Transmural Tumors Research Articles

Mesenteric lymphoma - a case report

Gastrointestinal stromal tumors (GISTs) comprise a group of smooth muscle mensenchymal alimentary tract tumors of variablemalignancy (1) GISTs may ouccur anywhere along the gastrointestinal tract, they are most commont located in the stomach (50-60%) and the small intestine (30-35%), and less frequenty in the colon, rectum and esophagus (2). More rarely, GISTs may arisefrom other intra-abdoment soft tissues within the abdominal cavity, usually in the omentum, mesentery or the retroperitoneumcalled extra-Gastrointestinal stromal tumors (EGIST). Three macroscopic growth patterns of GIST: intraluminal, exophytic(extraluminal), and transmural tumors with the distribution was even (3).In this study, we describe case report of 70-year-old man was admitted to our hospital because of abdominal pain over 6months with no pathological antecedent. Abdominal ultrasound (US) reveal a omental solid mass in left lumbar region, andon CT scan show a solid mass in left lumbar region in front of the descending colon, maesuring around 50x87x133mm withwell-circumscribed, slight enhance with intravenous contrast, no hemmorrage or necrosis area, no calcification and not clearlyseparated from small-bowel. Patient has laparoscopic surgeon, diagnosis was a pedunculated exoluminal gastrointestinal stromal tumor (GIST). Histopathological examination report and the CD-117 immunoreactive score is suitable of endoscopicallyinvisible medium sized.

Role of endoscopic ultrasound in esophageal cancer.

Esophageal cancer (ECA) affects 1 in 125 men and 1 in 417 for women and accounts for 2.6% of all cancer related deaths in the United States. The associated survival rate depends on the stage of the cancer at the time of diagnosis, making adequate work up and staging imperative. The 5-year survival rate for localized disease is 46.4%, regional disease is 25.6%, and distant/metastatic disease is 5.2%. Additionally, treatment is stage-dependent, making staging all that much important. For nonmetastatic transmural tumors (T3) and/or those that have locoregional lymph node involvement (N), neoadjuvant therapy is recommended. Conversely, for those who have earlier tumors, upfront surgical resection is reasonable. While positron emission tomography/computed tomography and other cross sectional imaging modalities are exceptional for detecting distant disease, they are inaccurate in staging locoregional disease. Endoscopic ultrasound (EUS) has played a key role in the locoregional (T and N) staging of newly diagnosed ECA and has an evolving role in restaging after neoadjuvant therapy. There is even data to support that the use of EUS facilitates proper triaging of patients and may ultimately save money by avoiding unnecessary or futile treatment. This manuscript will review the current role of EUS on staging and restaging of ECA.

Integrating Anatomical, Molecular and Clinical Risk Factors in Gastrointestinal Stromal Tumor of the Stomach

BackgroundAdjuvant imatinib for 3 years is recommended to patients with high-risk gastrointestinal stromal tumor (GIST). Risk stratification is inaccurate, and risk assessments are further complicated by the increased use of neoadjuvant treatment. Anatomical criteria for prognostication have not been investigated.MethodsClinical, molecular, and anatomical variables were retrospectively studied in a population-based cohort of 295 patients with gastric GIST resected between 2000 and 2018. Gastric subsite was divided into the upper, middle, and lower thirds. Growth pattern was classified as luminal, exophytic, or transmural based on imaging and surgical reports.ResultsOf 113 tumors in the upper third of the stomach, 103 (91.2%) were KIT mutated, 7 (6.2%) were PDGFRA mutated, and 104 (92.0%) harbored genotypes sensitive to imatinib. Transmural tumors were strongly associated with a high mitotic index. Five-year recurrence-free survival (RFS) was 71% for patients with transmural tumors versus 96% with luminal or exophytic tumors (hazard ratio [HR] 8.45, 95% confidence interval [CI] 3.69–19.36; p < 0.001), and, in high-risk patients, 5-year RFS was 46% for patients with transmural tumors versus 83% with luminal or exophytic tumors (HR 4.47, 95% CI 1.71–11.66; p = 0.001). Among 134 patients with tumors > 5 cm, there were 29 recurrences. Only five patients with exophytic or luminal tumors had recurrent disease, of whom four had tumor rupture. Five-year RFS for patients with exophytic/luminal tumors >5 cm without rupture was 98%.ConclusionsIn the upper third, over 90% of tumors were sensitive to imatinib. Patients with exophytic or luminal tumors without rupture, irrespective of size, had an excellent prognosis and may not benefit from adjuvant therapy.

