Left Extended Hepatectomy for a Metastatic Gastrointestinal Stromal Tumor After a Disease-Free Interval of 17 Years: Report of a Case

Gastrointestinal stromal tumors (GISTs), although rare, are frequently diagnosed with liver metastasis. These metastatic GISTs are poorly responsive to conventional chemotherapy; however, recent studies report improved survival after complete surgical resection of liver metastases. On the other hand, few reports describe the treatment of delayed liver metastasis after resection of a primary GIST. We report the case of a 55-year-old woman found to have liver metastasis from a GIST after a 17-year disease-free interval. The patient underwent a left extended hepatectomy for a complete resection of the metastatic GIST and is alive and well 30 months later. To our knowledge, this is the longest disease-free interval reported in the literature, and emphasizes the importance of considering late metastasis when evaluating patients with a history of GIST. Thus, surgical resection of delayed liver metastasis from a GIST should be considered as primary therapy.     

Successful Resection of an Advanced Duodenal Gastrointestinal Stromal Tumor After Down-Staging with Imatinib: Report of a Case

The diagnosis of gastrointestinal stromal tumor (GIST) relies on a combination of the following criteria: anatomic location, typical histopathology, and the presence of CD 117-antigen (the tyrosine kinase receptor, c-kit) or CD 34-antigen. Imatinib mesylate, a specific tyrosine kinase inhibitor, is highly efficient against locally advanced or metastatic GIST. We report a case of unresectable duodenal GIST, which we were able to resect with curative intent after down-staging treatment with a dosage of imatinib 400 mg daily for 8 months. We performed Whipple's procedure combined with en bloc resection of the right kidney and adrenal gland. The patient was recurrence free at his 24-month follow-up examination. Down-staging treatment may be worthwhile in selected patients, but further prospective studies of imatinib in this setting are necessary. We think that imatinib should be continued postoperatively, as the risk of recurrence in these patients may be high.     

Case report of diffusely metastatic rectal GIST

This is a case report of an aggressive, diffusely disseminated Stage IV rectal gastrointestinal stromal tumor (GIST) in a 57-year-old male that presented for symptoms of malaise, constipation, and twenty pound weight loss in 2 months. Upon rectal examination, a hard 4 centimeter submucosal mass was found at the 9–12 o’clock position. Liver and lung metastases were visualized on computerized tomography (CT) of the chest, abdomen, and pelvis on metastatic work-up. He was deemed a poor surgical candidate due to diffuse metastatic disease and referred for palliative chemotherapy. The patient had suffered a perforation of his rectal wall two weeks after his initial presentation and passed away shortly thereafter. He never received palliative chemotherapy. We present a case report as a unique case of an extremely aggressive and quickly fatal GIST tumor.     

Hypoglycemia in the setting of advanced gastrointestinal stromal tumor

We report a rare case of symptomatic hypoglycemia in a patient with intra-abdominal recurrence of a previously resected gastrointestinal stromal tumor (GIST). The patient is a 65-year-old woman who underwent resection of a large abdominal mass arising from the stomach, histologically diagnosed as a high-grade leiomyosarcoma. She was lost to follow up. Five years later, the mass recurred; core biopsy demonstrated a CD 117-positive, spindle-cell tumor, consistent with a GIST. She was placed on Gleevec, as there was evidence of multifocal disease, but imaging revealed only mild improvement. Subsequently, her clinical status deteriorated, and she was hospitalized for dehydration, vomiting, and mental status changes. Her blood glucose on admission was 22 mg/dL, and a dextrose infusion (50%) was necessary to maintain adequate blood glucose levels. Measurements of insulin, proinsulin, c-peptide, beta-hydroxybutyrate, and thyroid-stimulating hormone were normal, as were cosyntropin stimulation and glucagon response tests. Suspicions arose for tumor-secreted insulin-like factor. She underwent resection of the dominant 44-cm recurrence, with immediate rebound hyperglycemia, followed by complete normalization of her blood glucose levels. She was discharged on postoperative Day 5 without symptoms or insulin, and is alive with disease at 20 months. Paraneoplastic syndromes occur in only 15 per cent of patients with known malignancies (e.g., lung cancer and metastatic carcinoid), and are rarely reported in the setting of GIST. Hypoglycemia is most often observed in presence of insulinoma and only isolated case reports in GIST patients exist. Overexpression of insulin-like growth factor II is thought to be the mechanism of action. Supportive management and palliative resection or debulking is recommended when possible.     

