A pseudoaneurysm of the right hepatic artery treated successfully with a stent graft - A case report.

INTRODUCTION: Haemobilia caused by pseudoaneurysms of the right hepatic or cystic artery is rare. Haemobilia classically causes gastro-intestinal hemorrhage, jaundice and upper abdominal pain. PRESENTATION OF CASE: A 76-year old female underwent laparoscopic cholecystectomy because of a severe acute on chronic cholecystitis. A massive arterial bleeding occurred during surgery, which was controlled with hemoclips. Approximately one week after surgery the patient developed severe colic pains and cholestatic liver enzyme alterations. Endo-ultrasound showed normal-width bile ducts, however during a subsequent ERCP haemobilia was observed. On computed tomography a pseudoaneurysm of the right hepatic artery was seen. Selective embolization was initially successful, however, a rebleed was observed two weeks later and a 6 × 50 mm Viabahn stent graft was placed in the right hepatic artery uneventfully. The patient remained free of complaints during 3-years of follow-up. DISCUSSION: Pseudoaneurysms of the cystic or hepatic arteries are described to be cholecystectomy or cholecystitis related. The etiology of the pseudoaneurysm in this case can be inflammatory or iatrogenic. Embolization is the golden standard in pseudoaneurysm treatment. Stent graft implantation has not been frequently described as an alternative option to surgery after a failed attempt of embolization. CONCLUSION: This case report presents a probable cholecystitis related pseudoaneurysm of the right hepatic artery, which caused haemobilia after cholecystectomy. The pseudoaneurysm was successfully eliminated with a stent graft after embolization had failed. Stent grafts should be considered a minimal invasive and effective alternative after failed embolization of a pseudoaneurysm.

[Eccrine carcinoma: a diagnostic pitfall]. / Het eccrien carcinoom, een diagnostische valkuil.

BACKGROUND: The eccrine carcinoma is a rare form of skin adnexal malignancy, usually presenting as a locally invasive, solitary lesion in the head and neck region. Histopathologically and immunohistochemically, eccrine carcinomas are difficult to differentiate from cutaneous metastases of breast carcinomas. Unlike treatment of cutaneous metastasis, treatment of a solitary eccrine carcinoma entails excision, generally without systematic therapy. CASE DESCRIPTION: A seventy-year-old woman previously treated definitively for (in situ) breast cancer, undergoes excision of a nodule on the scalp. The histopathology report indicates a lesion typical for cutaneous metastasis of breast carcinoma. However, diagnostic imaging excludes recurrent breast cancer or metastatic spread. When subsequent clonality testing with archived breast cancer tissue from the earlier episode does not show a relation, the pathologist defines the lesion as an eccrine carcinoma. A re-excision is conducted, which does not show any residual tumorous tissue, and at follow-up there are no signs of recurrence or metastases. CONCLUSION: As a relatively rare and unknown malignancy, with strong histopathological resemblance to cutaneous metastasis of breast carcinoma, eccrine carcinoma poses a diagnostic challenge to both clinicians and pathologists.

Worse Survival in Elderly Patients with Extremity Soft-Tissue Sarcoma.

BACKGROUND: Nearly half of soft-tissue sarcoma (STS) patients are over the age of 65, and the behavior of cancer in these elderly patients is poorly understood. The aim of this study was to assess the impact of age, sarcoma histotype, grade, stage, and treatment modalities on survival of extremity STS (ESTS) patients. METHODS: Patients ≥18 years diagnosed with ESTS between 1989 and 2008 were selected from the Netherlands Cancer Registry. Survival rates and patient and treatment characteristics were analyzed for all patients. Relative survival and relative excess risk of death were estimated for young (<65 years) and older (>65 years) patients. RESULTS: Overall, 3066 patients were included in this study. Histotype was different between young (<65 years) and elderly (>65 years) patients (p < 0.001). Patients over the age of 65 were more often diagnosed with high-stage ESTS and an increasing proportion of high-grade ESTS (p < 0.001). The proportion of patients who received no treatment increased with age, and the elderly received fewer combined-modality treatments. Age was significantly associated with relative 5-year survival [72.7 % for younger patients and 43.8 % for the oldest elderly (>85 years)]. In multivariable analysis, age still remained a significant prognostic factor. CONCLUSIONS: Different distribution of sarcoma histotypes, more high-stage and high-grade sarcomas at diagnosis, less aggressive treatment, and worse survival rates emphasize the need for optimizing sarcoma research and care of the elderly.

Cecal volvulus caused by a large uterine leiomyoma.

