Extragastrointestinal Stromal Tumor (EGIST) in the abdominal wall: Case report and literature review

INTRODUCTION

Gastro Intestinal Stromal Tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract (GI). GIST that arises primarily outside the GI tract is termed Extragastrointestinal Stromal Tumor (EGIST). To the best of our knowledge, few cases of EGIST in the abdominal wall were reported.

PRESENTATION OF CASE

We present a rare case of EGIST in the abdominal wall of a 57 year-old female patient. The asymptomatic tumor was located in the superior aspect of the left rectus abdominis muscle, measured 5.4 × cm 5.3 × cm 6.9 cm and was well circumscribed. Histological examination showed an epithelioid cell morphology. The mitotic count was 7/50 HPFs. Immunohistochemistry showed diffuse strong CD117 positivity, focal positivity for S100. The tumor was excised and the margins were free of malignancy. The patient was doing well postoperatively and was discharged on STI-571 regimen.

DISCUSSION

Although GIST is the most common mesenchymal tumor of the gastrointestinal tract, a case with EGIST in the abdominal wall is rare. Positive immunohistochemical staining for CD117 is a defining feature of GISTs. A great percentage of EGISTs represent a metastasis from a primary GIST. In our case, the clinical and diagnostic work-up have been proved it to be an EGIST.

CONCLUSION

The existing data on EGIST is insufficient to make a final conclusion regarding the malignant potential and clinicopathological factors of EGISTs that determine patient prognosis. Thus a follow-up for a long period is required. EGISTs should be kept in mind in the differential diagnosis for patients presenting with solid mass of the abdominal wall.     

Laparoscopic Management of Obstructing Small Bowel GIST Tumor

Background:

Gastrointestinal stromal (GIST) tumors make < 1% of all gastrointestinal neoplasms and 20% of small bowel neoplasms. The most common acute presenting symptom of these tumors is gastrointestinal hemorrhage with obstruction being rare. We discuss our laparoscopic approach to 2 patients with small bowel GIST tumors that presented to our institution with obstruction of the small bowel.

Case Presentation:

Two patients presented to the emergency department with signs and symptoms of small bowel obstruction. On workup, each was found to have a solid lesion either within or adjacent to the small bowel at the point of obstruction and both were emergently taken to the operating room. The pathologic diagnosis of small bowel GIST tumor was the same in both cases, but the pathophysiologies of the obstructing tumors were different.

Results:

Both patients underwent laparoscopic surgery with successful resection of the lesions. The details and crucial points of the laparoscopic approach to these tumors are described with specific attention to its appropriateness and safety in treating GIST tumors. Attention to particular details of the manipulation and management of the bowel in the face of obstruction and removal of the lesions is described.

Conclusion:

The laparoscopic approach to GIST tumors of the small bowel, even in the face of emergent surgery, is a safe method.     

Large gastrointestinal stromal tumor and advanced adenocarcinoma in the rectum coexistent with an incidental prostate carcinoma: A case report

INTRODUCTION

Gastrointestinal stromal tumors (GISTs) are the leading mesenchymal neoplasia in the gastrointestinal tract, but GIST arising from the rectum is rare. When a secondary neoplasia coexists in the vicinity of a rectal GIST, more aggressive treatment strategies may be needed to cure the diseases.

PRESENTATION OF CASE

We herein describe a 76-year-old man with a large gastrointestinal stromal tumor along with an advanced adenocarcinoma in the rectum that coexisted with prostate carcinoma. Preoperative examination revealed an advanced adenocarcinoma of the upper rectum and a large pelvic mass suggestive of a GIST or a neuroendocrine tumor arising from the anterior wall of the lower rectum. To eradicate the tumor, total pelvic exenteration with ureterocutaneous fistula was carried out after obtaining written informed consent. Immuhistochemical studies revealed the concurrence of an advanced rectal cancer (T3, N1, M0) and a malignant GIST (c-kit-positive, CD34-positive, vimentin-positive, and CAM5.2-negative), and an incidental prostatic acinar adenocarcinoma. The patient was given adjuvant chemotherapy with imatinib and remains disease-free as of 12 months after surgery.

