Successful surgical management of pulmonary and adrenal metastases from hepatocellular carcinoma.

This article reports a rare case of successful surgery for both lung and adrenal metastases after hepatic resection of hepatocellular carcinoma (HCC). A 55-year-old Japanese man with a 5-year history of chronic liver disease was admitted with an elevated serum alpha-fetoprotein (AFP) value and a liver tumour detected by ultrasonography. Hepatic angiogram showed a tumour stain with the right hepatic vein as a venous drain from the tumour. He underwent posterior-inferior subsegmentectomy of the right hepatic lobe following preoperative chemoembolization. Sixteen months after the first operation, he received pulmonary resection for a solitary metastasis in the right lung. A further 10 months later, a metastatic tumour was detected in the left adrenal gland without any recurrent or metastatic foci, and he underwent left adrenalectomy as his third operation. He is still alive, 8 months after his last operation, and 34 months after hepatic resection, with a normal value of AFP and without any recurrent or metastatic foci. This may be the first report of a patient who underwent successful surgery for pulmonary and adrenal metastases of HCC.     

Primary rectal carcinoma in patients with stage IV resectable disease at diagnosis

BACKGROUND: Rectal cancer is commonly diagnosed at a precocious stage, but for patients presenting at diagnosis with stage IV disease the best treatment is still undefined. The purpose of this study was to review the feasibility and outcome of multimodality treatment of rectal cancer patients metastatic at diagnosis. PATIENTS AND METHODS: From January 2000 to December 2005, 40 patients with histologically proven stage IV rectal adenocarcinoma (< 12 cm from the anal verge) were examined. Variables considered were age (under or over 65 years), tumour grade, presence of peritoneal carcinomatosis, type of surgery (palliative versus resection). RESULTS: The median age was 61 years (range, 32-83) and 27 were male and 13 female. Seventeen patients with unresectable or potentially resectable metastatic disease received neoadjuvant chemoradiotherapy (CHT-RT) with 5-fluorouracil (5FU) (plus oxaliplatin in 11 cases), radical surgery was performed in almost half of the cases; only in two patients were metastases also resected. If the patient is a candidate for radical surgical resection, the primary tumour should initially be treated as in a patient without metastatic disease and subsequently the primary tumour and metastases should be treated surgically. If the metastases are unresectable, the treatment of the primary lesion, according to the patient's symptoms, should be by palliative CHT. It is still not determined whether RT should be reserved for the symptomatic cases as an alternative to local surgery. In five patients treated with neoadjuvant CHT alone, radical local surgery was performed in two cases. Eighteen symptomatic patients were resected primarily; all of them received a postoperative CHT but only five of them also received postoperative RT. Nevertheless, after a multimodality treatment (neoadjuvant CHT +/- RT) 22.5% achieved a response rate (RR) (one complete remission (CR) and eight partial remission (PR)). Considering that all except two of the patients were locally radically resected and two of them also underwent metastases surgery, the overall RR was 17.5% (four CR and three PR). All of the CR were disease-free and alive after a median follow-up of 19.3 months. Age > or = 65 years had no impact on overall survival (OS), but the presence of peritoneal carcinosis in five patients showed a trend towards diminished survival, although it was not statistically significant (p = 0.08). CONCLUSION: The best treatment on diagnosis of metastatic rectal cancer is a multimodality CHT-RT approach. New prospective studies should evaluate non cross-resistant regimens as additional therapy for those patients with a systemic residual disease after common CHT-RT.     

Long-term survival in bronchogenic carcinoma with a solitary metastasis

Partial resection of a huge anaplastic large cell carcinoma of the upper lobe of the right lung was performed in a 47-year-old patient in order to relieve symptoms of pulmonary hypertrophic osteoarthropathy. Several months later a solitary metastasis was noted in the muscles of the right forearm. The metastasis was resected and the forearm irradiated. The patient was further treated with injections of autologous tumour cell vaccine and BCG. Today, 7 years later, the patient is alive, without any signs of neoplastic disease.     

