Invasion Of Adjacent Structures Research Articles

Risk of concomitant malignancy in hyperfunctioning adrenal incidentalomas

BackgroundAdrenal masses are common incidental findings on radiologic imaging. The association between malignancy and hormonal hyperactivity found in incidentally discovered adrenal tumors, however, remains unclear. MethodsA retrospective analysis of prospectively collected data from patients who underwent adrenalectomy for incidentally discovered adrenal tumors at a single institution. Outcomes and operative data were compared by univariate analysis. Area under the curve was used to analyze the effect of tumor size in predicting malignancy. ResultsThere were 49 patients who initially presented with adrenal incidentalomas that underwent adrenalectomy. Most patients were Caucasian women with an average age of 51 ± 14 years. Of this group, 24 patients underwent resection for hyperfunctioning adrenal glands. There were no significant differences in malignancy rates between hyperfunctional and nonfunctional tumors (4.1% vs. 12.0%, P = 0.32). On final histopathology, there were four patients with adrenal malignancies: two adrenocortical carcinomas and two metastatic from renal carcinoma. Only one patient with a hyperfunctioning adrenal tumor had underlying malignancy. Overall, invasion of adjacent structures (P < 0.001), presence of lymphadenopathy (P = 0.02), metastasis (P = 0.03), irregular tumor margins (P = 0.01), heterogeneity (P = 0.05), and tumor size >6 cm (P = 0.04) on radiologic imaging were strongly associated with malignancy in adrenal incidentalomas. ConclusionsThe risk of concomitant malignancy and hormonal hyperactivity in adrenal incidentalomas is very low. Tumor size (>6 cm) and radiographic features remain the most important predictors of adrenal malignancy, regardless of tumor function.

Pitfalls in the Staging of Cancer of Oral Cavity Cancer

Oral cavity cancer comprises nearly 30% of all malignant tumors of the head and neck. After a definitive diagnosis has been made, imaging is essential for staging the primary tumor by evaluating submucosal spread and invasion of adjacent structures, and to identify nodal or distant metastasis. Oral cavity anatomy is one of the most complex in the head and neck. Therefore, knowledge of anatomic subsites and spread patterns is critical for accurate staging. This article begins with a discussion of imaging techniques, and then presents a detailed review of normal anatomy followed by imaging's role in tumor staging highlighting potential pitfalls.

RETRACTED: Ten years of experience with microsurgical treatment of large and giant petroclival meningiomas

During a 10-year period, 41 patients underwent surgery for resection of large or giant petroclival meningiomas. Gross total resection (GTR) was accomplished in 25 patients (61.0%), subtotal resection (STR) in 15 patients (36.6%), and partial resection in one patient (2.4%). Postoperative complications were observed in 27 patients (65.9%). Postoperative radiosurgery was administered in six patients who had residual tumors. Survival and postoperative quality of life are the goals of successful surgery on large or giant petroclival meningiomas, and the strategic surgical approach is based on the tumor location, the direction of growth, the invasion of adjacent structures, patient age and neurosurgeon expertise. Selectively pursuing STR with radiotherapy rather than GTR is a reasonable strategy.

MRI and CT of anal carcinoma: a pictorial review

BackgroundSquamocellular anal carcinoma is increasingly diagnosed in patients with risk factors.MethodsState-of-the-art imaging with magnetic resonance imaging (MRI) using phased-array coils and volumetric multidetector computed tomography (CT) provides detailed visualisation of anal disorders, identification and extent assessment of neoplastic tissue, detection and characterisation of nodal and visceral metastases. MRI has been recommended by the European Society for Medical Oncology (ESMO) as the preferred modality of choice to stage anal cancer, taking into account the maximum tumour diameter, invasion of adjacent structures and regional lymph node involvement.ResultsCross-sectional imaging techniques allow the identification of coexisting complications, and differentiation from other perineal abnormalities.ConclusionCross-sectional imaging is useful for planning radiotherapy, surgical drainage or salvage abdomino-perineal resection. After chemo-radiotherapy, MRI follow-up provides confident reassessment of therapeutic response, persistent or recurrent disease.Teaching Points • Anal carcinoma is increasingly diagnosed in patients with human immunodeficiency virus (HIV), anoreceptive intercourse, chronic inflammatory bowel disease. • An established association exists with human papillomavirus (HPV) infection and premalignant intra-epithelial dysplasia. • Phased-array MRI is recommended as the preferred imaging modality for regional staging. • Imaging allows detection of infectious complications, planning of radiotherapy or salvage surgery. • Follow-up MRI allows reliable assessment of therapeutic response after chemo-radiotherapy.

