Primary rectal squamous cell carcinoma treated with surgery and radiotherapy

Primary squamous cell carcinoma of the rectum is a rare malignancy,and the discrete dual lesions of rectum are even rarer.There is currently no effective and satisfactory treatment for this disease.Here we report a case of an elderly female with bi-primary squamous cell carcinoma of the rectum treated with radical resection and radiotherapy.The patient is still alive 43mo after the initial curative resection of the tumor.We suggest that surgery as the primary treatment followed by concomitant radiotherapy may be an effective protocol for elderly patients with rectal squamous cell carcinoma.     

Emergency caudate lobectomy for ruptured hepatocellular carcinoma with multiple primary cancers

We report a case of metachronous multiple primary malignancies involving both rectum and liver with colonic metastasis from hepatocellular carcinoma(HCC) through hematogenous pathway.A 72-year-old woman was admitted to the emergency department with right upper abdominal pain for 4 h.Considering her surgical history of Mile’s procedure plus liver resection for rectal cancer with liver metastasis three years ago and the finding of urgent computed tomography scan on admission,the preoperative diagnosis was spontaneous rupture of rectal liver metastasis located in caudate lobe and colonic metastasis from rectal cancer. The patient underwent an emergency isolated caudate lobectomy at a hemorrhagic shock status.Pathology reported a primary HCC in the caudate lobe and colonic metastasis of HCC with tumor embolus in the surrounding vessels of the intestine.No regional lymph node involvement was found.It is hypothesized that HCC may disseminate hematogenously to the ascending colon,thus making it a rare case.     

Giant gastrointestinal stromal tumour of rare sarcomatoid epithelioid subtype:Case study and literature review

Gastrointestinal stromal tumours(GISTs) are the most common mesenchymal tumours of the gastrointestinal tract,but they represent less than 3% of all gastrointestinal tract malignancies.This is a detailed case study of a 52-yearold male patient treated for very uncommon histological subtype of gastric GIST with atypical clinical presentation,asymptomatic progress and late diagnosis.The resected tumour,giant in diameters,was confirmed to represent the most rare histopathologic subtype of GISTs- sarcomatoid epithelioid GIST.We report this case and review the literature with a special focus on pathomorphological evaluation,biological aggressiveness and prognostic factors.To our knowledge this is the first report of giant GIST of very uncommon sarcomatoid epithelioid subtype.It is concluded that clinicians should pay attention to the fact that initial diagnosis may be delayed due to mildly asymptomatic and non-specific clinical presentation.Asymptomatic tumours diagnosed at a late stage,which is often the case,can be large on presentation.Prognosis for patients diagnosed with GIST depend on tumour size,mitotic rate,histopathologic subtype and tumour location.That is why early diagnosis and R0 resection,which is usually feasible and safe even in giant gastric sarcomatoid epithelioid subtype of GISTs,are the key factors for further treatment and good prognosis.     

Melanoma of the rectum： A rare entity

A 41-year-old man presented with a 6-mo history of changed defecation and rectal bleeding. A 3-cm polypoid tumor of the lower rectum was found at rectosigmoidoscopy, which proved to be a leiomyosarcoma upon biopsy. Dissemination studies did not show any metastases. He was underwent to an abdomino-perineal resection （APR）. Histopathology of the specimen showed a melanoma （S-100 stain positive）. Two years after the resection, metastases in the abdomen and right lung were found. He died one and half years later. Primary anorectal melanoma is a rare and very aggressive disorder. According to current data, one should always perform a S-100 stain when anorectal sarcoma is suspected. A positive S-100 stain suggests the tumour to be most likely a melanoma. Subsequently, thorough dissemination studies need to be performed. Depending on the outcome of the dissemination studies, a surgical resection has to be performed. Nowadays, a sphincter-saving local excision combined with adjuvant loco-regional radiotherapy should be preferred in case of small tumors. The same loco-regional control is achieved with less ＂loss of function＂ compared to non-sphincter saving surgery. Only in the case of large and obstructing tumors an abdomino-perineal resection is the treatment of choice.     