Primary and metastatic pulmonary meningioma

Patient 1 was a 53-year-old man who had a very rare primary pulmonary meningioma that developed in the left lingular segment. When this report was written, 7 years had passed since he underwent operation, and no recurrence of the meningioma had been detected. In Patient 2, a 61-year-old woman, multiple pulmonary metastases were confirmed 19 years after she had undergone operation for multiple cerebellar meningiomas, and the metastases were resected. After 2 years, multiple intraperitoneal metastases were found, and thus aggressive surgery was performed. Currently, 22 years after the operation for the primary cerebellar meningioma, the patient is alive without any subjective symptoms, although intraperitoneal metastases have recurred. To date, only four cases (all in women) of primary pulmonary meningioma have been reported. Case 1 reported in this article is thus the first case in a male patient to be reported, and, in addition, this patient also has the first reported case to have been evaluated for more than 5 years. In Case 2, however, each of the excised extracranial tumor lesions was histologically homogeneous and showed a hemangiopericytomatous pattern. The histologic picture of those tumor lesions was exactly the same as the picture of a small portion of the cerebellar meningiomas excised 19 years earlier. Thus, all those extracranial tumor lesions were diagnosed to be metastatic meningiomas. However, it is difficult to explain why there had been no symptoms for as long as 19 years until the pulmonary metastases were discovered.     

Metastatic malignant acrospiroma of the hand.

We present the case of a 37-year-old man with multiple pulmonary metastases of a primarily unknown primary tumour. Thorough revision of the medical history yielded that he had already passed three resections of a right palmar mass, which had been described as a benign tumour. Clinical examination showed a thickened scar with a suspicious palpable mass in the right hand. Excision of this scar and the tumour mass with histopathological examination now revealed a malignant acrospiroma. Resection of the pulmonary metastases histologically also confirmed a malignant acrospiroma. The following radical resection of the metacarpals II and III with the index and middle finger under the assumption of a wide compartment resection achieved tumour free margins and proved to be efficient with the patient being relapse free for 4 years from this operation. Although the prognosis of this tumour is generally unfavourable this particular case demonstrates the value of a radical surgical resection as the mainstay of treating such highly malignant sweat gland tumours of the hand. Copyright Harcourt Publishers Limited.     

Metastatic Choroid Lexus Papilloma: A Case Report

Choroid plexus papillomas (CPPs) are generally regarded as benign tumours, with a favourable long-term prognosis. Complete resection should result in cure. We present a case of diffuse craniospinal seeding from an apparently completely resected fourth ventricular primary tumour. A 51-year-old male is discussed, who presented 5 years following complete resection of a CPP from the fourth ventricle, with a progressive history of left sided tinnitus, hearing loss, impotence and recent low back pain. Imaging demonstrated multiple craniospinal lesions explaining his symptomatology. Differential diagnosis lay between long standing CSF seeding, malignant transformation in the primary tumour, or metastatic spread from an undefined source. He underwent whole body FDG-PET scan which demonstrated a single metabolically active lesion in the sacral canal. A subtotal excision biopsy of this sacral lesion was performed which was indistinguishable histologically from the primary tumour resected from the fourth ventricle.Histological and functional imaging characteristics of the primary tumour have been unhelpful in predicting its subsequent behaviour. The present case illustrates the extremely rare consequences of metastases from this histologically benign tumour and adds to the literature on metastatic craniospinal disease.This revised version was published online in April 2005 with corrections to figure 3-C.     

Chest wall sarcoma: outcome in 22 patients after resection requiring thoracic cage reconstruction

Purpose. To evaluate the outcome after resection of malignant chest wall sarcoma, requiring reconstruction of the chest wall.Subjects. Twenty-two patients, 15 with primary tumours, were operated on in our institution between 1983 and 1996. Four patients underwent surgery after a previous intralesional or marginal excision and three patients because of a local recurrence.Methods. The tumour was resected 'en bloc', including skin, muscle and thoracic skeleton. When necessary, adjacent organs invaded by the tumour, such as lung, pericardium and diaphragm, were also removed to obtain a wide margin. Reconstruction of the chest wall was performed with Marlex mesh (n=9), methylmethacrylate cement (n=2) or a Marlex methylmethacrylate 'sandwich' (n=11).Results. The median tumour size was 9.5 (2-20) cm. The most common type of tumour was chondrosarcoma (12 cases). No patient died in hospital. Five patients required reoperation because of complications, two patients because of loosening of the acrylate prosthesis, two because of necrosis of soft tissue coverage and one was reoperated because of bleeding. Four patients died of generalized tumour disease between 5 and 77 months after surgery and one patient died of a local recurrence 32 months after the primary operation. Seventeen patients are alive, with a median follow-up of 36 (4-162) months. Microscopic radicality (negative margin) was achieved in 17 patients but 5 of these had local recurrences. Two of five patients with positive margins had a local recurrence of the tumour. Of the seven patients with local recurrences, two also developed metastases.Discussion. Large chest wall sarcomas can be successfully resected and the chest wall reconstructed with low morbidity and mortality.     

