Surgical implications of colorectal cancer genetics

The applications of genetics in colorectal cancer are not limited to identifying germ-line mutations in genetically predisposed individuals. Reports on the use of molecular genetic markers as prognostic factors and for assessing the response to therapy are common in the literature. As the primary caregiver in the majority of these cases, the surgeon needs a basic knowledge of colorectal genetics and their implications to the patient and the patient's family. This article discusses a practical approach for some of the issues likely to be encountered by the surgeon.     

Surgical implications of population screening for mammary carcinoma

With the introduction of population screening for mammary carcinoma, in the initial phase an increase of malignancies has to be expected. Later on in the program the type of lesions will change. In the case of nonpalpable lesions close cooperation between surgeon, radiologist and pathologist is mandatory. The treatment of patients with ductal carcinoma in situ needs special attention because of the complexity of the problem, and requires careful preparation of the disciplines involved.     

Surgical implications of hepatic venocclusive disease following bone marrow transplantation

Hepatic venocclusive disease occurs with a spectrum of severity in an estimated 21% of bone marrow transplant patients. Clinical features include severe right upper quadrant pain, ascites, weight gain and initially minimal derangement of liver function. In contrast to hepatic graft versus host disease, venocclusive disease usually occurs within the first three weeks of engraftment and in autologous grafts. Urgent surgical consultation is requested when these features are prominent enough to mimic common acute processes requiring laparotomy. This condition must be included in the differential diagnosis in order to avoid an unnecessary laparotomy in this select group of patients who are usually severely thrombocytopenic and leukopenic. Clinical diagnosis alone is very reliable.     

Surgical implications of sentinel node with micrometastatic disease in invasive breast cancer.

AIM: To assess the rate of positive axillary clearance (AC) when the sentinel node biopsy (SNB) contains micrometastatic disease in invasive breast cancer and to evaluate the factors that could predict positivity. PATIENTS AND METHODS: This is a prospective study carried out on 542 successive women undergoing SNB for unifocal T0-T1 N0 invasive breast cancer without previous treatment. RESULTS: Five hundred and twenty-five sentinel nodes (SN) were found, 142 contained metastases. Fifty-five of the positive SN contained micrometastatic disease only. Of them, 40 patients underwent completion of AC. Six out of 40 patients who had micrometastatic SN had a positive AC, five for micrometastasis between 0.2 and 2 mm (5/34), one for isolated cells in the SN (1/6). None of the studied factors (age, histological tumour size, histological grade, estradiol receptor (ER), histological tumour type, size and method of micrometastasis detection) could significantly predict the status of the AC. CONCLUSION: As long as the results of ongoing prospective randomised studies are unknown, it remains necessary to perform AC when the SNB contains micrometastatic disease, whatever the size or the detection mode of the metastasis.     

Pleomorphic liposarcoma originating from intracranial dura mater

Liposarcomas are the most common soft tissue sarcoma found in adults; however, disease involving or spreading to the head and neck is extremely rare. To our knowledge, we present the first case of primary pleomorphic liposarcoma originating intracranially. A 56-year-old man presented with new weakness and imaging findings confirming a right frontal mass. After resection, histological analysis confirmed the diagnosis of pleomorphic liposarcoma. The patient underwent radiation treatment and surveillance imaging, which revealed no other areas of disease. Results reported previously in the literature indicate that pleomorphic liposarcoma is very aggressive in nature. The authors review the few cases of primary or metastatic pleomorphic liposarcoma that have been reported involving the head.     

Surgical implications for liver metastases in nephroblastoma--data from the SIOP/GPOH study

BACKGROUND: In children with Wilms' tumor, the 5-year overall survival rate is over 90% in the SIOP/GPOH study group. However, a small group of patients have tumor lesions in the liver at the time of initial diagnosis or as a recurrence. This group seems to have a worse prognosis in terms of survival. The treatment and outcome of patients with a hepatic recurrence were analyzed compared to previously published data of patients with primary hepatic metastases. PATIENTS AND METHODS: We reviewed the records of 45 out of 1365 patients enrolled in the SIOP 93-01/GPOH study and the SIOP 2001/GPOH study between April 1, 1994 and September 30, 2004. Median age at diagnosis was 6.49 years (1.37-34.16 years) in 29 patients who were initially presented with hepatic metastases (group I) with 9 males and 20 females. In 16 children who had a recurrence of a nephroblastoma in the liver (group II), median age at diagnosis was 4.62 years (1.84-31.08 years) with 9 males and 7 females. RESULTS: In group I out of 29 patients, 11 died at a median of 13.07 months. Overall survival in group I was 62.58%. In group II, 9 patients died at a median 52 months. Overall survival in group II was 54.7%. CONCLUSION: This report suggests that when complicated by metastases of the liver, Wilms' tumor has a less favorable outcome. Chemotherapy and radiotherapy play a definitive role in the treatment of these children. The importance of complete resection of hepatic lesions in both groups should be emphasized.     