Extragastrointestinal stromal tumor (EGIST)--a case review

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract. Due to the presence of thyrosine kinase receptors within the tumor tissue, GIST is thought to originate from gastrointestinal pacemaker cells, the intersticial cells of Cajal. Tumors with the same morphological and imunohistochemical characteristics detected outside the gastrointestinal tract, are called extragastrointestinal stromal tumors (EGIST). Biological characteristics of these tumors is uncertain and the malignancy rates are difficult to predict. Surgical R0 resection in resecable tumors is the only option with the potential for complete cure. Nevertheless, the recurrence rates are high. Adjuvant biological treatment with imatinib, a thyrosine kinase inhibitor, reduces the risk of relapses. Imatinib administration is also the principal treatment method in metastatic GIST disorders. The article offers a short and complex overview of gastrointestinal stromal tumor (GIST) problematics and presents a case report of a patient suffering from EGIST of mesocolon transversum treated by R0 resection which was performed under multidisciplinary cooperation, with a specialist follow up.     

Gastrointestinaler Stromatumor der Bauchwand

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract. We describe here for the first time a patient with a huge GIST of the abdominal wall without any relation to the gastrointestinal tract, omentum, or mesentery. With regard to the size of 24 cm and a low mitotic index, this GIST is considered an intermediate risk for metastasis. Radical surgical resection was performed with negative pathologic resection margins. The classic immunohistochemical phenotype of the tumor described facilitates the differential diagnosis to exclude abdominal desmoid tumor and solitary fibrous tumor (SFT). In the case of metastasis, therapeutic nihilism no longer seems justified with the availability of imatinib, a tyrosine kinase inhibitor, which shows encouraging results in the therapy of metastatic GIST.     

Jejunal GIST masquerading as an ovarian mass: A case report

Introduction and importanceGastrointestinal stromal tumors (GIST) are rare mesenchymal tumors originating in the wall of the gastrointestinal tract. Jejunal GIST is the rarest subtype. Large GIST can present with an abdominopelvic mass which can be preoperatively misdiagnosed as a gynecological tumor.Case historyA 44-year regularly menstruating woman presented with lower abdominal pain which was diagnosed as a malignant ovarian tumor preoperatively with an MRI. However, intraoperatively, a lobulated mass was present in the abdominal cavity arising from a jejunal portion of the small intestine. With an intraoperative diagnosis of jejunal GIST, the mass was excised and jejunum anastomosed. Histopathology examination report showed GIST which was further confirmed by immunohistochemistry.DiscussionGIST presenting as a large abdominopelvic mass can mimic a gynecological tumor. Contrast-enhanced CT scan is the preferred imaging modality for the evaluation of patients with suspected GIST to determine the extent of the tumor, the presence or absence of metastatic disease alongside evaluation of the possibility of complete resection. Adjuvant imatinib therapy following complete excision can decrease the disease recurrence.ConclusionGynecologists should keep in mind that primary gastrointestinal tumors can present as a pelvic mass. A proper histopathological examination helps to confirm the diagnosis. Complete surgical removal of the tumor should be obtained as it determines the prognosis of the disease.     