Radical prostatectomy in very high-risk localized prostate cancer: Long-term outcomes and outcome predictors

The objective of this study was to present the long-term outcomes and determine outcome predictors in very high-risk (cT3b-T4) prostate cancer (PCa) after radical prostatectomy (RP). Between January 1989 and December 2004, 51 patients with cT3b-T4 PCa underwent RP. Kaplan-Meier survival analysis was used to calculate the biochemical progression-free survival (BPFS), clinical progression-free survival (CPFS), cancer-specific survival (CSS) and overall survival (OS) rate. Multivariate Cox proportional hazard models were used to determine the predictive power of clinical and pathological variables in BPFS and CPFS. Median follow-up was 108 months [interquartile range (IQR) 73.5-144.5]. The median serum prostate-specific antigen (PSA) was 16.9 ng/ml (IQR 7-37.2). Median biopsy and pathological Gleason (pGS) score were both scored as 7 (range 4-10 and 5-9, respectively). Overstaging was frequent (37.2%); four patients (7.8%) had organ-confined stage pT2, while 15 (29.4%) had extracapsular extension only (pT3a). Another 23 (45.1%) were confirmed with seminal vesicle invasion (pT3b) and nine (17.7%) had adjacent structure invasion (pT4). Eleven patients (21.6%) had lymph-node involvement. Thirty-two patients (62.7%) had positive surgical margins. The BPFS, CPFS, CSS and OS at 5 and 10 years were 52.7%, 45.8%;78.0%, 72.5%; 91.9%, 91.9% and 88.0%, 70.7%. In the multivariate Cox proportional hazard models, pathological stage was an independent predictor of BPFS while preoperative PSA and pGS was an independent predictor of CPFS. The management of cT3b-T4 PCa typically consists of a multimodality treatment in which RP is a valuable first step. Overstaging was frequent (37.2%), and almost one-quarter of the patients remained free of additional treatments. Long-term cancer-related outcomes were very satisfactory.

Gastrectomy with D2 lymphadenectomy for gastric cancer: Morbidity, mortality and survival

Background: Gastric cancer is the second leading cause of cancer death worldwide. Ninety percent of gastric cancers are adenocarcinomas. Overall, there is a decline in the incidence of gastric cancer in most countries over the past 50 years. Surgical resection is the most effective treatment for curable gastric cancer.Controversy still surrounds the value of extensive lymph node dissection in the curative treatment of gastric carcinoma.The overall 5-year survival rate among patients with resectable gastric cancer ranges from 10% to 30% in the western world. In contrast, Japanese publications report a marked improvement in survival (between 50% and 62%), largely attributed to lymph node resection known as D2 lymphadenectomy. Methods: The study was a prospective follow-up study on 50 consecutive patients with gastric cancer (without distant metastasis) between March 2007 and February 2010 in the Upper Gastrointestinal Surgery Unit, Faculty of Medicine, Alexandria University, Egypt. Patients less than 65 years with histologically proven and potentially curable gastric cancer were eligible for the study. Preoperatively, all patients underwent upper gastrointestinal endoscopy with biopsies and histopathologic examination. Computed tomography (CT) scans were done to look for metastases. Results: Fifty patients (36 men, 14 women) underwent gastric resection with D2- lymphadenectomy for gastric adenocarcinoma in a three-year period. Mean age was 46.6 years with a range of23-65 years. Almost 44% of patients had preoperative co-morbid disease. Weight loss with epigastric pain and dyspepsia was the most common presenting symptom (80%). Fifty patients underwent D2 resection, fifteen patients with radical total gastrectomy and intra­ abdominal oesophagojejunostomy, and thirty five patients with a subtotal distal gastrectomy and gastrojejunostomy. The hospital mortality rate was 4% (2/50). Morbidity rate was 44% (22/50), some of them presented with more than one complication. The median time of hospital stay was 22 days (mean 22.3, range 13-40). Follow-up included clinical, laboratory and CT examinations of all patients after surgery. In addition, when relapse was suspected, endoscopy with biopsy, and CT scan were performed. During the first 2 years follow-up, locoregional relapses were observed in 2 patients (local lymph nodes relapse). One patient presented with peritoneal recurrence, and 2 patients showed distant spread. The overall actuarial one-year survival in D2 patients was 92% and 76% at two years. Conclusion: The results obtained in our series of patients submitted to gastrectomy with D2 lymphadenectomy suggest that this technique offers low morbidity, mortality and acceptable 2-year survival rates. The survival benefit with D2 is obtained when a tumor invades muscularis propria or penetrates serosa without invasion of adjacent structures.