A comprehensive characterization of anatomical and molecular risk factors in gastric gastrointestinal stromal tumor.

e23522 Background: Gastric gastrointestinal stromal tumors (GISTs) demonstrate a remarkable diversity in clinical behavior. Established risk criteria identify patients prone to recurrence with high sensitivity, but factors that could add specificity are needed. The aim of this study was to give a comprehensive picture of localized, gastric GIST and to investigate prognostic factors in a large population-based series. Methods: Patients with primary gastric GISTs completely resected between 2000 and 2018 were identified in the sarcoma database at Oslo University Hospital. Gastric subsite was divided into the upper, middle and lower thirds. Macroscopic growth pattern was coded as luminal, exophytic or transmural based on imaging and surgical reports. Mutation analysis was performed by Sanger sequencing of selected exons in KIT and PDGFRA and by targeted next-generation sequencing. Results: The cohort comprised 295 patients, representing 98% of patients in the region reported to the Cancer Registry of Norway. Of 292 tumors suitable for anatomical classification, 122 were located in the upper third of the stomach (41.8%), 120 in the middle (41.1%), and 50 in the lower third (17.1%). The number of luminal, exophytic and transmural tumors were 100 (34.1%), 94 (32.1%) and 99 (33.8%), and 2 tumors were not possible to classify. KIT mutations were detected in 183 of 256 tumors (71.5%), PDGFRA mutations in 62 (24.2%), whereas only 11 patients (4.3%) had KIT and PDGFRA wild-type tumors. Transmural tumors were larger, more often located in the upper third, had higher mitotic activity, and more frequently had KIT del557/558 mutations. KIT mutated tumors had a predilection for the upper two thirds and PDGFRA mutated tumors for the lower two thirds. Recurrences were rare among patients with luminal or exophytic tumors, with a 5-year RFS of 96% versus 71% with transmural tumors (HR 8.45; 95% CI 3.69-19.36; P&lt; 0.001). RFS was inferior with transmural tumor growth also among high-risk patients, 46% versus 83% at 5 years ( P= 0.001). Excluding patients with tumor rupture, there was only one recurrence among 24 high-risk patients with luminal or exophytic primaries, of whom 11 did not receive adjuvant therapy. Conclusions: This study provides a comprehensive, population-based description of the anatomical and molecular landscape of gastric GIST, with findings of potential clinical relevance. Transmural tumor growth was a predictor of poor outcome and could supplement established risk factors in selecting patients for adjuvant imatinib treatment.

Leiomyoma Infarction after Uterine Artery Embolization: Influence of Embolic Agent and Leiomyoma Size and Location on Outcome

PurposeTo study the factors that might impact infarction of individual uterine leiomyomas and total tumor burden after uterine artery embolization (UAE). Materials and MethodsThis retrospective study included 91 patients (mean age, 44 y [range, 34–54 y]) who underwent UAE with tris-acryl gelatin microspheres (TAGMs) or nonspherical polyvinyl alcohol (PVA) particles. Twenty-one patients were treated with PVA (23%) and 70 were treated with TAGMs (77%). A total of 356 uterine leiomyomas were assessed, with a median uterine volume of 533 cm3 (range, 321–848 cm3). A reader masked to demographic and technical details reviewed contrast-enhanced magnetic resonance images before and 3 months after UAE to estimate the extent of tumor infarction. ResultsThere was no significant difference in global or individual tumor infarction rate between embolizations with TAGMs and PVA particles (P = .73 and P = .3, respectively). Global infarction was not affected by age (P = .53), race (P = .12), number of leiomyomas (P = .72), or uterine volume (P = .74). Leiomyoma size did not influence individual tumor infarction (P = .41). Leiomyoma location was the sole factor that influenced individual tumor infarction rates, with pedunculated serosal tumors significantly less likely to show complete infarction than transmural tumors (odds ratio, 0.24; P = .01). ConclusionsNonspherical PVA particles and TAGMs produce similar rates of uterine leiomyoma infarction. Complete infarction of individual tumors is less likely in serosal and pedunculated serosal tumors.