INTRODUCTION: Cecal volvulus is a relatively uncommon encountered clinical condition. PRESENTATION OF CASE: A 48-year-old patient known with a large uterine leiomyoma, presented with progressive abdominal pain since one week. An abdominal computed tomography scan revealed a very large leiomyoma of the uterus, severely distended loops of the small bowel with a caliber change and a suggested 'whirl sign' of the mesenteric vessels. A laparotomy was performed, showing a very large uterus as well as torsion of the mesentery of the cecum with a sharp demarcated area of necrosis of the right hemicolon. DISCUSSION: Cecal volvulus due to a large uterine mass is a rare encountered clinical entity. The suggested mechanism might be the same mechanism causing cecal volvulus during pregnancy; the enlarged uterus raisingout the mobile cecum out of the pelvis. Obstruction may occur from kinking of the colon at a fixed point. CONCLUSION: This case demonstrates that uterine leiomyoma can be a cause of a cecal volvulus, leading to severe intestinal strangulation.

A very late recurrence of a formerly misdiagnosed low grade endometrial stromal sarcoma metastasized to the colon.

INTRODUCTION: Endometrial stromal sarcomas are rare mesenchymal neoplasms of the uterus with an indolent clinical course but a high risk of recurrence. PRESENTATION OF CASE: We report a case of a 78 year old woman who presented with rectal bleeding and recurrent urinary tract infections, caused by a very late recurrence of a formerly misdiagnosed low grade endometrial stromal sarcoma, metastasized to the colon. DISCUSSION: Endometrial stromal sarcomas are difficult to diagnose, both due to the rarity of the tumor and because of the close resemblance of the tumor to normal stromal tissue. These tumors are known for a high tendency of recurrence, therefore long term follow up is required in patients with endometrial stromal sarcoma. CONCLUSION: In patients with a history known for endometrial stromal sarcoma recurrence should always be considered.

[Limb perfusion in soft tissue sarcomas: twenty years of experience]. / Ledemaatperfusie bij wekedelensarcomen: ervaringen van 20 jaar.

OBJECTIVE: To evaluate the long-term results of 20 years of experience with isolated limb perfusion (ILP) with tumour necrosis factor α (TNF-α) and melphalan, followed by surgical resection and adjuvant radiotherapy, for the treatment of advanced soft tissue sarcomas of the extremities. DESIGN: Retrospective cohort study. METHOD: From 1991 to 2011, 113 patients with primary irresectable soft tissue sarcomas underwent 117 ILPs at the University Medical Centre Groningen. 96 ILPs (82%) were performed in the lower limb, and 21 (18%) in the upper limb. The dosages used were 1-4 mg TNF-α and 10-13 mg/l melphalan. RESULTS: After a median follow up of 8 (range 2-15) weeks after ILP, 107 tumours were resected: 81 (76%) of the resection margins were tumor-free. After the resection, 69 patients (61%) received adjuvant radiotherapy. In total, 85 ILPs resulted in a tumoural response; 16 patients (14%) developed a local recurrence and after 46 treatments (39%), distant metastases had developed. After a median follow-up of 51 months, the limb had been spared in 88 patients (78%). The 10- year disease-specific survival was 53.8%. There was a median follow-up period of 76 months (range: 7-234); still alive at the end of this period were 56 patients (50%). A total of 83 perfusion- or resection-related complications occurred from 58 ILPs (50%): 55 (66%) early and 28 (34%) late treatment-related complications. None of the patients died as a result of the treatment. CONCLUSION: ILP is a safe and effective procedure in the treatment of advanced primary irresectable soft tissue sarcoma that can prevent amputation in many cases. It is however associated with significant morbidity and is burdensome for the patient.

TNF dose reduction and shortening of duration of isolated limb perfusion for locally advanced soft tissue sarcoma of the extremities is safe and effective in terms of long-term patient outcome.

BACKGROUND AND OBJECTIVES: Dose reduction and shortening of duration of perfusion in isolated limb perfusion with TNF-α and Melphalan (TM-ILP) are associated with less systemic toxicity and seem to be safe and effective on short-term. However, data on long-term patient outcome are scarce. METHODS: From 1991 to 2008, 102 TM-ILPs were performed in 98 patients for a locally advanced soft tissue sarcoma of the extremity. Perfusions were categorized in three groups: (A) high dose and long duration (n = 59), (B) high dose and short duration (n = 16), and (C) low dose and short duration (n = 27). Long-term local control rates and (limb)-survival were evaluated. RESULTS: Limb salvage rates were in group A 76.3%, B 62.5%, and C 85.2% (P = 0.2). With a median follow up of 76 (range 4-203) months, 50 patients were still alive (51%). Disease-specific 5-year survival was not different between the three groups: A 55.4%, B 52.5%, and C 57.3% (P = 0.9). There was no difference in local recurrence-free 5-year survival (adjusted P = 0.1) and distant metastases-free survival (P = 0.9). CONCLUSIONS: Dose reduction and shorter duration of TM-ILP seem to be safe and effective regarding long-term patient outcome, as 5-year local control rates and (limb)-survival are not compromised.

