Primary Gastrointestinal Stromal Tumors

Y. Wiener¹, R. Gold¹, S. Zehavy², J. Sandbank², A. Halevy¹

¹Dept. of General Surgery and Dept. of Pathology, ²Assaf Harofeh Medical Center, Israel

Stromal tumors of the GI tract are rare. In the retrospective and prospective study we investigated the relationship between tumor symptomatology, tumor grade and prognostic factors. During the period May 1993 - September 1999, 11 female and 13 male patients with a mean age of 62 (range-29-81) years were operated for primary gastrointestinal stomal tumors (GIST) in our department. Observed signs and symptoms were: GI bleeding (65%), abdominal pain (45%), abdominal mass (15%) and weakness (5%). In 4 patients tumor was an incidental finding during investigation or operation for another tumor. Tumor location (in decreasing order) was: stomach (15), small bowel (SB, 6), esophagus (1), duodenum (1) and colon (1). Preoperative biopsy or FNA were diagnostic in less than 50% of the cases. Operative procedures included wedge resection (8 patients), resection of segment of bowel (10) and extended resection (6), of diaphragm, SB, colon, bladder, kidney and liver. The mean tumor size was 7.8 (range-0.9-22) cm. Four tumors were graded as benign, 8 of indeterminate malignant potential and 12 malignant.

Conclusion: The main presentation of GIST is acute GI bleeding. Endoscopy is most effective for studying proximal tumors, and CT should be used to identify distal GI tract tumors. Tumor size or malignancy were not necessarily predictive of GI bleeding. When invasive to adjacent organs is present, wide excision should be contemplated as long-term survival can be achieved.

אסנת ולפיש, שלמה ולפיש

היחידה למחלות הכרכשת והחלחולת, מרכז רפואי סורוקה, באר-שבע

הגישה הניתוחית לשאת רחיקנית של החלחולת (הממוקמת בשליש הרחיקני של החלחולת, במרחק של פחות מ- 5-6 ס"מ מפי-הטבעת), השתנתה באופן משמעותי לאורך השנים. במשך שנים רבות, כריתה רדיקלית בניתוח בטן כמו כריתה ביטנית-עכוזית (כב"ע) היתה הניתוח היחיד שנערך בחולים עם שאת בחלחולת הרחיקנית, שאינם מתאימים לכריתה קידמית נמוכה, הטיפול בכב"ע מאפשר הישרדות בשיעור גבוה מהמטופלים הלוקים בשאת מוקדית (ללא גרורות); אולם הוא כרוך בתחלואה רבה, גורם גם לאין-אונות ולעיתים לפגיעה בתיפקוד דרכי השתן. התמותה הסב-ניתוחית נעה בין 1%-5% ועולה בחולים עם סיכון ניתוחי גבוה. בנוסף, כב"ע מלווה בפיום קבוע של הכרכשת, דבר הגורם לנכות גופנית. נוכח כל זאת, גברה בשנים האחרונות ההתעניינות בניתוחים משמרי פי הטבעת בחולים עם שאת רחיקנית של החלחולת. מטרת הניתוחים האלה היא שימור איכות החיים של החולים, ללא פגיעה בסיכויי ריפויים מהשאת הממארת.

Pleomorphic Carcinoma of the Lung Heralded as Bilateral Leg Pain

George Habib, Hanna Rawashdi, Jonathan Peleg, Walid Saliba

Depts. of Medicine B, Neurology and Radiology, Lady Davis Medical Center, Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel

This is a case of a 70 year old male patient suffering from bilateral leg pain for 2 months. Physical examination disclosed clubbing. X-rays of the legs showed bilateral periosteal elevation with subperiosteal bone formation. ⁹⁹T^M-diphosphonate bone scan was negative. A search for malignancy revealed pleomorphic carcinoma of the right lung. Pain symptom disappeared 2 days after resection of the tumor. Repeated X-rays of the legs, three and a half months later showed no change and the clubbing persisted.

Hence, unexplained bilateral leg pain should raise suspicion of hypertrophic osteoarthropathy, and elicit a search for secondary disease especially lung tumor. It is interesting to point out the negative bone scan and the rapid resolution of patient symptoms after resection of the tumor.

Aggressive Surgical Approach in Pancreatic Cancer - Is it Justified?