Detailed clinicopathological characteristics and possible lymphomagenesis of type II intestinal enteropathy-associated T-cell lymphoma in Japan.

Twenty-six Japanese cases of type II enteropathy-associated T-cell lymphoma (EATL) were examined. Multiple tumors throughout the small intestine were found in 15 patients (58%) and duodenal and colonic mucosal lesions in 8 and 6 cases, respectively. Histologically, intramucosal tumor spread and a zone of neoplastic intraepithelial lymphocytes (IELs) neighboring the main transmural tumors were detected in 20 (91%) and 17 (77%) of the 22 cases examined, respectively. Inside and outside the IEL zone, some degree of enteropathy with many reactive small IELs and villous atrophy was detected in 11 cases (50%). Immunohistologically, many CD56/CD8-positive small IELs were found in the enteropathic lesions of 4 (36%) and 7 (64%) of these 11 cases. Lymphoma cells expressed tyrosine kinase receptor c-Met, serial phosphorylated (p)-mitogen-activated protein kinase/extracellular signal-regulated kinase, c-Myc, and Bcl2 in 18 (78%), 21 (91%), 11 (42%), and 19 (73%) of the total cases, respectively. By fluorescence in situ hybridization, chromosomal loci 7q31 (c-Met) and 8q24 (c-Myc) were amplified in 11 (65%) and 12 (71%) of the 17 cases analyzed. Gain of 7q31 and c-Met expression were significantly (P < .01) higher than in peripheral CD8-positive T-cell or CD56-positive natural killer-cell lymphomas. Enteropathy was seen near the IEL zone in type II EATL, and activation of the c-Met, mitogen-activated protein kinase/extracellular signal-regulated kinase-mitogen-activated protein kinase pathway, and c-Myc-Bcl2-mediated cell survival may play important roles in lymphomagenesis, converting enteropathy to type II EATL. Seven cases in the early clinical stages I and II-1 showed significantly (P < .01) better prognoses than did those in the advanced stages. Early detection of the mucosal lesions and tumors may improve patient prognosis.

Multidetector Computed Tomography Virtual Cystoscopy: An Effective Diagnostic Tool in Patients With Hematuria

To evaluate the efficacy and the potential use of multidetector computed tomography virtual cystoscopy (MDCT-VC) in patients with gross hematuria. A total of 32 patients underwent MDCT-VC, cystoscopy, and a cytologic examination. The slice thickness of MDCT was 1 mm. Bladder distension was done with room air. The data were converted into 3-dimensional virtual reconstructive models. The data sets were reviewed independently by 2 experienced radiologists. Tumors confined to the mucosa, infiltrating the muscularis, and transmural tumors were distinguished. VC showed a sensitivity and specificity of 100%. The radiologic accuracy regarding T stage correlated in 87.5%. MDCT-VC identified 21 bladder lesions suspicious for bladder cancer in 18 patients. The histologic results showed 22 patients with bladder lesions, 18 were diagnosed with transitional cell carcinoma of the bladder, 3 had bladder endometriosis, and 1 had an infiltrating colon cancer. Four patients had concomitant carcinoma in situ lesions, which were not seen completely with MDCT-VC. However, cytology was positive in those cases. Ten patients did not have any tumor signs on VC and the subsequent conventional cystoscopy did not bring any change to the initial tumor-free diagnosis of VC. MDCT-VC combined with urine cytology is a good alternative to conventional cystoscopy for patients with painless gross hematuria. It should be used as a decision-making aid to identify patients who will benefit from additional cystoscopic examination. Future developments should focus on the visibility of sessile and carcinoma in situ lesions.