Neoadjuvant radiotherapy for rectal cancer management

Thirty per cent of all colorectal tumours develop in the rectum.The location of the rectum within the bony pelvis and its proximity to vital structures presents significant therapeutic challenges when considering neoadjuvant options and surgical interventions.Most patients with early rectal cancer can be adequately managed by surgery alone.However,a significant proportion of patients with rectal cancer present with locally advanced disease and will potentially benefit from down staging prior to surgery.Neoadjuvant therapy involves a variety of options including radiotherapy,chemotherapy used alone or in combination.Neoadjuvant radiotherapy in rectal cancer has been shown to be effective in reducing tumour burden in advance of curative surgery.The gold standard surgical rectal cancer management aims to achieve surgical removal of the tumour and all draining lymph nodes,within an intact mesorectal package,in order to minimise local recurrence.It is critically important that all rectal cancer cases are discussed at a multidisciplinary meeting represented by all relevant specialties.Pre-operative staging including CT thorax,abdomen,pelvis to assess for distal disease and magnetic resonance imaging to assess local involvement is essential.Staging radiology and MDT discussion are integral in identifying patients who require neoadjuvant radiotherapy.While Neoadjuvant radiotherapy is potentially beneficial it may also result in morbidity and thus should be reserved for those patients who are at a high risk of local failure,which includes patients with nodal involvement,extramural venous invasion and threatened circumferential margin.The aim of this review is to discuss the role of neoadjuvant radiotherapy in the management of rectal cancer.     

Simultaneous laparoscopy-assisted low anterior resection and distal gastrectomy for synchronous carcinoma of rectum and stomach

Laparoscopic resection of rectal cancer or gastric cancer has been advocated for the benefits of a reduced morbidity,a shorter treatment time,and similar outcomes.However,simultaneous laparoscopy-assisted low anterior resection and distal gastrectomy for synchronous carcinoma of rectum and stomach are rarely documented in literature.Endoscopic examination revealed a synchronous carcinoma of rectum and stomach in a 55-year-old male patient with rectal bleeding and epigastric discomfort.He underwent a simultaneous laparoscopy-assisted low anterior resection and distal gastrectomy with regional lymph nodes dissected.The operation time was 270 min and the estimated blood loss was 120 mL.The patient required parenteral analgesia for less than 24 h.Flatus was passed on postoperative day 3,and a solid diet was resumed on postoperative day 7.He was discharged on postoperative day 13.With the advances in laparoscopic technology and experience,simultaneous resection is an attractive alternative to a synchronous gastrointestinal cancer.     

Functional activity of the rectum： A conduit organ or a storage organ or both？

AIM: To investigate whether the degree of rectal distension could define the rectum functions as a conduit or reservoir. METHODS: Response of the rectal and anal pressure to 2 types of rectal balloon distension, rapid voluminous and slow gradual distention, was recorded in 21 healthy volunteers (12 men, 9 women, age 41.7±10.6 years). The test was repeated with sphincteric squeeze on urgent sensation. RESULTS: Rapid voluminous rectal distension resulted in a significant rectal pressure increase (P < 0.001), an anal pressure decline (P < 0.05) and balloon expulsion. The subjects felt urgent sensation but did not feel the 1st rectal sensation. On urgent sensation, anal squeeze caused a significant rectal pressure decrease (P < 0.001) and urgency disappearance. Slow incremental rectal filling drew a rectometrogram with a "tone" limb representing a gradual rectal pressure increase during rectal filling, and an "evacuation limb" representing a sharp pressure increase during balloon expulsion. The curve recorded both the 1st rectal sensation and the urgent sensation. CONCLUSION: The rectum has apparently two functions: transportation (conduit) and storage, both depending on the degree of rectal filling. If the fecal material received by the rectum is small, it is stored in the rectum until a big volume is reached that can affect a degree of rectal distension sufficient to initiate the defecation reflex. Large volume rectal distension evokes directly the rectoanal inhibitory reflex with a resulting defecation.     