Postoperative chemoradiotherapy in high risk locally advanced gastric cancer

PurposeTo evaluate treatment outcome of patients with high risk locally advanced gastric cancer after postoperative chemoradiotherapy.Materials and MethodsBetween May 2003 and May 2012, thirteen patients who underwent postoperative chemoradiotherapy for gastric cancer with resection margin involvement or adjacent structure invasion were retrospectively analyzed. Concurrent chemotherapy was administered in 10 patients. Median dose of radiation was 50.4 Gy (range, 45 to 55.8 Gy).ResultsThe median follow-up duration for surviving patients was 48 months (range, 5 to 108 months). The 5-year overall survival rate was 42% and the 5-year disease-free survival rate was 28%. Major pattern of failure was peritoneal seeding with 46%. Locoregional recurrence was reported in only one patient. Grade 2 or higher gastrointestinal toxicity occurred in 54% of the patients. However, there was only one patient with higher than grade 3 toxicity.ConclusionDespite reported suggested role of adjuvant radiotherapy with combination chemotherapy in gastric cancer, only very small portion of the patients underwent the treatment. Results from this study show that postoperative chemoradiotherapy provided excellent locoregional control with acceptable and manageable treatment related toxicity in patients with high risk locally advanced gastric cancer. Thus, postoperative chemoradiotherapy may improve treatment result in terms of locoregional control in these high risk patients. However, as these findings are based on small series, validation with larger cohort is suggested.

Apocrine Hidrocystoma of the Cheek

Apocrine Hidrocystoma of the Cheek

Thyroid Paragangliomas Are Locally Aggressive

Thyroid paraganglioma (TP) is a very rare neoplasm that can be misdiagnosed. We evaluated the clinical and pathologic characteristics of three patients with TP. The records of all patients from 1981 to 2008 who had thyroidectomy with a final histologic diagnosis of TP were retrieved, and histology was reviewed by a single pathologist. Head and neck paragangliomas arising outside of the thyroid were excluded. TP accounted for 3 of all 6782 (0.04%) patients undergoing thyroidectomy during three decades. One patient has been previously reported and will not be discussed. In the remaining two patients and a surgical pathology consult case that we also describe herein, the mean age at diagnosis was 56 years (40-67) and two patients were men. Presenting features were indicative of advanced local invasion, including stridor, tracheal invasion, compression of the great vessels, and hemoptysis. The diagnosis of TP was not suspected preoperatively; in two patients, fine-needle aspiration (FNA) cytology was inadequate for diagnosis because of excessive blood. Intraoperative frozen section analysis suggested medullary thyroid cancer in two patients and oncocytic (Hurthle) cell carcinoma in one patient. Local invasion was common, requiring concurrent tracheal resection in two of three patients, and present histologically in all three patients. In all three cases, immunohistochemical analysis was negative for cytokeratin AE1/3, calcitonin, and thyroglobulin but positive for S100, highlighting sustentacular cells. After resection of a large TP with tracheal and vascular invasion, a 67-year-old woman experienced a 7-year disease-free interval. Primary TP is indeed rare. It does occur in men, frequently presents with compressive symptoms, and is typically locally aggressive, but does not appear to cause symptoms suggestive of catecholamine excess. Despite invasion of adjacent structures, aggressive resection can achieve a long disease-free interval.