Hybrid Procedure for Obstructive Neonatal Left Ventricular Tumors

We report the use of the hybrid procedure (bilateral pulmonary artery banding, ductal stenting followed by balloon atrial septostomy) in the first week of life for the treatment of two neonates with large left ventricular fibromas resulting in univentricular physiology. At 6 months of age, second-stage palliation was accomplished and the 2 patients are growing well with normal developmental milestones and no atrial or ventricular ectopy at 30 months. Hybrid strategy offers a less invasive, initial intervention and the options of typical second-stage univentricular palliation, conversion to biventricular physiology in cases of tumor regression, planned surgical resection, or transplantation.

En bloc vs transhiatal esophagectomy for stage T3 N1 adenocarcinoma of the distal esophagus.

En bloc esophagectomy (EBE) provides improved survival over transhiatal esophagectomy (THE) in patients with similarly sized transmural tumors (T3) and lymph node metastases (N1). A retrospective case-control study of 2 methods of esophageal resection for cancer. University hospital (tertiary referral center for esophageal disease). There were 49 patients (27 who underwent EBE and 22 who underwent THE) with similar T3 N1 disease and the following matched criteria: tumors of similar size and location, more than 20 lymph nodes in the surgical specimen, R0 resection, no previous chemotherapy or radiation therapy, and follow-up until death or for a minimum of 5 years. Main Outcome Measure Survival adjusted for differences in demographic and patient characteristics. The number of nodes harvested was greatest after EBE vs THE (median, 52 vs 29 [range, 21-85 vs 20-60]; P<.001). The median number of involved nodes was similar after EBE vs THE (median, 5 vs 7 [range, 1-19 vs 1-16]). The only 2 independent factors that affected survival in a Cox analysis were the number of involved lymph nodes (P =.01) and the type of resection (P =.03). Patients who underwent EBE had a survival benefit over those who underwent THE (P =.01). The survival benefit of EBE was seen only in patients with fewer than 9 involved lymph nodes (P<.001). En bloc esophagectomy confers a better survival than THE in patients with T3 N1 disease and fewer than 9 lymph node metastases.

Gastrointestinal stromal tumors and leiomyosarcomas in the colon: a clinicopathologic, immunohistochemical, and molecular genetic study of 44 cases.

Gastrointestinal stromal tumors (GISTs), mesenchymal tumors largely specific for the gastrointestinal tract, have been well defined in the stomach and small intestine, but have not been extensively documented or contrasted with true smooth muscle tumors in the colon. This study was undertaken to determine the clinicopathologic features of GISTs of the colon, excluding the rectum, and to compare them with leiomyosarcomas (LMSs) of the same location. A total of 37 colonic GISTs and seven LMSs from the files of the Armed Forces Institute of Pathology and the Haartman Institute of the University of Helsinki were analyzed. The GISTs occurred predominantly in adults older than 50 years of age (median, 67 yrs), and most were histologically malignant; four small benign tumors (< or = 1 cm) were incidentally detected, and 10 others had minimal mitotic activity (five or fewer mitoses per 50 high-power fields). The colonic GISTs were typically transmural tumors with frequent intraluminal and outward bulging components. Histologically, they usually showed a spindle cell pattern (92%), whereas 8% were epithelioid. Most tumors (19 of 25) were positive for CD117 (KIT) and for CD34 (16 of 27); six tumors coexpressed alpha-smooth muscle actin and CD117; none showed desmin or S-100 protein. C-kit mutations in exon 11 were seen in 5 (36%) of 14 colonic GISTs. None of the patients with incidental small tumors had a recurrence, whereas 2 of 10 patients with tumors larger than 1 cm but minimal mitotic activity died of the disease with liver metastasis. Nearly all patients whose tumor was larger than 1 cm and showed more than five mitoses per 50 high-power fields died of disease; half had evidence of metastasis. LMSs were typically intraluminally bulging, polypoid masses that showed a histologic likeness to differentiated smooth muscle cells. They occurred in five men and two women with a median age of 61 years. Most LMSs were high-grade histologically and showed smooth muscle actin, desmin, or both. All were negative for CD34 and CD117 and lacked c-kit mutations. Five of the seven patients died of disease, and two had a long-term survival, despite high mitotic activity. These results show that KIT-positive GISTs are more common than LMSs of the colon, and these tumor groups have clinicopathologic differences that warrant their separation.