Neutron-induced apoptosis of HR8348 cells in vitro

INTRODUCTIONTo date,the major therapy for rectal carcinoma isextensive abdomino-perineal resection.Unfortunately,after resection of rectal carcinoma,many patients still die of blood-borne metastases,usually in the liver or lungs,or local pelvicrecurrence,which is the major cause ofmorbidity and mortality in patients with rectalcarcinoma.Pre-or postoperative radiotherapy canreduce the incidence of local recurrence,buteven with moderately high radiation doses,manypatients are not locally controlled and have distantmetastases.The reason for this may be low     

Endoscopic submucosal dissection for colorectal neoplasms: A review

The introduction of colorectal endoscopic submucosal dissection(ESD)has expanded the application of endoscopic treatment,which can be used for lesions with a low metastatic potential regardless of their size.ESD has the advantage of achieving en bloc resection with a lower local recurrence rate compared with that of piecemeal endoscopic mucosal resection.Moreover,in the past,surgery was indicated in patients with large lesions spreading to almost the entire circumference of the rectum,regardless of the depth of invasion,as endoscopic resection of these lesions was technically difficult.Therefore,a prime benefit of ESD is significant improvement in the quality of life for patients who have large rectal lesions.On the other hand,ESD is not as widely applied in the treatment of colorectal neoplasms as it is in gastric cancers owing to the associated technical difficulty,longer procedural duration,and increased risk of perforation.To diversify the available endoscopic treatment strategies for superficial colorectal neoplasms,endoscopists performing ESD need torecognize its indications,the technical issues involved in its application,and the associated complications.This review outlines the methods and type of devices used for colorectal ESD,and the training required by endoscopists to perform this procedure.     

Presacral ganglioneuroma： A case report and review of literature

Presacral ganglioneuromas are so rare benign tumors that only 17 cases have been reported in the literature. They are abdominal masses growing slowly and differential diagnoses have to be considered. Surgical resection is important for definitive diagnosis because it represents the only therapeutic choice. Because of the benign nature of ganglioneuroma, adjuvant chemoor radiotherapy is not indicated but regular follow-up is necessary for an early diagnosis of potential local recurrence. We report a case of a 64-year-old man with a presacral ganglioneuroma.     

直肠胃肠间质瘤的临床病理特征和治疗

直肠是胃肠间质瘤的第三常见部位，其临床病理学特征、生物学行为和解剖部位使其诊治具有特殊性。好发于直肠中下段且术后局部复发率偏高，都是直肠GIST治疗的难点。伊马替尼的围手术期治疗增加了手术切除率并降低了复发率，5 cm以下的直肠GIST靶向治疗联合手术切除治疗效果较好。目前，国内外尚缺少直肠GIST的诊疗规范，未来还需要更多的前瞻性多中心研究进一步探讨并得出结论。     

Gastrointestinal stromal tumour of the rectum

Gastrointestinal stromal tumour (GIST) has been immunohistochemically defined as the tumour lacking differentiation towards either leiomyomatous tumour or schwannoma. We report a 75-year-old man who underwent an abdominoperineal resection of a large submucosal tumour of the rectum. The excised specimen was revealed to be an elastic soft tumour, 8 x 7 x 6 cm in size, which histologically consisted of spindle-shaped cells without nuclear atypia. The mitotic count was fewer than 2 per 10 high-power fields. The tumour cells were positive for staining of CD34 and c-kit protein, while the lesions were negative for alpha-smooth muscle actin, HHF-35, neuron-specific enolase, and S-100 protein. The diagnosis of GIST was confirmed by immunohistochemical examination of the tumour. From these findings, the present case is thought to be potentially malignant, and a long-term follow-up observation is needed for the case.     

