor reduced markedly and the gastric cancer pathologically disappeared, enabling partial gastrectomy and left hepatectomy. Histological examination showed a well-differentiated adenocarcinoma in the mucosal layer of the resected stomach. A resected specimen of the liver showed a moderately-differentiated adenocarcinoma with signet-ring cells, compatible to liver metastasis from gastric cancer. Bile leakage the remaining liver occurred, but he recovered soon. Gastrointestinal examination revealed another early gastric cancer after seeing him for 2 years on an outpatient basis. We conducted subtotal gastrectomy, and the patient remains alive 30 months after the first operation. This case suggests that S-1/CDDP chemotherapy may reduce the stage of unresectable liver metastasis from gastric cancer and make a curative operation possible.     

Giant malignant mesenchymoma of the spermatic cord with bidirectional differentiation.

BACKGROUND: Spermatic cord neoplasms are a rare tumor entity and, moreover, of benign behavior. Malignant tumors of the spermatic cord are mostly of mesenchymal origin. We present the unusual case of a giant malignant mesenchymoma of the spermatic cord with bidirectional differentiation into a liposarcoma and a leiomyosarcoma. CASE REPORT: A 84-year-old male patient presented with a scrotal mass on the left side which was observed growing since 1 year and misdiagnosed as scrotal hernia or testicular hydrocele. Ultrasound and computed tomography demonstrated a solid tumor suggesting a spermatic cord tumor. The patient underwent hemiscrotectomy, and the histological examination of the 2,500-gram specimen revealed a malignant mesenchymoma originating from the spermatic cord with two distinct histopathological compartments of liposarcoma and leiomyosarcoma. Because an adjuvant therapy protocol is of questionable effect and because of the patient's age no further therapy was applied. The patient was closely followed and is now, 5 years after surgery, still free of disease. CONCLUSION: Even in older patients, scrotal masses should be considered malignant tumors as long as no benign diagnosis has been proven. Although malignant mesenchymomas are rare tumors with poor prognosis, in selected cases even large tumor masses, as presented, can be cured by surgery.     

A case of multiple brain metastases of uterine leiomyosarcoma with a literature review

Brain metastasis from uterine leiomyosarcoma is extremely rare, and prognostically alarming despite various treatments. The authors report a case of multiple brain metastases from uterine leiomyosarcoma who took a favorable course after tumor resection and γ-knife treatment. A 50-year-old woman with a history of hysterectomy for uterine leiomyosarcoma two years earlier, presented with a recent onset of headaches and vomiting. Multiple cerebral lesions were found by magnetic resonance imaging (MRI). The Karnofsky performance scale (KPS) was 40 with left hemiparesis and cerebellar ataxia. She was treated by resection of the left occipital and cerebellar tumors, followed by γ-knife irradiation of the residual tumors. KPS was 70 at her discharge from the hospital. MRI failed to show recurrence of the intracranial lesions 6 months after irradiation. She remained at home until she died from massive intra-abdominal bleeding. This is the first case with multiple brain metastases from uterine leiomyosarcoma, who survived with remarkable neurological improvement for 12 months. No comparable survival has been reported in the literature. It is evident that surgical resection and additional γ-knife irradiation contributed to early neurological recovery.     

A case report of primary adenocarcinoma of small intestine

This is an account of a case of primary adenocarcinoma of the small intestine with peritoneal dissemination successfully treated with chemotherapy. A 64-year-old woman was admitted with a complaint of severe abdominal distension. Abdominal computerized tomography revealed a bowel obstruction with tumor and the remarkable small bowel dilation of oral side of tumor. The tumor was found at surgery to be at the ileum 15 cm proximal from the ileocecal region. Peritoneal dissemination was recognized around the ileocecal region, so ileum partial resection was performed for the primary cancer lesion and dissemination region. Pathological diagnosis of the resected specimen was adenocarcinoma with lymph nodes metastasis. The peritoneal dissemination consisted of metastatic adenocarcinoma from small intestine. After an operation, internal use of S-1 was performed as adjuvant chemotherapy. But a recurrent lesion at the ovarium was detected 6 months after surgery. The patient was subsequently treated with resection of the ovarium. For lung metastasis, the combination chemotherapy with mFOLFOX6 + bevacizumab was administered. Primary small intestinal adenocarcinoma is a rare disease, and it is often diagnosed as advanced cancer because of few characteristic symptoms. So carcinoma of the small intestine usually has a poor prognosis.     

A case report of multiple advanced hepatocellular carcinomas treated by combination therapy with hepatectomy and particle therapy

We report a case of multiple advanced hepatocellular carcinomas (HCC) with bilobar distribution treated by combination therapy of hepatectomy and particle therapy (carbon ion therapy). A 73-year-old man who had been pointed out hepatic tumors on abdominal CT was referred to our hospital in February 2010. Advanced HCCs; 8 cm Vp3 and 6 cm in the left lobe, 4 cm in the posterior segment, and 1 .5 cm in the S8 area, were detected by abdominal dynamic CT and EOB-MRI etc. Curative resection was not applied due to insufficiency of liver function and unfavorable anatomical tumor locations. Accordingly we have developed a novel combination therapy of hepatectomy and particle therapy. First we performed left lobectomy in March 2010, followed by administration of particle therapy to the posterior segment for local control in May. Then, transcatheter arterial chemoembolization was administered 4 times for residual tumors. The patient survives for 15 months after the initial therapy with good local control.     