DISCUSSION

A PubMed search for the case of coexistence of GIST with two other malignancies revealed only four cases, making this very rare condition.

CONCLUSION

Radical surgery with perioperative adjuvant chemotherapy using tyrosine kinase inhibitors is the choice for treatment of large GISTs with a malignant potential. Our report suggests that aggressive surgical approach would be feasible, when a secondary tumor is present near the GIST.     

Adrenal Schwannoma

Background:

Adrenal schwannomas are very rare tumors that are difficult to diagnose preoperatively. We report the case of a left adrenal schwannoma incidentally discovered in a 55-year-old man during a postoperative checkup for a cutaneous malignant melanoma.

Methods:

The biological evaluation was unremarkable, and the radiological examination revealed the adrenal mass that was first considered a metastatic lesion. Adrenalectomy was performed by the laparoscopic approach.

Results:

The postoperative course was uneventful. Histological examination established the correct diagnosis of schwannoma, which was also confirmed by immunohistochemical staining.

Conclusions:

A nonsecreting adrenal mass can be easily misjudged, especially in the context of a recently operated on malignancy. Unilateral adrenal metastasis needs pathological confirmation, as it can dramatically affect prognosis. Unusual tumors of the adrenal gland may be found incidentally, and a malignant context will generate difficulties in establishing the right management. Complete laparoscopic excision is the treatment of choice whenever feasible and will also clarify pathology.     

FIRST BRAZILIAN CONSENSUS ON MULTIMODAL TREATMENT OF COLORECTAL LIVER METASTASES. MODULE 1: PRE-TREATMENT EVALUATION

Background

: Liver metastases of colorectal cancer are frequent and potentially fatal event in the evolution of patients with these tumors.

Aim

: In this module, was contextualized the clinical situations and parameterized epidemiological data and results of the various treatment modalities established.

Method:

Was realized deep discussion on detecting and staging metastatic colorectal cancer, as well as employment of imaging methods in the evaluation of response to instituted systemic therapy.

Results

: The next step was based on the definition of which patients would have their metastases considered resectable and how to expand the amount of patients elegible for modalities with curative intent.

Conclusion

: Were presented clinical, pathological and molecular prognostic factors, validated to be taken into account in clinical practice.     

Laparoscopic antral resection with Billroth I reconstruction for a gastric glomus tumor

INTRODUCTION

Gastric glomus tumors are fairly uncommon and mostly benign, with an estimated incidence of 1% of all GI soft tissue tumors. The most common GI site of involvement is the stomach, and in particular the antrum. Some cases have been discovered incidentally, but most are symptomatic presenting with GI bleeding, perforation or abdominal pain. Glomus tumors are submucosal tumors and hence mistaken with the more frequent gastrointestinal stromal tumors.

PRESENTATION OF CASE

A 33-year-old woman presented with intermittent dull upper abdominal pain for two days. Abdominal computed tomography (CT) was performed showing a hyperdense mass in the antrum. Endoscopy and endoscopic ultrasound revealed a submucosal antral mass along the greater curvature, suspicious for a gastrointestinal (GI) stromal tumor (GIST), a laparoscopic antrectomy with Billroth I reconstruction was done. Pathological examination revealed that the mass was a gastric glomus tumor.

DISCUSSION

The presented case report met all the usual standard criteria commonly used to identify glomus tumors, the uniqueness of the case lies in the occurrence of the glomus tumor in the stomach, first suspected as GIST, then confirmed as a gastric glomus tumor. The vast majority of glomus tumors of the GI tract have been described in the gastric antrum. They occur in adults of all ages with a significant female predominance (78%).