Endometriosis-associated intestinal tumors:A clinical and pathological study of 6 cases with a review of the literature

This cliniopathologic study of primary Mullerian tumors of thebowel arising in foci of endometriosis is based on six new cases and an analysis of 17 previously reported cases. Varieties of Mullerian tumors occur in the bowel; the most common types are endometrioid carcinoma, followed by various mixed Mullerian tumors and stromal sarcomas. Sevent-eight percent develop in the rectosigmoid colon, the remaining in the cecum or ileum. Those in the latter area tend to be sarcomas or mixed Mullerian tumors. Certain architectural growth characteristics, derived from precursor endometriosis, are common to most endometriosis-associated intestinal tumors (EAITs). Seventy percent of EAlTs occur in the outer bowel wall. Transmural tumors tend to form lu rdnal polyps and assume an hourglass shape. Metachronous or synchronous Mullerian tumors occur in 39% of cases. Seventy percent of women with EAlTs are in their mid 30s to early 50s. Common presenting symptoms are abdominal or pelvic pain, melena, and an abdominal or pelvic mass. Documented in 26% of patients is a history of prolonged unopposed estrogen therapy. Only 28.5% of cases die of their tumors, but follow-up is less than 5 years in all but 2 patients.

Comparison of a Linear Miniature Ultrasound Probe and a Radial-scanning Echoendoscope in TN Staging of Esophageal Cancer

Background: Endoscopic ultrasonography is a precise method for TN staging of esophageal cancer. We explored the staging properties of a linear miniprobe as compared with a radial-scanning echoendoscope. Methods: Sixty-eight patients with esophageal cancer underwent preoperative TN staging using a 20-MHz linear miniprobe and a 7.5/12-MHz radial-scanning echoendoscope. Tumor stage was verified by surgery and/or histology. Results: T and N stages were verified in 53 and 54 patients, respectively. T-staging accuracy using the echoendoscope was 70%. The high-frequency miniprobe could not differentiate between T3 and T4 tumors, but both systems had an accuracy of 87% in discriminating between T1, T2, and T3/4 stages. With traversable tumors, the accuracy of N staging was significantly better with the echoendoscope than with the miniprobe (90% vs. 48%, P = 0.008). Conclusions: The two endosonographic systems had similar accuracy for assessing transmural tumor growth, but the echoendoscope was superior in staging advanced transmural tumors and in predicting lymph node metastasis with traversable tumors.

Node status in transmural esophageal adenocarcinoma and outcome after en bloc esophagectomy

Objective: Adenocarcinoma has replaced squamous cell as the most common esophageal cancer in the United States. The purpose of this study was to determine the prevalence and location of lymph node metastases, the feasibility of performing an R 0 resection, and disease recurrence and survival in patients with transmural adenocarcinoma of the lower esophagus and gastroesophageal junction. Methods: Forty-four patients with transmural adenocarcinoma underwent en bloc esophagectomy with systematic thoracic and abdominal lymphadenectomy. They were followed up for a median of 23 months. Results: Actuarial survival for the entire group was 26% at 5 years. The most important predictors of the likelihood of recurrent disease and 5-year survival were the presence and number of lymph node metastases and the ratio of involved to total removed nodes. Seven patients (16%) were found to have no lymph node metastases and had an 85% 5-year survival. In contrast, patients with more than 4 involved nodes or a node ratio greater than 0.1 had a high likelihood of recurrence and death. Location of involved lymph nodes did not predict the likelihood of recurrence or death. Despite all patients having transmural tumors, recurrence within the field of the en bloc resection occurred in only 1 patient (2%). Conclusions: En bloc esophagectomy in patients with transmural esophageal adenocarcinoma is required to obtain the survival benefit of an R 0 resection, to adequately assess lymphatic tumor burden, and to be able to predict the likelihood of recurrence and death and thereby guide the use of postoperative adjuvant therapy. (J Thorac Cardiovasc Surg 1999;117:960-8)

Results of local excision followed by postoperative radiation therapy for rectal cancer

There is increasing interest in the use of local excision and postoperative radiation therapy (RT) as a sphincter-sparing approach for selected rectal cancers. The data suggest that this treatment approach should be limited to patients with either T1 tumors with adverse pathologic factors or T2 tumors. Transmural (T3) tumors have a 25% local recurrence rate with this technique and are treated more effectively with standard surgery and pre- or postoperative therapy. The results of local excision and postoperative RT are encouraging, however, more experience is needed to determine if this approach will have similar local control and survival rates as standard surgery.