V. Catz-Snir, P. Schachter, M. Shimonov, Y. Avni, G. Gvirtz, A. Czerniak

Depts. of Surgery, Gastroenterology, and Diagnostic Radiology, Wolfson Medical Center, Tel Aviv and Sackler Faculty of Medicine, Tel Aviv University

Resection presents the only possible cure for pancreatic cancer. An aggressive surgical approach may extend the limits of resection and offer cure for more patients. 37 women and 28 men, mean age 67 years (range 33-84) with focal lesions underwent pancreatic resection (1993-1999). Cancer of the pancreatic head was found in 45, in the ampulla in 4 and in the body or tail in 16. There was a tumor larger than 5 cm in 9, vascular involvement in 8, and a combination of both in 6.

All were evaluated prior to exploration by standard imaging techniques and 44 by laparoscopic ultrasound. 42 underwent pancreatico-duodenectomy, 16 distal subtotal pancreatectomy and 3 local excision of an ampullary tumor. Benign lesions were found in 8 (focal pancreatitis in 4 and a serous cystic lesion in 4).

Curative resection (microscopically negative margins, negative lymph nodes) was achieved in 44. 2 died 1-2 months after operation of septic complications (2% operative mortality). The most frequent complication (in 8) was pancreatic fistula. Additional complications included anastomotic-line bleeding (3), intra-abdominal abscess (2), and 1 developed portal vein thrombosis (treated surgically).

1- and 2-year survival in those after curative pancreatic resection was 81% and 55% respectively. 2-year survival in those after palliative resection (positive resection margins or positive lymph nodes) was 50% and 11%, respectively.

After proper selection of those in whom it is possible to perform curative resection, regardless of size or vascular involvement, an aggressive surgical approach is justified.
 

Metallic Stents for Acute Colonic Obstruction

M. Itkin, E. Atar, H. Neuman, D. Kravarosic, Z. Fuko, M. Kaz

Radiology and General Surgery B Depts., Golda Campus; and General Surgery Dept., Beilinson Campus; Rabin Medical Center, Petah Tikva

The traditional treatment of acute colonic obstruction, usually caused by malignant tumor, is a 2-stage surgical procedure. The first operation is emergent and includes primary tumor resection and end colostomy followed by scheduled colostomy closure. These operations are associated with high mortality and morbidity. Recently, insertion of self-expanding metallic stents for temporary colonic decompression has been was introduced. With this new technique colostomy can be avoided in the acute phase.

In advanced colonic cancer stent-insertion is the only palliative treatment. It is done in the radiology department under fluoroscopic guidance. We present 2 cases of malignant colonic obstruction treated successfully by stent insertion.

Long-Term Sequelae of Malignant Tumors in Childhood

N. M. Peretz, H. Goldberg, A. Kuten, I. Meller, E. Krivoi, A. Lorber, L. Bentur, A. Lightman, V. Gorenberg, M. Ben Arush-Weyl

Pediatric Hematology-Oncology Dept., Oncology Center, Pediatric Cardiology Unit, Pulmonology and Gynecology Depts., and Pediatric Endocrinology Unit; Rambam Medical Center and Technion-Israel Institute of Technology, Haifa; and Orthopedic-Oncology Unit, Tel Aviv-Sourasky Medical Center and Tel Aviv University

110 children with malignant diseases (leukemia excepted) who survived 5-20 years (median 9) post-therapy were followed (1996-1998). Median age during follow-up was 15 years (range 5-23). The most common malignancies were brain tumors, lymphoma, retinoblastoma and Wilm's tumor.

The 174 late side-effects included endocrine disorders (19%), cognitive impairment (14%), orthopedic dysfunction (12%), alopecia (12%), dental damage (11%), psychological (8%) and neurological (8%) disturbances, and azoospermia or amenorrhea (5%). There was no cardiac or renal damage and no second malignancy.

29% of side-effects were severe. There was significant reduction in quality of life in 54 (49%), in 27 of whom it was severe enough to require psychological intervention. Treatment of brain tumor caused 98 late side-effects in 28 patients (sequelae-to-patient ratio [SPR] 3.3). Most cognitive, endocrine and neurological disorders, and most cases of alopecia, dental and psychological difficulties were in these patients. There were frequent late complications in those treated for retinoblastoma (SPR 1.8), and bone or soft tissue sarcomas (SPR 0.8). Those treated for Wilm's tumor had few side-effects (SPR 0.4).

Late side effects were most frequent after radiation, reaching as high as SPR 2.4. It averaged only 0.5 in those treated with chemotherapy alone or in combination with surgery.