CONCLUSION

This case may aid in improving the recognition and diagnosis of this rare entity and in differentiating it from more common GISTs and gastric carcinoids. A built up knowledge between physicians is extremely necessary to avoid common confusion in taking the right medical approach.Abbreviations: CT, computed tomography; GI, gastrointestinal; GIST, gastrointestinal stromal tumor; EU, emergency unit; EUS, endoscopic ultrasound; SMA, smooth muscle actin; KIT, proto-oncogene c-Kit or tyrosine-protein kinase Kit or CD117; AFIP, Armed Forces Institute of Pathology; AUBMC, American University of Beirut Medical Center     

Small-bowel resection for metastatic melanoma

Objective

To determine whether complete resection of small-bowel metastases from melanoma improves patient survival.

Design

A computer-aided chart review.

Setting

Hospitals associated with McGill University.

Patients

Twenty patients (17 men, 3 women), identified from 1524 patients with melanoma, who underwent surgery to the small bowel for metastases. Patient age and clinical presentation, tumour site and stage were recorded.

Intervention

Exploratory laparotomy with complete or partial resection of involved small bowel.

Main Outcome Measures

Operative morbidity, mortality and length of survival related to the extent of small-bowel resection.

Results

Eleven patients had complete resection, 8 patients had partial resection and 1 patient had a palliative bypass only. Long-term survival (ranging from 2 to 10 years) was 36% in those who had complete resection and 0% in those who had partial resection; operative morbidity and mortality were 20% and 15% respectively.

Conclusion

Complete resection of small-bowel metastases in patients with metastatic melanoma can result in long-term survival.     

Expanding the Indications for Laparoscopic Gastric Resection for Gastrointestinal Stromal Tumors

Objective:

Laparoscopic resection of large gastric gastrointestinal stromal tumors (GIST) has been controversial. This generally has been limited to small lesions. We hypothesize that laparoscopic mobilization and resection using, in some cases, extracorporeal anastomosis of the gastrointestinal (GI) tract is an oncologically safe alternative to open surgery even when tumors are large.

Methods:

Four patients underwent a laparoscopic approach for gastric GIST tumors >2 cm at Methodist Dallas Medical Center over a 6-month period. Patient demographics, operative findings, postoperative course, and pathologic characteristics were examined.

Results:

The mean age in this patient group was 58 years (range, 36 to 77). Gastrointestinal bleeding and dyspepsia were the most common symptoms. Seventy-five percent of the patients were females. Mean tumor size was 10 cm (range, 2.5 to 20) with distribution in the stomach as follows: 75% greater curvature and 25% antrum. Tumors were removed by wedge, sleeve, and partial gastrectomies. Two of these tumors showed a high grade and the other 2 a moderate grade of differentiation. The number of mitoses was <5/50 HPF in all the tumors. No intraoperative spillage occurred in any patients, even with the largest tumor (20 cm). Importantly, all tumors were excised with a negative gross and microscopic margin. Average length of stay was 4 days. No patients required reoperation, and there were no complications postoperatively.

Conclusion:

Minimally invasive assisted approaches may be an option to treat large GIST tumors. Obeying principles of minimal touch, no spillage, and obtaining a negative margin, a safe operation with a laparoscopic approach is feasible, even in giant tumors. The large size of diagnosed GIST tumors should not preclude a minimally invasive approach.     

Peptide Receptor Radionuclide Therapy (PRRT) in a Patient Affected by Metastatic Breast Cancer with Neuroendocrine Differentiation

Background

Breast cancer (BC) is the most frequent cancer in European women with nearly 30% of the patients eventually developing metastases. Neuroendocrine differentiation is a rare event, but overexpression of somatostatin receptors in BC has been reported in many studies.

Case Report

A patient with liver metastases from BC was treated with peptide receptor radionuclide therapy (PRRT). Computed tomography scan and biochemical examinations showed a clear response to radionuclide therapy.

Conclusion

PRRT may be useful in metastatic BC patients.     

Is radiotherapy an effective treatment option for recurrent metastatic malignant melanoma? A case report of short-course,large-fraction radiation and a literature review

BACKGROUND

Malignant melanoma is regarded to be radiation resistant. A case of recurrent malignant melanoma with in-transit metastasis treated with short-course, high-fraction palliative radiation is presented to illustrate the effectiveness of radiotherapy.