Skull-base invasion of nasopharyngeal carcinoma: magnetic resonance imaging findings and therapeutic implications

PURPOSE: To evaluate the value of skull-base abnormality on MRI for predicting local recurrence in nasopharyngeal carcinoma. MATERIALS AND METHODS: Between November 1988 and February 1997, 48 patients with NPC were examined with both MRI (1.5 T) and CT prior to radiation therapy. T classification (1987 UICC) based on physical examination and CT findings were T1 in 3 cases, T2 in 22, T3 in 9, and T4 in 14. On MRI, low-intensity tissue with Gd enhancement in the marrow of the skull was considered to be a suspicious finding of skull-base invasion. CT simulation was performed in all patients. The total dose to the primary tumor was 60-75 Gy (mean, 67 Gy). The mean follow-up period was 42 months. RESULTS: All 14 T4 patients had abnormal tissue in the marrow of the skull base on MRI. Thirty-eight percent (13 of 34) of T1-3 patients were suspected to have skull-base invasion based on MRI (0% for T1, 27% [6 of 22] for T2, and 78% [7 of 9] for T3). The 5-year local control rate was significantly different between T1-3 and T4 tumors (97% vs. 69%, p < 0.025) but was not different by the presence of the MRI abnormality in the skull base. CONCLUSION: Skull-base invasion suspected solely by MRI does not relate to local recurrence provided that careful treatment planning is performed with the aid of MRI and CT simulator.     

Homologous dura for myringoplasty —An analysis of 120 cases

Homologous dura is an excellent graft material for repairing tympanic membrane perforations. The graft preservation technique is simple and the graft take rate is comparable to grafting with temporalis facia and tragal perichondrium. We had done myringoplasty with homologous dura in 120 patients in the last 8 months. We had a graft take rate 85.8% with improvement of hearing in 84%. The preservation technique and surgical procedures are discussed and the relevant literatures are reviewed.     

Spontaneous penetration of dura mater and bone by glioblastoma multiforme

Three patients are reported whose cranial dura mater and bones were penetrated by intracranial glioblastomas in the absence of previous craniotomy or radiotherapy. The gliomatous nature of the tumors was confirmed by localization of cytoplasmic glial fibrillary acidic protein (GFAP) of the neoplastic cells. Review of the literature disclosed only 15 glioblastomas, including the three cases, spontaneously penetrating the cranial dura mater and bones. These patients ranged in age from 3.5 to 70 years with an average age of 40 years. The male/female ratio was 5/8. Five glioblastomas were in the temporal lobes, three were in the frontal lobes, three were in the frontotemporal regions, two were in the occipital lobes, one was in the frontoparietal region, and one was in the temporoparietooccipital region. Six glioblastomas also had spontaneous distant metastases. In the absence of previous craniotomy and radiotherapy, rapid growth of the glioblastomas may promote such spontaneous penetration into the cranial dura mater and bones.     

Primary low grade B-cell lymphoma of the dura in an immunocompetent patient

Primary central nervous system lymphomas (PCNSL) represent only 1% of all non-Hodgkin's lymphomas (NHLs). A 66 year-old woman was hospitalized due to multiple episodes of syncope and seizures which occurred the week before admission to the hospital. A computerized tomography (CT) scan of the brain showed a right parietal lesion suggesting a subdural effusion. The patient was operated and a dural lesion extending to the epidural space and cerebral cortex was excised. Histologic findings suggested diagnosis of a low-grade lymphoma of the mucosa associated lymphoid tissue (MALT) type. The patient was treated with radiation therapy and has now completely recovered 12 months after surgery.     

Primary osteogenic sarcoma arising from the dura mater: case report

A 42-year-old woman who sought treatment for left drop foot was found to have a right frontoparietal parasagittal mass. Gross total resection of the tumor was performed and pathologic analysis revealed high grade osteoblastic osteosarcoma. The patient received adjuvant chemotherapy and continues to do well with no evidence of metastases or local recurrence 3 years after initial presentation.     