Pancreatic and gastric metastases of leiomyosarcoma arising in the left leg

Pancreatic or gastric metastases from other primary malignancies are rare, especially from leiomyosarcoma. We report a case of leiomyosarcoma in the left lower leg with metastases to the pancreas and stomach. A 61-year-old man had liver cirrhosis caused by hepatitis C virus infection and was followed up by his primary physician. Two years before presentation at our hospital, he had undergone surgical resection of leiomyosarcoma in the left lower leg and systemic chemotherapy for multiple metastatic tumors in the lung. On admission, endoscopic examination and computed tomography were performed for a routine checkup to exclude esophageal varices and liver tumor. Although the patient had no specific symptoms, multiple gastric and pancreatic metastases were identified by endoscopy and computed tomography, respectively. In general, metastases to the pancreas and stomach are rare. We discuss the clinical and diagnostic findings of pancreatic and gastric metastases by reviewing previously reported cases.     

Cystadenoma of the liver treated by hepatic resection

The aim of this study is to report a case of cystadenoma in the liver and to discuss the difficulty of differential diagnosis from malignant tumor and the importance of complete resection of the lesion. The patient had epigastric pain and abdominal distension. Hepatic ultrasonography, abdominal CT, magnetic resonance imaging, and angiography showed the presence of a large cystic tumor occupying the right hepatic lobe indicating malignant cystadenocarcinoma. Needle biopsy of the cyst wall with sampling of the cyst fluid was performed, which revealed no malignant cells. However, CEA and CA19-9 were markedly elevated in the cyst fluid. A right trisegmentectomy via laparotomy was performed successfully. The resected tumor was 11.5x9x9 cm in size and weighed 1,240 g. The cut surface revealed the tumor with multiple cysts, in some parts of which large papillary projections into lumina were seen. Though the gross features were similar to those of cystadenocarcinoma, the final histological diagnosis was benign cystadenoma of the liver. The postoperative course was uneventful, and the patient is in good condition six years postoperatively without evidence of recurrence of the disease.     

Tumeur fibreuse de la plèvre : difficultés diagnostiques et particularités radiologiques et anatomopathologiques

Solitary fibroma of the pleura is a rare tumor that accounts for 5–10% of pleural tumors whose diagnosis is often difficult. We report the case of a 47-year-old patient who consulted for left chest pain lasting for 1 year, exertional dyspnea, and dry cough. The review noted a syndrome of pleural effusion of the left basal chest. Chest radiography objectified an opacity of lower 2/3 of the left hemithorax. Thoracentesis was white. Chest CT showed a large tissue formation. Puncture and trans-parietal biopsy-guided scan were inconclusive. The histological examination with immunohistochemistry further after surgical resection of the tumor confirmed the diagnosis of pleural fibroma. The postoperative course was uneventful. Through this observation we emphasize the diagnostic difficulty and we will clarify the clinical, radiological, and pathological findings of this tumor.     

A case of curative resected hilar bile duct cancer with hepatic artery and portal vein reconstructions

The indication for surgical resection due to hilar bile duct cancer (BDC) with vessel reconstructions is still controversial. We report herein a successfully resected case due to hilar BDC with hepatic artery (HA) and portal vein (PV) reconstructions using autograft from a resected liver. A 57-year-old woman was diagnosed as hilar BDC, and computed tomography showed a tumor invaded left and common hepatic duct, right and left HA, and left main branch of PV. Because the extrahepatic area of right HA was free from the tumor, we performed left hepatectomy and caudate lobectomy with HA and PV reconstructions. We used auto left hepatic vein graft from the resected liver for PV reconstruction, because there was no appropriate size vein graft, e.g. inferior mesenteric vein. The patient is alive without any evidence of recurrence for 8 months after the surgery.     

The caudate lobe of the liver: implications of embryology and anatomy for surgery

The anatomy of the caudate lobe has technical and possibly oncologic implications for surgeons. The complex anatomy of the lobe is clarified by embryologic and anatomic analysis. This posterior sector is embryonically and anatomically independent of the right and left liver and the main portal fissure. The caudate lobe represents the only part of the liver that is in contact with the vena cava, except at the entrance of the main hepatic veins into the vena cava, and provides an anastomosis between the hepatic veins and vena cava. The entire caudate lobe is a single anatomic segment that is defined by the presence of portal venous and hepatic arterial branches, which supply the lobe, draining biliary ducts, and hepatic veins. Because no separate veins, arteries, or ducts can be defined for the right paracaval portion of the posterior liver and because pedicles cross the proposed division between the right and left caudate, the concept of segment IX is abandoned. The significance of caudate anatomy is reflected in the increase in the frequency and safety of major hepatic resection for primary and metastatic tumors in the liver. Right hepatic lobectomy routinely involves resection of the right portion of the caudate lobe (C. Couinaud, unpublished data, 1999). In the case of hilar bile duct cancer, which may extend into the dorsal ducts (especially the right lateral duct), partial or total caudate lobectomy is often necessary for complete extirpation of the tumor. Isolated caudate lobectomy can be performed for hepatocellular carcinoma that arises in the caudate lobe or for other tumors that arise in the lobe. The caudate lobe can be resected as part of the donor liver in preparation for a living related donor transplantation. Knowledge of the surgical anatomy of the caudate lobe is an essential part of the repertoire for surgeons who perform liver transplants or treat hepatobiliary cancer.