METHOD

An 80-year-old woman initially treated surgically for a primary malignant melanoma of the left lower leg presented with multiple in-transit metastases. Palliative radiation was offered to treat two fungating in-transit masses that were resistant to treatments of isolated limb infusion and intralesional interleukin-2.

RESULTS

Treatment consisted of short-course, high-fraction radiation with 800 cGy fractions given over three weeks on days 0, 7 and 21, for a total dose of 2400 cGy. She experienced a complete response that was maintained for six months.

CONCLUSIONS

Radiation is an effective treatment option for palliation of recurrent malignant melanoma. Complete response is possible even with short-course, high-fraction radiation.     

Hypoglycemia with a large retroperitoneal mass – Case report

INTRODUCTION

Retroperitoneal sarcomas have rarely been reported to secrete insulin-growth factor II (IGF-II) and produce an enigmatic hypoglycemia. IGF-II-secreting sarcomas represent an extremely rare subset of soft tissue tumors, and reports are limited to a handful of cases.

PRESENTATION OF CASE

The authors present the case of hypoglycemia due to an IGF-II-secreting retroperitoneal sarcoma that was successfully treated by complete surgical resection. This report describes the diagnosis and management of this rare syndrome with 1-year follow-up and a review of the literature.

DISCUSSION

Steroid and growth hormone therapies also have efficacy to treat this hypoglycemia in some patients. However, outcomes appear better if combined with surgical resection.

CONCLUSIONS

The findings of this case report and review of the literature support a primary role for complete tumor resection to address tumor-induced hypoglycemia.Abbreviations: F, female; GH, recombinant human growth hormone; M, male; MO, months; NR, not reported; RP, Retroperitoneum; YR, years     

Neoadjuvant chemotherapy for primary adenocarcinomas of the urinary bladder: a single-site experience

Background

Adenocarcinoma of the urinary bladder is a rare malignancy. Radical surgery is suggested as the best available treatment for early-stage disease, but there is currently no consensus on standard chemotherapy regimen for advanced stage. We assessed the feasibility and effect of neoadjuvant chemotherapy with gemcitabine and cisplatin (GC) plus S-1 for patients with locally advanced primary adenocarcinomas of the urinary bladder.

Methods

Six patients with locally advanced urachal or non-urachal (n = 3, each) primary adenocarcinoma of the bladder were treated from October 2010 to October 2013 at a single center. All the patients were treated with 3 cycles (21d, each) of GC plus S-1 (gemcitabine, 1000 mg/m², days 1 and 8; cisplatin, 70 mg/m², day 2; and S-1, 50 mg bid, day 1-14). After neoadjuvant chemotherapy, patients with urachal cancer were treated with en bloc radical cystectomy and umbilectomy; the remaining 3 patients were treated with cystectomy.

Results

All patients successfully completed the neoadjuvant chemotherapy without serious side effects. Two patients were assessed as complete response, 2 as partial response, 1 as stable disease and 1 as progressive disease.

Conclusions

Despite the limitations of a small study population, the GC plus S-1 regimen for locally advanced primary adenocarcinoma of the urinary bladder was effective, and facilitated the success of surgery to a certain extent. Short follow-up time was also a limitation of our study. More studies are needed to evaluate the results.     

Laparoscopic Approach for Metachronous Cecal and Sigmoid Volvulus

Background:

Metachronous colonic volvulus is a rare event that has never been approached laparoscopically.

Methods:

Here we discuss the case of a 63-year-old female with a metachronous sigmoid and cecal volvulus.

Results:

The patient underwent 2 separate successful laparoscopic resections.

Discussion and Conclusion:

The following is a discussion of the case and the laparoscopic technique, accompanied by a brief review of colonic volvulus. In experienced hands, laparoscopy is a safe approach for acute colonic volvulus.     

Laparoscopic Resection of an Undifferentiated Pleomorphic Splenic Sarcoma

Background:

Splenic tumors are rare. Malignant fibrous histiocytoma (MFH) of the spleen is one of the least common primary splenic tumors. Review of the literature shows that a laparoscopic resection has never been tried.