Rectal GIST Presenting as a Submucosal Calculus

This case report presents an incidental finding of a rectal GIST (gastrointestinal stromal tumor) presenting as a submucosal calculus, not previously reported. A 53-year-old man without a significant medical history presented with abdominal pain in the left lower quadrant, and with constipation. Upon rectal examination, a hard submucosal swelling was palpated 4 cm from the anus, at 3 o'clock, in the left rectum wall. X-ray photos, computerized tomography (CT)-scan and a magnetic resonance imaging (MRI) scan clearly showed a calculus. Excision revealed a turnip-like lesion, 3.1 x 2.3 x 1.8 cm. Analysis showed it was a rectal GIST, a rare mesenchymal tumor of the gastrointestinal tract, which expressed CD117 (or c-kit, a marker of kit-receptor tyrosine kinase) and CD34. Calcification is not a usual clinicopathological feature of GISTs [1-3], and although a number of rectal GISTs have been reported [4-9], we have found no cases so far of rectal GIST presenting as a submucosal calculus.In general, GISTs are rare mesenchymal tumors of the gastrointestinal tract (nerve tissue, smooth muscle). Histology and immunohistochemistry discriminate gastrointestinal stromal tumors from leiomyomas and neurinomas. The most important location is the stomach; the rectal location is rare. Usually, the classic signs of malignancy such as cellular invasion and metastasis are missing. A set of histologic criteria stratifies GIST for risk of malignant behavior such as mitotic activity and tumor size, cellular pleomorphism, developmental stage of the cell and quantity of cytoplasma [7,13]. Tumors with a high mitotic activity and size above 5 cm are considered malignant. Recent pharmacological advances such as tyrosine kinase inhibitors have determined c-kit (i.e., CD117) as the most important marker, amongst others. C-kit positive tumors respond extremely well to chemotherapy with Imatinib (Glivec, Gleevec) [10-12].     

Clinical Study on Gastrointestinal Stromal Tumors (GIST) in Iceland, 1990–2003

This is a whole population-based study on clinical symptoms, surgical treatment, and outcome of GIST. All mesenchymal tumors in the digestive tract diagnosed from 1990 to 2003 were identified. All reports were reviewed, all tumors were stained with antibodies to c-kit, and the diagnosis of GIST was confirmed. Clinical, pathological, treatment, and outcome data were analyzed. The study included 53 patients with GIST. The mean age at diagnosis was 65.8±13.6 years (SD). Tumor distribution included 62% in the upper, 32% in the middle, and 6% in the lower digestive tract. Mean tumor size was 4.9±4.4 cm (SD). Gastrointestinal (GI) bleeding was the main symptom in 53% (20/38) of symptomatic cases; most presented with acute gastrointestinal bleeding. Complete surgical resection was performed in 87% (46/53) of patients. Eight of the 53 tumors (15.1%) metastasized, 7 of which were nongastric. The disease-specific death rate at 5 years was 85%, and 5-year survival after complete resection was 64.1%. We conclude that GISTs are often found incidentally but GI bleeding is the most common presentation. Five-year survival is better than previously reported and gastric GIST seems to be more benign than nongastric. GIST seems to metastasize mainly intra-abdominally. These authors contributed equally to the study.     

Laparoscopic sphincter-preserving surgery (intersphincteric resection) after neoadjuvant imatinib treatment for gastrointestinal stromal tumor (GIST) of the rectum

Background

Gastrointestinal stromal tumors (GISTs) of the rectum are rarely found, and radical surgery such as abdominoperineal resection would be necessary for large rectal GIST. On the other hand, therapy for GIST has changed significantly with the use of imatinib. Neoadjuvant imatinib therapy may reduce tumor size and may potentially prevent extended surgery. Moreover, when sphincter-preserving surgery is carried out laparoscopically, it can be performed as minimally invasive surgery with preservation of the anus.

Methods

From 2008 to 2011, five patients with rectal GIST were treated in our hospital. All patients received preoperative imatinib treatment (400 mg/day) and underwent laparoscopic sphincter-preserving surgery after 4–12 months of this treatment.