Pulmonary metastases from adrenal cortical carcinoma: Results of resection

Adrenal cortical carcinoma is uncommon. There is little controversy concerning treatment of the primary tumor. However, data concerning the treatment of pulmonary metastases are sparse. In order to assess the results of therapy, we reviewed our 14-year experience. Records of 24 patients admitted to our institution from 1973 to 1991 with the diagnosis of adrenal cortical carcinoma and pulmonary metastases were reviewed. Ten patients underwent pulmonary resection, 12 received chemotherapy, and 2 no therapy. In the pulmonary resection group, treatment consisted of wedge resection in 8 patients and lobectomy in 2. For the 10 patients undergoing resection of their pulmonary metastases, the 5-year survival was 71% (median not reached at 5 years). This was significantly longer than those not resected, with no one alive at 3 years (median survival 11 months). Our data suggest that those patients who are able to have their pulmonary metastases resected survive significantly longer than those who do not. © 1993 Wiley-Liss, Inc.     

Successful treatment with cetuximab combination chemotherapy in a case of FOLFOX-refractory rectal cancer with previously unresectable multiple liver metastases leading to complete resection

Most colorectal cancer patients with liver metastases are not resectable upon initial diagnosis. Recently, chemotherapy improves overall survival of initially unresectable patients by allowing tumor downstaging and complete resection. We report a FOLFOX-refractory rectal cancer patient with unresectable multiple liver metastases, whose tumors could be downstaged and completely resected after initiation of FOLFIRI with cetuximab. Case: A 41-year-old male demonstrated rectal cancer with unresectable multiple liver metastases. He was treated by FOLFOX4 therapy as first-line chemotherapy. After initiating 14 courses, he was treated by FOLFIRI with cetuximab because of disease progression. After initiation of chemotherapy, radiographic examination demonstrated remarkable reduction of primary rectal tumor and metastatic liver tumors. He underwent complete rectal tumor resection after 13 courses of chemotherapy, and metastatic liver tumor resection after 18 courses of chemotherapy.     

Liver resection for metastatic non-colorectal non-neuroendocrine hepatic neoplasms.

AIMS: Major liver surgery can be performed safely and hepatic resection for metastatic disease is increasingly carried out. However, the role of liver resection for hepatic metastases from non-colorectal, non-neuroendocrine (NCNN) cancers is unknown. Our aim was to evaluate our experience from hepatectomies for NCNN metastases. A retrospective study of 170 patients with liver resection performed the last 8 years was performed in two liver units in affiliated university hospitals. METHODS: Eighteen patients underwent liver resection for NCNN tumours. Origins included kidney (n=6), breast (n=4), gastric tumours (n=4), intestinal leiomyosarcoma (n=2) and malignant melanoma and in one patient a metastatic papillary of unknown origin was found. Eleven patients underwent a hepatic lobectomy and seven had local resections. Ten hepatectomies were performed at the same time with the primary tumour resection (synchronous resections) with five of those in an en bloc fashion with the primary tumour. RESULTS: There were no post-operative deaths and the peri-operative morbidity was minimal. During a median follow-up time of 3.2 years, 14 patients are alive with one of them having developed pulmonary metastases. CONCLUSION: In carefully selected patients with NCNN liver metastasis, liver resection can prolong survival as well and improve quality of life. Copyright Harcourt Publishers Limited.     

Spontaneous regression of intestinal malignant melanoma from an occult primary site

A 55-year-old male patient who had melena and intussusception that proved to be due to malignant melanoma of the small intestine is described. History and close examination failed to show any evidence of a primary lesion. The diagnosis was made after a biopsy was performed on two lesions in the neck. This was followed by a palliative and incomplete resection of an involved ileal segment. The patient did not receive chemotherapy, radiotherapy, or immunotherapy. He is now alive 8 years after diagnosis without evidence of malignancy. This case represents spontaneous regression of malignant melanoma of the small intestine that is considered either a primary intestinal tumor or a metastatic tumor from an occult regressed primary. The latter assumption makes this case unique in that spontaneous regression occurred twice, once in the occult primary lesion and once in the intestinal metastases.     