Phase II trial of preoperative S-1 plus cisplatin followed by surgery for initially unresectable locally advanced gastric cancer

BackgroundThe aim of this study was to evaluate the efficacy and feasibility of preoperative chemotherapy with S-1 plus cisplatin in patients with initially unresectable locally advanced gastric cancer. MethodsWe enrolled patients with initially unresectable locally advanced gastric cancer because of severe lymph node metastases or invasion of adjacent structures. Preoperative chemotherapy consisted of S-1 at 80 mg/m2 divided in two daily doses for 21 days and cisplatin at 60 mg/m2 intravenously on day 8, repeated every 35 days. If a tumor decreased in size, patients received 1 or 2 more courses. Surgery involved radical resection with D2 lymphadenectomy. ResultsBetween December 2000 and December 2007, 27 patients were enrolled on the study. No CR was obtained, but PR was seen in 17 cases, and the response rate was 63.0%. Thirteen patients (48.1%) had R0 resections. There were no treatment related deaths. The median overall survival time (MST) and the 3-year overall survival (OS) of all patients were 31.4 months and 31.0%, respectively. Among the 13 patients who underwent curative resection, the median disease-free survival (DFS) and the 3-year DFS were 17.4 months and 23.1%, respectively. The MST and the 3-year OS were 50.1 months and 53.8%, respectively. The most common site of initial recurrence after the R0 resection was the para-aortic lymph nodes. ConclusionsPreoperative S-1 plus cisplatin can be safely delivered to patients undergoing radical gastrectomy. This regimen is promising as neoadjuvant chemotherapy for resectable gastric cancer. For initially unresectable locally advanced gastric cancer, new trials using more effective regimens along with extended lymph node dissection are necessary.

Costal hydatidosis: management and outcome of five cases

The objective of the study was to report our experience in the management of hydatid cyst of the rib. Between December 2001 and December 2009, five patients underwent surgery for costal echinococcosis, among 234 cases of thoracic hydatid cyst managed in our department. They were three males and two females, aged 19-42 years (mean = 32.2 years). Imaging investigations consisted of chest X-ray and computed tomography. All the patients underwent thoracotomy along the arch of the infected rib. Albendazole was administered postoperatively in all cases. The echinococcosis of the rib was primary in 3 cases, and secondary to thoracic localization in 2 cases. It was located on the right side in four patients, on the left one in one patient. The posterior arch was the site of infection in 3 cases, and the anterior arch in 2 cases. The cyst was intact in 4 cases, and the invasion of adjacent structures was found in one case. There were no concomitant hydatid lesions. The resection of the infected rib was total in 2 cases and partial in 3 cases. There was no postoperative mortality or complications. The follow-up ranged from 28 months to 8 years did not show any recurrence. Echinococcosis of the rib is very rare. To avoid complications and to allow the eradication of the disease, the combination of surgery and antihelmentic drugs remains the best therapeutic choice.

A to Z of Desmoid Tumors

The purpose of this article is to illustrate the common locations of desmoid tumors (deep fibromatosis), complications of intra- and extraabdominal desmoids, and treatment-related changes in their imaging appearance. Desmoids are locally aggressive fibrous tumors with a tendency to recur. Desmoids can be intraabdominal, in the abdominal wall, or extraabdominal. Complications, such as compression or invasion of adjacent structures, and abscess formation can occur. Treatment options include observation, local treatment (surgery, radiotherapy), or systemic therapy (conventional chemotherapy, molecular targeted agents).