Laparoscopic approach of gastric gastrointestinal stromal tumors (GISTs): is it still a courageous choice? Report of two cases

Gastrointestinal stromal tumors (GISTs) are a well-defined clinicopathologic and molecular tumor entity, representing the most common gastrointestinal mesenchymal neoplasm. Differential diagnosis between GIST and other mesenchymal malignancies is crucial, given the successful management using targeted therapy in metastatic GIST. The mainstay of treatment remains surgery, complete tumor resection being the most important independent prognostic factor. Videolaparoscopic approach is still controversial for the high risk of tumor rupture or bleeding. Here we report 2 cases of GIST surgically resected using a videolaparoscopic approach and discuss the efficacy of this technique in selected patients.     

Penile and urethral metastases from superficial bladder tumor after TUR: a case report

A case of metastasis to the penis and the urethra from superficial bladder tumor of transitional cell carcinoma (TCC), grade 3 is reported. A 52-year-old male patient was diagnosed to have TCC of the urinary bladder (grade 3, stage pT1a) in May, 1985 and was treated initially with transurethral resection followed by adriamycin (ADR) instillation. In February, 1986, urethroscopy showed a papillary tumor in the cavernosal urethra and a metastatic tumor was noted in the corpus spongiosum penis. Biopsy of urethral tumor revealed TCC, grade 3. Therefore partial urethrectomy with resection of penile tumor was performed. Although the patient underwent combination chemotherapy involving CAP (cisplatin + ADR + cyclophosphamide) and M-VAC (methotrexate + vincristine + ADR + cisplatin) regimens, local lesion and metastatic lesions progressed, and he died in June 1986, 20 days after emasculation. The management of superficial bladder tumor with TCC, grade 3 was reviewed and discussed here.     

Perisacral gastrointestinal stromal tumor with intracranial metastasis. Case report

A 68-year-old woman presented with an extremely rare intracranial metastasis from a gastrointestinal stromal tumor (GIST) manifesting as left hemiparesis 2 years after resection of a sacral tumor adjacent to the coccygeal bone. Magnetic resonance imaging revealed an intracranial tumor in the right parietal lobe. Craniotomy was performed to completely remove the tumor. Although the tumor was located extra-axially, only internal carotid angiography showed mass staining. Seven months after surgery, the tumor recurred. Repeat craniotomy was performed to remove the recurrent tumor. Immunohistochemical analysis showed that the tumor cells were positive for c-kit and CD34, and the tumors were identified as intracranial metastasis of GIST. Following the second intracranial surgery, the patient developed severe lower back pain caused by metastatic tumor invading the lumbar spine and ureter. To avoid surgical complications and to reduce tumor volume, imatinib mesylate (Gleevec) was administered. The severe pain was relieved, although the tumor was not reduced. In this case, the extra-axial tumor was fed only by the internal carotid artery.     

Cecal gastrointestinal stromal tumor causing ileocolic intussusception in an adult: A rare case report

Introduction and importanceCecal gastrointestinal stromal tumors (GIST) constitute a rarer subtype of all GISTs. Rarely, it can present with ileocolic intussusception in adults making it a challenging diagnosis due to non-specific clinical features.Case presentationA 30-year previously healthy woman presented with lower abdominal pain and a distended abdomen who was subsequently diagnosed with ileocolic intussusception on a CT scan. Intraoperatively, a pedunculated polypoid hard mass was identified in the cecum and thus, a standard right hemicolectomy was performed with the suspicion of malignancy. Histopathology of the resected mass confirmed CD117 negative, spindle type GIST.DiscussionCecal GIST presenting in the form of ileocolic intussusception is rare. Contrast-enhanced CT scan is the preferred imaging modality for the evaluation of patients with suspected GIST to determine the extent of the tumor, the presence or absence of metastatic disease alongside evaluation of the possibility of complete resection. Adjuvant imatinib therapy following complete resection decreases the disease recurrence.ConclusionIntussusception in an adult can be the first manifestation of underlying malignancy like GIST. Complete surgical resection of the tumor with a negative margin offers long-term survival.     