Reduction of late side-effects in these patients requires using less toxic modalities, as long as cure rate is not compromised. When considering secondary strategies, screening for early detection of late complications would enable immediate solutions, such as hormonal replacement or providing compensating skills for post-treatment disability.
 

Photodynamic Therapy of Nasal Basal Cell Carcinoma

R. Haddad, M. Cohen, O. Kaplan, R. Greenberg, H. Kashtan

Dept. of Surgery, Tel Aviv-Sourasky Medical Center

Photodynamic therapy (PDT) is a noninvasive modality used topically for several skin cancers. We evaluated the effects of PDT on basal cell carcinoma (BCC) of the nose, using aminolevulinic acid (ALA) as a photosensitizer and a non-laser light source (Versa-LightTM). The advantages of this light source are synergistic, hyperthermia and fewer side effects.

A paste of 20% ALA was applied topically to biopsy-proven BCC of the nose. Lesions were covered with occlusive light-shielding dressing and after 18 hours they were submitted to 10 minutes of exposure to the light. Initial evaluation was made after 21 days and every 3 months thereafter. Patients who did not respond after 2 treatments were referred for surgery.

Mean follow-up in 31 patients was 19 months (range 6-36). There were no significant side-effects. There was complete response in 24/27 (88.9%), in whom there was recurrence in 2/27 (7.4%).
 

Pulmonary Adenocarcinoma in Myasthenia Gravis - Auto-Immunity and Late Development of Malignancy

Yitzhak Rosen, Yair Levy, Yehuda Shoenfeld

Dept. of Internal Medicine B, Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University

We report a 75-year-old man with myasthenia gravis for many years, who was hospitalized because of cough, fever, and dyspnea. Chest x-ray revealed a bilateral pleural effusion. Adenocarcinomatous cells were found in the pleural fluid. Computerized tomography of the chest showed widespread pulmonary dissemination of the tumor.

The relationship between myasthenia gravis, an autoimmune disease involving the motor end-plate, and malignancy (thymoma) has been widely recognized. Current literature documents few reports of lung malignancies with concurrent development of myasthenia gravis. A tentative explanation, based on current research, is provided for the possible role of myasthenia gravis and the late development of lung cancer. Moreover, a model for the autoimmune phenomenon and the development of late malignancies will be provided with explicit explanations. It is important to search for occult, developing malignancies in newly diagnosed autoimmune diseases.

Preservation of the Larynx in Advanced Cancer

Edward Rosenblatt, Nava Siegelmann-Danieli, Jemal Zidan, Nisim Haim, Abraham Kuten

Oncology Dept., Rambam Medical Center and Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa

The effectiveness of sequential chemo-radiotherapy in preserving the larynx in advanced laryngeal carcinoma was assessed. 4 Unselected patients (19 men and 2 women, mean age 60 years) with advanced squamous cell carcinoma of the larynx (T3-4/N0-3) received induction chemotherapy consisting of 2-3 cycles of cisplatin (100 mg/m²) and 5-flourouracil (1000 mg/m²/day) as a continuous infusion on days 1-5 followed by definitive radiotherapy: 50 Gy to the whole neck, 70 Gy to the larynx and clinically involved nodes, using a combination of 6 MV photons and 9-12 MeV electrons.

19 of The 21 patients responded to combined therapy but there was no response to induction therapy in 2 (10%) and 2 did not complete therapy due to severe toxicity. At a mean follow-up of 40 months, 7 had undergone total laryngectomy (33%), for an overall 5-year laryngeal preservation rate of 66%. Reasons for total laryngectomy in 2 patients were no response and in 5 tumor recurrence.

Mean survival was 39 months (range 11-46 months); at last follow-up 17 of 21 were alive and disease-free, 11 of whom had a functional larynx (65% of survivors). 2 died due to disease progression and 1 due to a cardiovascular event. Sequential chemo-radiation allows laryngeal preservation in about 2/3 of surviving patients without compromising survival.

Laparoscopic Ultrasound in Predicting Resectability of Choriocarcinoma

M. Shimonov, P. Schachter, G. Gvirtz, Y. Avni, A. Rosen, A. Czerniak

Depts. of Surgery, Ultrasound, and of Gastroenterology, Wolfson Medical Center, Holon and Sackler Faculty of Medicine, Tel Aviv University

Cholangiocarcinoma accounts for about 1% of all malignant tumors. They are difficult to diagnose because of their small size and their location. Although surgical resection is the best therapeutic approach, most patients undergo unnecessary exploratory laparotomy due to incorrect preoperative diagnosis.