Mediastinal mature teratoma in a Jehovah's Witness: Discrepancy between imaging and intraoperative findings

IntroductionPrimary mediastinal germ cell tumors are exceedingly rare but may present with a wide spectrum of elements. The occasional mediastinal teratoma that presents completely comprised of mature elements is a benign tumor but the appearance on imaging studies may be more suggestive of an invasive tumor. The treatment is complete resection but the assessment of resectability based on computed tomographic imaging can be misleading. Presentation of caseWe present a case of a 26 year old female, Jehovah's Witness who presented with a symptomatic mediastinal mass that on CT scan appeared to be unresectable due to presumed invasion of adjacent structures including the left pulmonary artery. Surgical exploration revealed an encapsulated, completely resectable mass which was excised without difficulty. Her early postoperative course was uneventful and at 18 months follow up is doing well without evidence of recurrence. DiscussionThe treatment of mature teratoma is complete surgical excision but the imaging studies may at times be misleading. We believe this case presents a unique clinical situation, since mediastinal mature teratomas are very rare and in addition, the preoperative decision to excise a mass becomes more complex in a case of a Jehovah's Witness. This case illustrates that the CT findings may be misleading when assessing a mediastinal mass. ConclusionResectability of a mediastinal mass can only be assessed at the time of operation and rarely should operation be denied solely on the basis of findings on imaging. Thus in these primary mediastinal tumors there should be a low threshold for proceeding with operation.

Surgery and High-Dose-Rate Intraoperative Radiation Therapy for Recurrent Squamous-Cell Carcinoma of the Anal Canal

Locoregionally recurrent squamous-cell carcinoma of the anal canal is managed with salvage surgery. High-dose-rate intraoperative radiation therapy has been used in selected patients with this disease to reduce the risk of local recurrence. The aim of this article is to present our institutional experience with this technique. Medical records of 14 patients with locoregionally recurrent squamous-cell carcinoma of the anal canal who underwent this technique between 1992 and 2007 were reviewed. The study was conducted at an academic cancer center. The median age was 45 years (range, 36-77), and 13 of the patients were women. All had prior radiation with or without chemotherapy. The surgical procedures included abdominoperineal resection with or without sacrectomy (n = 8), low anterior resection (n = 2), and pelvic exenteration (n = 4). The median radiation dose was 1500 cGy (range, 1500-1750). All cases of radiographic invasion of adjacent structures correctly predicted pathologic invasion. There was pathologic invasion into adjacent structures in 11 cases (79%), and adherence to the sacrum without invasion in 2 cases (14%). Surgical margins were positive (n = 6), close (<1 mm) (n = 3), and negative (n = 5). The median follow-up from our technique was 17 months (range, 5-145). Subsequent recurrence occurred in 11 cases, at a median of 8 months from treatment. Two-year actuarial control was 7.1%, and the overall survival was 21.4%. Acute toxicities included wound-healing complications (n = 6); gastrointestinal obstruction (n = 5); neurogenic bladder (n = 1); ureteral stricture (n = 3); and peripheral neuropathy (n = 2). This is a small retrospective series in which the meaningful analysis of associations between clinical variables and outcomes was not possible. Salvage surgery with high-dose-rate intraoperative radiation therapy did not appear to be associated with a locoregional control or survival benefit in this series. The addition of high-dose-rate intraoperative radiation therapy to salvage surgery is insufficient to compensate for positive surgical margins. Preoperative imaging should be used to aid in patient selection to identify those patients in whom negative margins can be obtained and to aid in the determination of appropriate salvage surgery.

Case 171: Facial Nerve Hemangioma

The patient’s imaging features, especially the honeycomb pattern of ossific changes in the geniculate fossa, were virtually pathognomonic for ossifying hemangioma of the facial nerve.