[Elastofibroma dorsi: a characteristic, benign, subscapular swelling]. / Elastofibroma dorsi: een karakteristieke, benigne, subscapulaire zwelling.

Two female patients, 63 and 67 years old, visited the outpatient department with a growing soft tissue swelling bilaterally in the subscapular region. Using CT imaging and core needle biopsy, the first patient was diagnosed with an 'elastofibroma dorsi'. Because of the benign character of this lesion an expectative policy was followed. In the second patient the swelling was resected unilaterally without preoperative clinical and radiological confirmation of the diagnosis. Pathological examination revealed the typical characteristics of an elastofibroma. As there were no symptoms, the contralateral lesion was not excised. Elastofibromas are benign, soft tissue tumours, for which resection is only indicated if they are symptomatic. To prevent unnecessary anxiety, medical investigations and interventions, on encountering subscapular soft tissue tumours, elastofibroma dorsi should be considered.

Isolated limb perfusion and external beam radiotherapy for soft tissue sarcomas of the extremity: long-term effects on normal tissue according to the LENT-SOMA scoring system.

BACKGROUND: With the combined treatment procedure of isolated limb perfusion (ILP), delayed surgical resection and external beam radiotherapy (EBRT) for locally advanced soft tissue sarcomas (STS) of the extremities, limb salvage rates of more than 80% can be achieved. However, long-term damage to the healthy surrounding tissue cannot be prevented. We studied the late effects on the normal tissue using the LENT-SOMA scoring system. PATIENTS AND METHODS: A total of 32 patients-median age 47 (range 14-71) years-were treated for a locally advanced STS with ILP, surgical resection and often adjuvant 60-70 Gy EBRT. After a median follow-up of 88 (range 17-159) months, the patients were scored, using the LENT-SOMA scales, for the following late tissue damage: muscle/soft tissue, peripheral nerves, skin/subcutaneous tissue and vessels. RESULTS: According to the individual SOM parameters of the LENT-SOMA scales, 20 patients (63%) scored grade-3 toxicity on one or more separate items, reflecting severe symptoms with a negative impact on daily activities. Of these patients, 3 (9%) even scored grade-4 toxicity on some of the parameters, denoting irreversible functional damage necessitating major therapeutic intervention. CONCLUSIONS: In evaluating long-term morbidity after a combined treatment procedure for STS of the extremity, using modified LENT-SOMA scores, two-thirds of patients were found to have experienced serious late toxic effects.

Long-term locoregional vascular morbidity after isolated limb perfusion and external-beam radiotherapy for soft tissue sarcoma of the extremity.

BACKGROUND: Isolated limb perfusion (ILP) with tumor necrosis factor alpha (TNF-alpha) and melphalan, followed by delayed surgical resection and adjuvant external-beam radiotherapy is a limb salvage treatment strategy for locally advanced soft tissue sarcomas. The long-term vascular side effects of this combined procedure were evaluated. METHODS: Thirty-two patients were treated for a locally advanced sarcoma of the upper (n = 5) or lower limb (n = 27). All patients underwent a noninvasive vascular work-up. RESULTS: Five patients underwent a leg amputation, in two cases due to critical leg ischemia 10 years after ILP. With a median follow-up of 88 (range, 17-159) months, none of the patients with a salvaged lower leg (n = 22) experienced peripheral arterial occlusive disease. Ankle-brachial index (ABI) measurements in the involved leg (median, 1.02; range, .50-1.20) showed a significant decrease compared with the contralateral leg (median, 1.09; range, .91-1.36, P = .001). Pulsatility index (PI) was decreased in the treated leg in 17 of 22 patients at the femoral level (median, 6.30; range, 2.1-23.9 vs. median, 7.35; range, 4.8-21.9; P = .011) and in 19 of 20 patients at popliteal level (median, 8.35; range, 0-21.4 vs. median, 10.95; range, 8.0-32.6; P < .0005). In patients with follow-up of >5 years, there was more often a decrease in ABI (P = .024) and PI at femoral level (P = .011). CONCLUSIONS: ILP followed by resection and external-beam radiotherapy can lead to major late vascular morbidity that requires amputation. Objective measurements show a time-related decrease of ABI and femoral PI in the treated extremity.