Method:

We discuss the case of a 76-year-old man with a 7-cm MFH in the spleen and present a review of splenic sarcomas.

Results:

The patient underwent a successful laparoscopic splenectomy; pathology revealed a rare undifferentiated pleomorphic sarcoma of the spleen. A review of the international literature identified 15 additional cases of primary splenic MFH. Survival was rarely longer than 15 months.

Conclusion:

Malignant fibrous histiocytoma of the spleen is an exceedingly rare tumor with a poor prognosis. In experienced hands, laparoscopic splenectomy is a feasible operative choice for primary splenic sarcoma.     

Simultaneously diagnosed pulmonary thromboembolism and hemopericardium in a man with thoracic spinal cord injury

Background

Simultaneous pulmonary thromboembolism (PTE) and hemopericardium is a rare but life-threatening condition. As hemopericardium is a contraindication to anticoagulation treatment, it is challenging to handle both conditions together.

Objective

The objective of the study was to report a rare case of a man with thoracic spinal cord injury presenting with simultaneous PTE and hemopericardium.

Design

Case report.

Subject

A 43-year-old man with incomplete T9 paraplegia (American Spinal Injury Association Impairment Scale D) complained of fever one and a half months after spinal cord injury sustained in a fall.

Findings

During evaluation of fever origin, chest computed tomography and transthoracic echocardiogram revealed simultaneous PTE and hemopericardium. After serial echocardiograms over 2 days demonstrated stability, intravenous heparin, and oral warfarin were administered and his medical status was observed closely. Ultimately, both conditions improved without significant complications.

Conclusion

We report successful treatment of man with acute spinal cord injury who presented with simultaneously diagnosed PTE and hemopericardium, a rare complication involving two distinct and opposing pathological mechanisms and conflicting treatments.     

Profile of patients with gastrointestinal stromal tumors (GIST)

Background

There is an improvement on the GIST treatment in last decade due to biomolecular research and adjuvant therapy with tyrosine kinases inibitors. However, both modalities of treatment rarely are available in Brazilian public hospital.

Aim

Evaluate GIST patients profile in public oncologic hospital.

Methods

A retrospective study was made on patients with GIST diagnosed and treated between 2001 and 2013.

Results

Sixty-nine patients were included, mean age 59 years with slight predominance in females (51%). The main symptom was abdominal pain associated with incidental imaging finding. The occurrence of other associated neoplasm was in 28.8% of cases. The positivity of CD117 was 97.1%. The most frequent location was the stomach in 55.1% of cases. The R0 resection was possible in 63.8% and the recurrence rate was 20.3 %, with liver and peritoneum the main affected sites. Overall survival in the whole sample was 71%. Free survival rate of disease was 64%. The use of imatinib was limited to patients with residual disease (unresectable disease, R2 and R1 resection), metastatic disease or recurrence.

Conclusion

In order to improve GIST treatment is necessary to add the biomolecular analysis to risk stratification. However, for this to occur, incentive in biomolecular research is required, to increase the possibility of patient survival.     

Primary squamous cell carcinoma of breast with ipsilateral axillary lymph node metastasis: An unusual case

Introduction

Pure squamous cell carcinoma of the breast [SCCB] is rare.

Presentation of Case

We report a case of primary squamous cell carcinoma of breast with ipsilateral axillary lymph node metastasis in a 58year old woman.

Discussion

It is a breast carcinoma entirely composed of metaplastic squamous cells that may be keratinized, non-keratinized or spindled. The pure squamous cell carcinoma usually present with central cystic cavity, which we found in our case, also supported by immunohistochemical evidence.

Conclusion

Although a rare breast cancer subtype, SCCB is of considerable interest due to its pathological heterogeneity and differences in clinical behavior and less reported occurrence of nodal metastasis.     

Upper urinary tract disease: what we know today and unmet needs

Purpose

Upper tract urothelial carcinoma (UTUC) is a rare and poorly investigated disease. Intense collaborative efforts have increased our knowledge and improved the management of the disease. The objective of this review was to discuss recent advances and unmet needs in UTUC.