We present our experience with laparoscopic ultrasound (LU) in the evaluation of cholangiocarcinoma and determination of tumor spread and vascular involvement. Of 25 patients referred for surgery, 6 were excluded following LU and were referred instead for palliative treatment. Diagnosis of the tumor was successful in 92%, and vascular involvement was diagnosed in 46%. LU should be an integral diagnostic test in the evaluation of choriocarcinoma.

Breast Tumors Demonstrated by Tc-99m Sestamibi Scintimammography 

Itzhak Pappo, Tifha Horne, Herbert Merdad, Ruben Orda

Dept. of Surgery A, Institute of Nuclear Medicine and Dept. of Pathology, Assaf Harofeh Medical Center, Zrifin; and Sackler Faculty of Medicine, Tel Aviv University

Breast cancer can be detected by scintimammography using Tc-99m sestamibi (MIBI). The method is highly accurate, sensitive and specific. Histologically, most of the tumors have been adenocarcinomas.

We present 2 women with rare breast tumors, primary squamous cell carcinoma and malignant phyllodes tumor. In both, mammography and cytological biopsy were not diagnostic, but MIBI scintimammography demonstrated focal uptake in the diseased breast.

Thallium Scan and Pulmonary Carcinoma 

Adam Mor, Amit Segev, Rami Herskoviz, Yoseph A. Mekori

Dept. of Medicine B, Meir General Hospital, Kfar Saba Affiliated with Sackler Faculty of Medicine, Tel Aviv

A 70-year-old man was admitted for exacerbation of congestive heart failure. In his assessment thallium scan of the heart was performed. An incidental finding was a focus of absorption in the right lung. The lesion was later diagnosed as adenocarcinoma based on the cytological findings.

Breast Cancer after Mantle Field Irradiation for Hodgkin's Disease 

A. Katz, B. Brenner, A. Sulkes, H. Luria, G. Marshak, E. Fenig

Oncology Institute, Rabin Medical Center, Beilinson Campus, Petah Tikva and Sackler Faculty of Medicine, Tel Aviv University

Effective use of modern therapeutic modalities results in the cure of 75%-80% of Hodgkin's disease patients, regardless of stage. The major threat to continued survival is, therefore, not recurrent disease but development of second malignancies. Recent reports have firmly established the increased risk of breast cancer developing in women treated with mantle field irradiation.

We describe 3 women who developed breast carcinoma following mantle field irradiation for Hodgkin's disease. Their clinical course was consistent with that reported in larger series. They were relatively young when irradiated and there was a long interval between radiation therapy and the diagnosis of breast cancer.

Review of the literature shows that there may be a role for prophylactic mastectomy after irradiation for Hodkgin's disease.

Solid and Papillary Pancreatic Neoplasm 

Michal Soudack, Alon Ben-Nun, Leonid Malkin, Moshe Hashmonai

Depts. of Diagnostic Radiology, Surgery A and B, and Pathology, Rambam Medical Center, Haifa

Solid and papillary neoplasm of the pancreas is an interesting and rare malignant tumor. It occurs most commonly in young women. It was first described in 1959 and since then has been referred to by different names, including solid and cystic tumor, solid and cystic epithelial neoplasms, and others. Its malignant potential is low and metastasis is very rare.

Treatment includes partial pancreatectomy with full resection of the tumor. The prognosis is generally very good. We present 3 women (aged 17, 19, 39) diagnosed and treated for solid and papillary neoplasm of the pancreas. The unique clinical, histological, and epidemiological characteristics of this tumor are detailed.

Carcinoma of the Cecum Presenting as Intussusception

Mordechai Shimonov, Oleg Kaminsky, Maya Cohen

Depts. of Surgery and Radiology, Beilinson Campus, Rabin Medical Center, Petah Tikva

In intussusception a proximal bowel segment (intussusceptum) invaginates into the lumen of a distal bowel segment (intussuscipiens). In adults intussusception represents a rare cause of intestinal obstruction and is usually secondary to an identifiable lesion that requires surgical treatment other than that of the intussusception itself.

We present a 57-year-old woman with metastatic colonic carcinoma of the ileocecal valve and intussusception of the ileum into the cecum. Right hemicolectomy was performed.