Liver transplantation for symptomatic liver metastases of neuroendocrine tumours

Numerous reports have demonstrated that liver transplantation for neuroendocrine tumour metastasis is feasible. However, perioperative risks and long-term recurrences remain significant concerns. When liver transplantation is combined with extensive intestinal or pancreatic resection, the risk is particularly high.We report our institutional experience of liver transplantations performed for liver metastases secondary to neuroendocrine tumours, and in combination with a review of the literature, we propose a set of selection criteria. The key points include unresectable hepatic metastases of neuroendocrine origin, absence of extrahepatic metastases, symptomatic disease that is refractory to medical therapy, a Ki-67 level less than 2%, previous resection of the primary disease, and previous therapy for metastatic neuroendocrine tumour.In our experience, the patient in the first case had, post-transplantation, rapid disease progression because of an unidentified primary, and patient in the second case had primary non-function of the liver graft, requiring urgent re-transplantation. More recently, two liver transplantations were successfully performed. The indications were, in the first case, refractory hormonal secretion and, in the other, secondary biliary cirrhosis attributable to hepatic artery therapy with tumour in situ. Subclinical and stable recurrent disease has been detected by scintigraphy in the mesentery and lumbar spine in the former patient. A mesenteric recurrence developed in the latter patient 2 years post transplantation and was subsequently completely resected. At 4 and 5 years post transplantation, both patients are symptom-free.Recurrence after transplantation remains a significant concern, even with careful patient selection, but recurrences may remain indolent. If recurrences are progressive, they may still be amenable to additional medical or surgical therapy. A national or international consensus between oncologists and transplant specialists regarding the indications for liver transplantation is vital, because future progress will depend on careful patient selection and prospective study.     

An operated case of metastasis to the small intestine from lung cancer

We report a rare case of a 68-year-old man with long-term survival after a surgical treatment of solitary metachronous small intestinal metastasis from lung cancer. He underwent a right upper lobectomy for primary lung adenocarcinoma. Thirty -four months after the operation, a tumor of small bowel was detected by computed tomography. The tumor was resected and diagnosed as a metastasis from lung cancer. Eighteen months after resection of metastasis, a tumor located at mesentery of the jejunum was pointed out. The tumor was resected and also diagnosed as a metastasis from lung cancer. The postoperative course was uneventful, and the patient is still alive without recurrence for 3 years after the last operation. We reviewed of 222 Japanese cases that underwent a resection of small intestinal metastasis from lung cancer. Although the prognosis was extremely poor for those who underwent a resection of the primary lung cancer and who had no remnant metastatic lesion at the time of metastasectomy, they seem to have a longer survival time.     

Extraskeletal Osteosarcoma: A Clinicopathologic Study of Four Cases

Four cases of extraskeletal osteosarcoma which were treatedat the National Cancer Center Hospital have been reviewed. Therewere three males and one female. The ages of the patients were32, 48, 48 and 59 years. Three patients had a solitary tumorlocated in the lower leg, thigh or postirradiated abdominalwall. The remaining patient had multiple tumors in the ipsilateralthigh and buttock. The predominant histologic subtype was osteoblasticin two cases, chondroblastic in one and malignant fibrous histiocytoma-likein one. Only one patient with a subcutaneous tumor was freeof disease 16 years after a wide local excision without chemotherapy.The other three patients, two having undergone inadequate initialsurgery, and one with multiple tumors, developed pulmonary metastaseswithin 24 months of their initial surgical treatment. All ofthem died of the pulmonary metastases eight, 36 and 63 monthsafter their initial treatment. Postoperative intensive chemotherapywas given to two patients. Although both had developed metastasesto the lung, they received resection of the metastases combinedwith additional chemotherapy, surviving for more than 24 monthsafter the occurrence of their first metastases     

A Case of Solid Pseudopapillary Tumour of the Pancreas and Malignant Mesothelioma

INTRODUCTION: A 59-year-old man with previous exposure to asbestos presented with dyspnoea and pleuritic chest pain, had a pleural effusion and was treated for pneumonia. His symptom recurred and was found to have an abdominal mass. DISCUSSION: An abdominal computerised tomogram revealed pancreatic body mass arising. Pleural fluid cytology and a pleural biopsy failed to demonstrate malignancy. The pancreatic tumour was resected by distal pancreatectomy, segmental colectomy and splenectomy. The tumour was a solid pseudopapillary pancreatic tumour (SPT) with a high metastatic potential. The patient deteriorated and a repeat biopsy of the thickened pleura confirmed malignancy which was initially thought to be metastases from the SPT. Immunohistochemical staining confirmed malignant mesothelioma. The patient developed liver metastases and died 2 years from the diagnosis of metastatic disease.     