Learning from Haiti

In May, 2010, a 37-year-old woman, displaced from Port-au-Prince after the January earthquake, presented to Hopital St Therese in Hinche, Haiti, with a 2-year history of a slowly growing abdominal mass with associated dyspepsia, intermittent abdominal pain, anorexia, and weight loss. Her surgical history included four caesarean section deliveries and tubal ligation. Physical examination found her in no acute distress, with stable vital signs and a large, mobile abdominal mass extending to the subhepatic space. Haemoglobin was 106 g/L. A handwritten ultrasonography report from Port-au-Prince before the earthquake reported that she had a 10 cm mass with no further description, although on physical examination the mass was palpated at twice that size. No other laboratory or imaging capabilities were available. With no intensive care capabilities and limited transfusion supplies, local physicians had deferred surgery owing to diagnostic uncertainty. In July, 2010, a humanitarian surgical team brought a portable ultrasonography device to Hinche. Imaging of our patient showed a well-circumscribed, homogeneous, hypoechoic mass without invasion of adjacent structures (fi gure). The surgeons felt comfortable doing an exploratory laparotomy which revealed a well-demarcated, cystic ovarian mass, about 20 cm in diameter, resected via right oophorectomy (webappendix). Gross examination was consistent with a simple ovarian cyst. Diff erential diagnosis included ovarian and uterine tumours, pancreatic pseudocyst, and mesenteric cyst. She was discharged 2 days later with follow-up by local physicians. Our patient’s case demon strated the usefulness of portable devices in resource-limited settings. The well-circumscribed appearance of the mass and lack of invasion of adjacent structures provided enough supplementary information for surgeons to proceed safely at Hopital St Therese despite limited resources. Further delay in treatment would have resulted in persistent disability and possible torsion. In February, 2011, at last follow-up, she had had no complications. Diagnostic ultrasonography is a recognised, costeff ective aid for procedure-based care in under-resourced areas of the world. However, access to ultrasonography in these settings is limited. While we were in Haiti, ultrasonography also aided in the evaluation of soft-tissue masses, peritoneal disease, and uterine pathology. We recognise limitations of ultra sonography in these settings. Durability, weight, battery life, and ease of use all hindered our use of the device, but the greatest limitation of ultrasonography is cost. Although an aff ordable imaging modality, eff ective ultrasonography requires proper training, and the costs of both equipment and training can still exceed the budget for care of patients outside the industrialised world. Ideally, local physicians would be trained in diagnostic and therapeutic ultrasonographic techniques to allow for the continued benefi t of the local population. Although investment in both equipment and training would be needed, eff orts to implement programmes with portable ultrasonography in Tanzania and Rwanda have shown initial success, and we believe ultrasonography can play a vital role in resource-poor settings.

Dual inhibition of epidermal growth factor receptor and insulin‐like growth factor receptor I: Reduction of angiogenesis and tumor growth in cutaneous squamous cell carcinoma

Cutaneous squamous cell carcinoma (CSCC) is the second most common nonmelanoma skin cancer. Most of the approximately 250,000 cases occurring annually in the United States are small, nonaggressive, and cured by excision alone. However, a subset of these tumors which are defined by poorly differentiated histology, large tumor size, invasion of adjacent structures, and/or regional metastases can prove resistant to treatment despite adjuvant radiotherapy and can have an increased risk of recurrence and nodal metastasis. Novel therapeutic approaches are necessary to improve the outcomes for patients with aggressive CSCC. We analyzed the effect of targeted therapy on the growth and survival of CSCC cell lines using an anti-insulin-like growth factor-I receptor (IGF-IR) antibody, A12, alone or in combination with an anti-epidermal growth factor receptor (EGFR) antibody, cetuximab, both in vitro and in vivo in an athymic nude mouse model of CSCC. Treatment with A12 and cetuximab inhibited the signaling pathways of IGF-IR and EGFR and inhibited proliferation and induced apoptosis of squamous cell carcinoma (SCC) cell lines in vitro. Immunohistochemical staining revealed decreased proliferating cell nuclear antigen (PCNA), microvessel density, and increased apoptosis within the treated tumor xenografts. In addition, the administration of A12, alone or in combination with cetuximab inhibited the growth of tumors by 51% and 92%, respectively, and significantly enhanced survival in the nude mouse model of CSCC (p = .044 and p < .001, respectively). These data suggest that dual treatment with monoclonal antibodies to the EGFR and IGF-IR may be therapeutically useful in the treatment of CSCC.