Gastrointestinaler Stromatumor (GIST) der vorderen Rektumwand

This case report deals with a 79-year-old patient with a gastrointestinal stromal tumor (GIST) of the anterior rectal wall which was unusually located between the rectum and the prostate gland. In addition, this patient suffered from subvesical obstruction accompanied by an elevated PSA level. These circumstances led to our decision to operate on the tumor via simultaneous radical retropubic prostatectomy. In our opinion this resection technique was easier and less traumatic for the patient compared to procedures performed via the abdomen and perineum. This case report demonstrates that in the case of tumors located between the rectum and the prostate gland the differential diagnosis should include not only prostate carcinoma but also rare tumor entities such as GIST.     

Outcome of Metastatic GIST in the Era before Tyrosine Kinase Inhibitors

Background Treatment of metastatic GIST with imatinib mesylate results in a 2-year survival of approximately 72%. The outcome of patients with metastatic GIST not treated with tyrosine kinase inhibitors is not well defined. Methods One hundred nineteen patients with metastatic GIST diagnosed prior to July 1, 1998 (approximately 2 years prior to the use of imatinib for GIST) were identified from an institutional database of patients with pathologically confirmed GIST. Mutational analysis was performed in cases with available tissue. The log rank test and Cox regression models were used to assess prognostic factors. Results Median survival was 19 months with a 41% 2-year survival and a 25% 5-year survival. Resection of metastatic GIST was performed in 81 patients (68%), while 50 (42%) received conventional chemotherapy. Twelve patients (10%) were eventually started on imatinib. Primary tumor size <10 cm, <5 mitoses/50 HPF in the primary tumor, epithelioid morphology, longer disease-free interval, and surgical resection were independent predictors of improved survival on multivariate analysis. Mutational status did not predict outcome. In patients who underwent resection, the 2 year survival was 53%, and negative microscopic margins also independently predicted improved survival. Conclusions Treatment with imatinib appears to improve 2-year survival of metastatic GIST by approximately 20% when compared to surgery alone. The combination of imatinib and surgery for the treatment of metastatic GIST therefore warrants investigation. An erratum to this article can be found at     

Renal cell carcinoma involving penis and testis: unusual initial presentations of metastatic disease

Solitary parenchymal metastases of renal cell carcinoma to the penis or testis are rare. We report 1 case of each: in one instance the patient presented with priapism and an asymptomatic primary tumor; in the other, the metastasis presented as an acute change in a long-standing hydrocele more than one year after nephrectomy. Both patients had either positive margins at resection or recurrence after resection of the metastasis. While long-term, disease-free survival has been reported after orchiectomy for isolated metastatic disease, penile involvement appears to carry a very poor prognosis.     

Angiolymphoid hyperplasia with eosinophilia on penile skin in a 7-year-old child

We report a case of a 7-year-old child who presented with a painless mass on his penis. He underwent removal of the mass, which was found to be an angiolymphoid hyperplasia with eosinophilia. Angiolymphoid hyperplasia with eosinophilia is an extremely rare tumor, especially in children's penis. In this situation, treatment of this tumor should be considered carefully, and there is a chance of spontaneous regression after volume reduction surgery.     

Penile metastasis from esophageal squamous carcinoma after curative resection

The penis is an uncommon site of metastasis; with only about 300 cases reported in literature. The majority (75%) of primary tumors are located in the pelvis, and they arise from the genitourinary tract and rectum. We report on a patient with esophageal squamous carcinoma who underwent a curative resection and later developed metastatic nodules over the penis and perineum. We believe this is the first instance of this unusual presentation. He was offered palliation with weekly taxanes and had symptomatic relief with this regimen.     