Nodal metastasis and sites of recurrence after en bloc esophagectomy for adenocarcinoma

The operative specimens from 43 patients undergoing en bloc esophagectomy for adenocarcinoma of the lower esophagus or cardia were analyzed. Depth of invasion of the tumor and extent and location of lymph node metastases were determined. Postoperative recurrence was identified from positive findings on successive 3-month computed tomographic scans. Positive nodes occurred in 33% ( 2 / 6 ) of intramucosal tumors, 67% ( 6 / 9 ) of intramural tumors, and 89% ( 25 / 28 ) of transmural tumors ( p < 0.01). Commonly involved nodes were those in the lesser curve of the stomach (42%), parahiatal nodes (35%), paraesophageal nodes (28%), and celiac nodes (21%). Excluding perioperative deaths, follow-up was complete for 38 patients. Twenty patients had recurrence. Fifteen patients (40%, 15 / 38 ) had nodal recurrence: cervical, 7.9% ( 3 / 38 ); superior mediastinal, 21% ( 8 / 38 ); and abdominal, 24% ( 9 / 38 ) (retropancreatic in 7 and retrocrural in 2). Of 5 patients with nodal recurrence alone, 3 (60%) had recurrence at sites outside the margins of resection. Patients with four metastatic nodes or less had a survival advantage over those with more than four ( p < 0.05). There was no difference in survival according to location of nodal metastases. Two (22.2%) of 9 patients with celiac node metastases survived longer than 4 years. Adenocarcinoma of the lower esophagus and cardia spreads widely to mediastinal and abdominal nodes, and death can occur from nodal disease. Rates of lymph node metastases increase with the depth of the primary tumor. Patients with lymphatic metastases can be cured particularly if there are fewer than four nodes involved. Curative surgical therapy necessitates wide lymph node resection to ensure removal of all metastatic nodes.

Clinical experience with local excision and postoperative radiation therapy for rectal cancer

The standard surgical treatment for patients with potentially curable transmural and/or node-positive rectal cancer is a low anterior resection or abdominoperineal resection. There is increasing interest in the use of local excision and postoperative radiation therapy as primary therapy for selected rectal cancers. The limited data suggest that the approach of local excision and postoperative radiation therapy should be limited to patients with either T1 tumors with adverse pathologic factors or T2 tumors. Transmural tumors have a 24 percent local failure rate and are treated more effectively with standard surgery and preoperative or postoperative therapy. The results of local excision and postoperative radiation therapy are encouraging; however, more experience is needed to determine whether this approach ultimately has local control and survival rates similar to standard surgery.

Primary upper small-intestinal lymphoma and alpha-chain disease

Ten cases of primary upper small-intestinal lymphoma associated with alpha-chain protein in serum were discovered in a prospective study of the sera of patients with immunoproliferative small-intestinal disease (IPSID). Patients were mostly young males presenting with abdominal pain, weight loss, and diarrhea and showing laboratory evidence of carbohydrate, fat, and vitamin B12 malabsorption and hypoalbuminemia. The more frequently encountered pathologic abnormality was a diffusely nodular jejunal mucosa produced by a plasmacytic infiltrate of variable cell maturity involving a varible depth of small bowel wall with or without involvement of the mesenteric or para-aortic-lymph node complex and, in one instance, the liver. A less frequent picture included circumferential ulcerative and constrictive transmural tumors of the upper small intestine produced by a malignant lymphoma with involvement of abdominal lymph nodes. Small-intestinal surface epithelial abnormalities, a dense mantle of mature plasma cells overlying the lymphoma, a pronounced follicular lymphoid hyperplasia adjacent to and at distances from the lymphoma were other features of note in our IPSID cases associated with alpha-chain protein.