The Value of MR Imaging When the Site of Uterine Cancer Origin Is Uncertain

To assess the value of magnetic resonance (MR) imaging in determining the site of origin of newly diagnosed uterine cancer (corpus vs cervix) when clinical and/or histologic evaluation is indeterminate. The Institutional Review Board approved and waived informed consent for this HIPAA-compliant study of 59 women (median age, 59 years; range, 28-84 years) who underwent pelvic MR imaging to determine the anatomic origin of uterine cancer. Two radiologists independently retrospectively assessed all MR imaging studies. In 48 patients who underwent hysterectomy, surgical pathologic findings were the reference standard, and overall test yields and diagnostic likelihood ratios were measured. Accuracy in detecting invasion of adjacent structures was also calculated. For the remaining patients, imaging and biopsy findings are presented descriptively. At hysterectomy, 32 patients had uterine corpus cancer and 16 had cervical cancer. Overall test yields for reader 1 and reader 2 were 0.85 and 0.88, respectively. When a reader attributed a tumor's origin to either the uterine corpus or cervix, the odds of the tumor originating from that site were 4.80-6.35 times greater than they would have been if no other information were available. Accuracy levels in detecting invasion of the myometrium, cervical stroma, parametria and/or adnexae, and vagina, respectively, were 72%, 69%, 74%, and 85% for reader 1 and 78%, 77%, 76%, and 85% for reader 2. MR imaging is useful for determining the anatomic origin of uterine cancer and provides helpful information regarding invasion of adjacent structures.

The role of magnetic resonance imaging in the diagnostic evaluation of malignant peripheral nerve sheath tumors

Malignant peripheral nerve sheath tumors (MPNSTs) are the leading cause of mortality in patients with neurofibromatosis type-1 (NF1)); however, they may also arise sporadically. Differences in magnetic resonance imaging (MRI) features between MPNSTs arising in NF1 subjects versus non-NF1 subjects have not been studied before. The accuracy of MRI in distinguishing MPNSTs from benign peripheral nerve sheath tumors (BPNSTs) has also been debated. The objective of this study was to determine the potential differentiating MRI features between (a) NF1-related and non-NF1-related MPNSTs and (b) MPNSTs and BPNSTs. We retrospectively evaluated the MRI studies of 21 patients (12 NF1 subjects and nine non-NF1 subjects) with MPNSTs and 35 patients with BPNSTs. In all studies, the lesions were assessed in terms of size, margins, T1 and T2 signal characteristics, internal architecture, pattern of contrast enhancement, invasion of adjacent structures and necrosis/cystic degeneration as well as for the presence of tail-, target- and split-fat signs. MPNSTs of NF1 subjects occurred at an earlier age and displayed a higher incidence of necrosis/cystic degeneration compared with MPNSTs of non-NF1 subjects. Compared with BPNSTs, MPNSTs were significantly larger at the time of diagnosis and demonstrated a higher incidence of ill-defined margins (specificity 91%, sensitivity 52%) and invasion of adjacent structures (specificity 100%, sensitivity 43%). Differences exist between NF1-related and non-NF1-related MPNSTs regarding the age of occurrence and MRI appearance. In the MRI evaluation of peripheral nerve sheath tumors, the presence of ill-defined tumor margins and/or invasion of adjacent structures are highly specific for malignancy.

Surgically treated aggressive mediastinal sarcoma: a tumor of possible thymic origin

True sarcoma of thymus has no epithelial component. This tumor behaves aggressively with invasion of adjacent structures. Only a few anecdotal case reports of this tumor are found in the English-language literature, and they describe a poor outcome. This case report describes a rapidly growing thymic sarcoma diagnosed at an advanced stage with a compressive effect on the heart. The tumor was resected en bloc with adjacent invaded structures.