Penile malignant melanoma in a hemodialysis patient

Abstract:   A patient who was receiving hemodialysis treatment developed melanotic macules on the surface of the penis in 2002 and showed a tumor-like mass in the same region in July 2006. The patient presented with a pedunculated tumor of 3 cm in diameter on the right side of his penis. The tumor was resected for biopsy and was diagnosed as malignant melanoma. The melanoma was in stage IIIB with pT4 N1 M0. The patient received interferon-β for a total of three courses. A computed tomography scan in the 10th postoperative month did not find any additional metastatic foci or recurrence of the tumor. In the present case, side effects caused by interferon were not observed. Therefore, particularly in dialysis patients, immune therapy might be favored over anticancer drug treatment.     

Laparoscopic resection for a large gastrointestinal stromal tumor (GIST) with diaphragm invasion following preoperative imatinib treatment: A case report

IntroductionNeoadjuvant imatinib for large GISTs may prevent tumor rupture and the need for extended surgery by reducing tumor size. In this study, we present a case of large gastric GIST with diaphragm invasion, due to the patient receiving laparoscopic resection following preoperative imatinib treatment.Presentation of caseA 72-year-old woman was hospitalized with left hypochondriac pain for a month. Examinations revealed a large heterogeneous gastric mass measuring 80 mm in size, arising from the greater curvature of the corpus. The mass invaded the left thoracic diaphragm. Treatment with imatinib at an initial dosage of 400 mg/day was initiated. After a further two months of follow-up, the lesion had sustained reduction to 50 mm in size, however, the invasion to the diaphragm remained. The patient eventually underwent laparoscopic partial gastrectomy and partial resection of the diaphragm with curative intent. Adjuvant chemotherapy was initiated at one month after the surgery, however, was discontinued due to nausea. After one-year follow-up, no recurrence was noted.DiscussionNeoadjuvant imatinib may shrink tumor size remarkably and prevent tumor rupture during surgery, and thus lead to increased rates of complete resection. To date, several publications have directly compared the oncologic results between laparoscopic and open resection for GISTs. In the present case, the tumor was movable, and moderately fixed on diaphragm. It was favorable condition for laparoscopic surgery.ConclusionsThis is the first report of a large gastric GIST invading the diaphragm that was successfully treated by laparoscopic resection after tumor reduction by neoadjuvant imatinib.     

Successful resection of a gastrointestinal stromal tumor in the pelvis with imatinib mesylate as neoadjuvant therapy

We report a case of marginally resectable gastrointestinal stromal tumor (GIST) in the pelvis treated with neoadjuvant intent before subsequent successful surgical resection. A 46-year old man presented with urinary frequency and rectal discomfort with tenesmus. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a 12 cm diameter mass between the bladder and rectum and the margin of the tumor and prostate was unclear. No metastases were evident. Trans-rectal needle core biopsy confirmed c-kit positive GIST. Because of the locally advanced nature of the tumor,immediate surgical resection would have required total pelvic exenteration with eternal colostomy and urinary diversion. Therefore,the patient was treated with imatinib mesylate 400 mg daily in anticipation of adequate tumor size reduction to enable a more simplified surgical approach. After 3 months of imatinib therapy,MRI demonstrated a reduction in tumor size of 60%. Consequently,a complete surgical resection including the bladder,prostate and part of the sigmoid colon with temporary ileostomy and ileal conduit was performed. Pathological findings of the resected specimen showed widespread degeneration with cystic changes,necrosis, and hypocellularlity,as well as nodules of residual viable c-kit positive tumor cells. The patient has been treated with imatinib mesylate for 39 months following the operation without tumor recurrence.     

Duodenal GIST metastasized to skull and orbit managed by surgery: a case report

We report a 26-year-old man with 6 years, history of duodenal gastrointestinal stromal tumor (GIST) with liver, peritoneum and lung metastases. He presented with left eye ptosis, diplopia, left facial numbness and a left temporal fossa mass that was confirmed to be GIST with left skull and left orbit metastases. Craniectomy with cranioplasty, tumor excision and decompression were performed. There was an improvement of his visual symptoms and facial numbness. To our knowledge, this is one of the few reports of surgical management of GIST, metastasized to skull and orbit, with good symptomatic relief.