Methods

A non-systematic Medline/PubMed literature search was performed on UTUC using the terms “upper tract urothelial carcinoma” with different combinations of keywords. Original articles, reviews and editorials in English language were selected based on their clinical relevance.

Results

UTUC is a disease with specific epidemiologic and risk factors different to urothelial carcinoma of the bladder (UCB). Similarly to UCB, smoking increases the risk of UTUC and worsens its prognosis, whereas aristolochic acid (AA) exposure and mismatch repair genes abnormality are UTUC specific risk factors. A growing understanding of biological pathways involved in the tumorigenesis of UTUC has led to the identification of promising prognostic/predictive biomarkers. Risk stratification of UTUC is difficult due to limitations in staging and grading. Modern imaging and endoscopy have improved clinical decision-making, and allowed kidney-sparing management and surveillance in favorable-risk tumors. In high-risk tumors, radical nephroureterectomy (RNU) remains the standard. Complete removal of the intramural ureter is necessary with inferiority of endoscopic management. Post-RNU intravesical instillation has been shown to decrease bladder cancer recurrence rates. While the role of neoadjuvant cisplatin based combination chemotherapy and lymphadenectomy are not clearly established, the body of evidence suggests a survival benefit to these. There is currently no evidence for adjuvant chemotherapy (AC) in UTUC.

Conclusions

Despite growing interest and understanding of UTUC, its management remains challenging, requiring further high quality multicenter collaborations. Accurate risk estimation is necessary to avoid unnecessary RNUs while advances in technology are still required for optimal kidney-sparing approaches.     

Pandora's box and retrorectal tumors in laparoscopy: A case report and review of the literature

INTRODUCTION

Retrorectal tumors are uncommon and the etiology diverse. Literature to define the preoperative diagnosis and plan the intraoperative management are uncommon.

PRESENTATION OF CASE

We describe a case of a 44 year old patient with a laparoscopic approach for the removal of a retrorectal tumor and emphasize on the preoperative diagnostics and the intraoperative, minimal invasive approach.

DISCUSSION

Especially because these tumors are rare and often an incidental finding in gynecologic surgery, it is important to know the various differential diagnoses and its consequences with the laparoscopic approach.

CONCLUSION

We suggest the laparoscopic approach in cases of retroperitoneal cysts of unknown origin is ideal also because anatomic structures, mostly nerves, can be easily spared.     

Synchronous gist,colon and breast adenocarcinoma with double colonic polyp metastases

INTRODUCTION

Long term survivors of breast cancer are at risk of developing distant metastasis years after the initial treatment. We report a case of breast adenocarcinoma with colonic polyp metastases, as well as synchronous primary colonic adenocarcinoma and a gastric GIST.

PRESENTATION OF CASE

An 83 year old female underwent colonoscopy for rectal bleeding. This showed a primary colonic adenocarcinoma, a pedunculated polyp in the ascending colon and two polyps in the sigmoid colon. A staging CT scan did not show distant metastasis, but revealed a small gastric GIST which was managed conservatively. A right hemicolectomy showed a T3N0 colonic adenocarcinoma and a polyp contained metastatic adenocarcinoma from a breast primary. The patient had undergone surgery 30 years ago for an invasive lobular carcinoma. Further clinical assessment demonstrated an impalpable grade II Invasive ductal carcinoma in the contralateral breast. She was started on hormonal treatment and at 18 months follow-up, she was well with stable disease.

DISCUSSION

Invasive lobular cancer is the most common histological type of breast cancer that metastasizes to the colon. There is no consensus on the management of breast cancer metastasis to the gastrointestinal tract. Co-existence of a GIST and an adenocarcinoma at two separate locations is uncommon. These are two different cancer entities and it is unclear whether these two are related by as causal relationship.

CONCLUSION

This is a rare case of three distinct tumours; association between them is unlikely. However, the case highlights the importance of a multidisciplinary approach to cancer treatment.