Vascular density in colorectal liver metastases increases after removal of the primary tumor in human cancer patients

In animal models, explosive growth of metastases after removal of the primary tumor has been attributed to abolishment of angiogenesis inhibition. We investigated the influence of (removal of) the primary tumor on vascularization of liver metastases in human colorectal cancer patients. We analyzed vascular density in synchronous liver metastases from patients with the primary tumor in situ, in synchronous metastases from patients with the primary tumor resected and in metachronous metastases. In a limited number of cases, biopsies from metastases from the same patient before and within 3 months after resection were analyzed. In addition, vascular density in metastases was compared to the vascular density in the corresponding primary tumor. Peritumoral and intratumoral vascular density were determined by staining for endothelial antigens CD31 and CD34, respectively. Both peritumoral and intratumoral vascular density were elevated in synchronous metastases from patients with the primary tumor removed compared to synchronous metastases from patients with the primary tumor in situ. Comparable results were observed in patients with metachronous metastases. An increase in vascular density after resection of the colorectal malignancy was also observed in biopsies taken from the same patient before and after tumor resection. Remarkably, vascular density in the liver metastases was always lower than that in the corresponding primary tumor. Our data show for the first time in humans that the presence of a primary tumor is correlated with decreased vascularization of its distant metastases. Resection of the primary tumor results in an increased vascularization of metastatic lesions.     

Prolonged remission in a patient with transitional cell carcinoma of the bladder developing brain metastases after systemic chemotherapy: a case report

We report a case of an excellent response to M-VAC chemotherapy in a patient with pulmonary metastases from transitional cell carcinoma of the bladder. He subsequently presented with acute neurological symptoms seven weeks after the completion of chemotherapy. Computed tomography of the brain revealed a solitary 24 mm x 26 mm lesion in the frontal lobe. The brain metastasis was resected. The patient was closely followed for disease progression and showed no evidence of disease up to 42 months after surgery. The presentation and treatment policy are discussed. This case suggests that long-term remission may be obtained after surgical resection of a single brain metastasis in patients with disseminated urothelial cancer who completely responded to systemic chemotherapy.     

Cystic pulmonary metastatic sarcoma

Neoplastic cavitary lesions are an unusual type of pulmonary metastases. The authors report two cases of cystic metastatic sarcoma of the lungs that illustrate the clinical, radiologic, and pathologic difficulties encountered in the diagnosis of these lesions. In one patient, multiple small, thin-walled cystic metastases from a lower leg leiomyosarcoma were the only manifestation of metastatic disease. The cystic lesions did not change over an 8-month period and a diagnosis of malignancy was not established until spontaneous pneumothorax, presumably due to rupture of the malignant blebs, prompted a thoracotomy. In the second patient, three thin-walled bullae developed after treatment of noncystic pulmonary metastases from a lower-leg synovial sarcoma. In both patients, the cystic lesions were not evident on chest radiographs, but were well visualized with computed tomography (CT), where they mimicked benign bullous disease. However, additional small cavitary lesions not seen with CT were present in resected pulmonary wedge specimens from both patients. A great degree of variability in the cellular composition of the cyst wall lining in both cases, and a lack of any solid neoplastic tissue masses in one case, led to histopathologic difficulties that required immunohistochemical studies for definitive diagnosis of the metastatic disease. These cases show that pulmonary bullae, even though thin-walled and benign-appearing on CT, may be a manifestation of pulmonary metastases. These lesions must therefore be surgically removed from patients in whom a curative resection of pulmonary metastases is warranted.     

Peritoneal carcinomatosis from solid pseudopapillary neoplasm (Frantz's tumour) of the pancreas treated with HIPEC

Solid pseudopapillary neoplasm (SPN) is a rare malignant tumour accounting for 0.1% to 2.7% of all pancreatic neoplasms and affecting young women. Peritoneal carcinomatosis (PC) is even rarer, with only 11 reported cases. We describe a twelfth case occurring 13 years after the resection of an SPN which ruptured peroperatively. This 35-year-old woman had first undergone complete cytoreductive surgery (CCRS) alone and disease had relapsed within 8 months. Ultimately, further CCRS was combined with hyperthermic intraperitoneal chemotherapy (HIPEC) with oxaliplatin and irinotecan. The patient is now alive and disease free 31 months after her last operation. In the literature, the surgical treatment of PC from an SPN has yielded disappointing results, with a 58% recurrence rate at intervals ranging from 1 to 19 years. As none of these patients developed distant metastases, indicating a strictly peritoneal disease, HIPEC might be a solution for preventing such recurrences.