Results

Initial median tumor size was 31 mm (range, 24–88). At the time of operation, the median tumor size was 24 mm (range, 11–52). Sphincter-preserving surgery was performed in all patients. Three patients underwent laparoscopic intersphincteric resection (ISR), and two patients underwent transanal full-thickness local resection and recto-anal anastomosis following laparoscopic ISR. Macroscopically complete resection was achieved, and microscopically, the resection margin was not involved of residual tumors. The median duration of postoperative hospital stay was 16 days (range, 13–30). No recurrence occurred in all patients during 1 to 4 years.

Conclusions

The present study suggests that neoadjuvant imatinib therapy might be effective to prevent extended surgery for rectal GIST, and laparoscopic sphincter-preserving surgery is safe and technically feasible. We recommend a combination of neoadjuvant imatinib therapy and laparoscopic ISR for locally advanced rectal GIST.     

36例胃肠道间质瘤的临床诊治

目的 总结胃肠道间质瘤（GIST）的临床诊疗经验和病理学特征.方法 回顾性分析1995年1月至2005年4月收治的36例GIST的临床和病理资料.结果 最常见临床表现为腹部隐痛不适19例,消化道出血12例,腹部包块7例.最常见病变部位为胃26例,小肠8例.病程1天～2年.肿块平均直径7.2 cm.CD117阳性29例,CD34阳性30例.总的随访率为31/36（86.1%）,11例死亡.结论 GIST术前确诊较困难,确诊主要依靠病理学观察和免疫组化检测,联合CD117和CD34检测可提高GIST诊断.手术完整切除肿瘤是治疗GIST的关键.     

Successful resection of a giant recurrent gastrointestinal stromal tumour with imatinib mesylate as neoadjuvant therapy

Gastrointestinal stromal tumours (GISTs) are the most common mesenchymal tumours of the GI tract. Although a great deal of experience has been obtained with imatinib mesylate in patients with unresectable or metastatic GISTs, its role in the neoadjuvant setting is not well established. In this paper, we describe a case of successful resection of a giant recurrent GIST with imatinib mesylate as neoadjuvant therapy.     

Imatinib mesylate neoadjuvant treatment for rectal malignant gastrointestinal stromal tumor

Surgical treatments including radical resection and local excision remain the main treatment for primary rectal gastrointestinal stromal tumors （GISTs）. However, since patients with high-grade rectal GISTs have a higher risk of tumor recurrence and a shorter life expectancy, neoadjuvant treatment is necessary. In this case report, the efficacy of imatinib mesylate （IM） as a neoadjuvant therapy was assessed in an old man with malignant rectal GIST. The patient received IM preoperative treatment for a short period of one and a half months; at the end of the IM treatment, computed tomography scanning showed a markedly reduced tumor size and cystic changes of the tissue. At that time, a function sphincter-sparing surgery was performed. The histological examination of the resected specimen detected no tumor cells, but residual blood vessels and scattered inflammatory lymphocytes. After surgery, the patient has been followed up without additional IM treatment and remained disease-free for 57 mo. This case indicates that IM neoadjuvant therapy can dramatically improve the prognosis of rectal malignant GIST.     

Neoadjuvant Imatinib in Gastrointestinal Stromal Tumor of the Rectum: Report of a Case

Gastrointestinal stromal tumors are rare tumors of the gastrointestinal tract. Gastrointestinal stromal tumors involving the rectum are uncommon. We describe a case of a 43-year-old female with a gastrointestinal stromal tumor of the rectum who declined abdominoperineal resection. Neoadjuvant treatment with imatinib decreased her tumor size, permitting sphincter-sparing transanal excision. She had no evidence of disease for 24 months postoperatively until she recurred with lung metastases. Microdissection genotyping of the recurrent lesion revealed a deletion in exon 11. Further mutational analysis showed that her metastatic lesion was concordant with her primary rectal lesion, suggesting that systemic micrometastasis was previously present at initial diagnosis. Deletion in exon 11 predicts for response with imatinib treatment and is associated with a longer event-free and overall survival. Current studies are underway that may help us optimize the treatment for patients with gastrointestinl stromal tumors.Reprints are not available.