Superficial cerebral astrocytoma attached to dura. Report of six cases in infants

Reported are six cases of meningocerebral astrocytomas attached to dura, involving the superficial cortex, in infants under 1 year of age. They represent 1.25% of 483 intracranial tumors in infancy studied at the Children's Hospital in the last 12 years. Five were located in the frontal lobes, with variable extension to the parietal or the parietotemporal regions, and one was located in the parietal lobe. They were all composed of a solid portion and one or more cysts, and they measured approximately 6 to 12 cm in diameter. They had spindle cells, some more plump, arranged in areas in a storiform pattern. Tumors were very rich in reticulin fibers, mimicking a mesenchymal tumor. There was very slight pleomorphism. Bizarre giant cells were not observed and lipidization was not a prominent feature. Immunoperoxidase technique was performed in all of them and showed glial fibrillary acidic protein (GFAP) in most of the tumor cells. We believe their origin is from subpial astrocytes. They probably represent a separate entity whose diagnosis cannot be accurately established without immunohistochemical techniques.     

Chronic subdural hematoma secondary to metastasis of adenocarcinoma of the dura mater and skull--a case report

A 72 year-old woman was hospitalized with the complaint of headache and nausea. Under the diagnosis of right chronic subdural hematoma, a small craniotomy was performed for the total removal of the hematoma. The patient died 14 days after the operation because of the complication of acute DIC. Histologically, metastasis of adenocarcinoma was detected in the dura mater and skull. Previously reported cases of subdural hematoma secondary to cancer were reviewed in the literature. It is considered that a coagulation defect such as DIC may play a significant role in the development of subdural hematoma. It is suggested that the chronic subdural hematoma in the present case was caused by chronic DIC due to metastasis of bone marrow, and that the patient deteriorated as a result of acute DIC triggered by the surgical therapy.     

Determination of the inferior border of the thecal sac using magnetic resonance imaging: implications on radiation therapy treatment planning

Purpose: To determine whether the traditional teaching of placing the caudal border of the spinal field at the S2–S3 interspace in children receiving craniospinal irradiation (CSI) is appropriate.Methods and Materials: Twenty-three children had magnetic resonance imaging (MRI) of the spine with gadolinium prior to craniospinal irradiation at one institution. Thecal sac termination using MRI was determined by drawing a perpendicular line from the point of convergence of dural margins to the corresponding vertebral body.Results: Location of thecal sac termination varied from mid-S1 to low S3 vertebral body, with the most frequent site at the upper S2 vertebral level. Only 2 of 23 (8.7%) children had thecal sac terminations below the S2–S3 interspace. For the nine patients with neuraxis disease, none had thecal sac terminations below the S2–S3 interspace. In seven of the nine patients who had neuraxis seeding at initial presentation, MRI of the spine after CSI was performed and showed that thecal sac termination was lower after radiation therapy in two children, higher in one, and the same in four.Conclusions: In 2 of 23 children (8.7%), placement of the inferior border at the bottom of the S2 vertebral body would have missed the entire thecal sac. Treatment to the entire neuraxis with adequate coverage of distal spinal theca can be achieved by using MRI. Individualized spinal fields using the MRI may help minimize radiation scatter to the gonads while adequately covering the target volume.     

Fascin expression in skull base chordoma: correlation with tumor recurrence and dura erosion

Skull base chordomas are invasive tumors, with high rate of local recurrence even when totally extracted. The aggressive biological behavior in chordoma remains unclear. The purpose of this study was to investigate the relationship between fascin expression and tumor biological behavior in skull base chordoma. Using immunohistochemical techniques, we investigated the expression of fascin in 34 patients with skull base chordomas (19 primary tumors and 20 recurrent tumors). Correlation between fascin expression and clinicopathological factors such as patient's age, sex, tumor locations, and fascin expression in recurrent tumor and in tumor with dura mater erosion was analyzed. Various extent of fascin expression was observed in all tumors. There was a higher fascin expression in recurrent chordoma than in primary chordoma, and the difference was statistically significant (p?=?0.031). No difference of fascin expression was found between histology types. Interestingly, in 8 tumors where the cranial base dura was eroded, there was a high level of fascin expression (p?=?0.047). These immunohistochemical findings suggest that fascin expression was correlated with tumor recurrence and high invasiveness, and that fascin overexpression may play an important role in the biologic behavior of skull base chordomas.