Hemangioma of the umbilical cord with associated amnionic inclusion cyst: two uncommon entities occurring simultaneously.

This corrects the article DOI: 10.32074/1591-951X-26-17.

Hemangioma of the umbilical cord with associated amnionic inclusion cyst: two uncommon entities occurring simultaneously.

Umbilical cord hemangioma is an uncommon benign vascular neoplasm arising from the free segment of the umbilical cord, distinct from placental and fetal insertion, and is thought to originate from endothelial cells of the umbilical vessels. Cystic changes in the umbilical cord rarely occur as a consequence of the damage to the amnionic surface of the cord caused by the presence of the hemangioma. Until now, a total of 8 cases of umbilical cord hemangioma associated with cystic changes in the umbilical cord have been reported in the literature, however, among these cases, only one showed an associated cyst derived from inclusion of the amniotic epithelium, and the remaining seven cases consisted of hemangiomas with associated pseudocyst of the umbilical cord. We herein report a case of umbilical cord hemangioma with an associated amnionic epithelial inclusion cyst. Clinicopathological features and differential diagnostic considerations are also discussed.

Gemcitabine in advanced angiosarcoma: a retrospective case series analysis from the Italian Rare Cancer Network.

BACKGROUND: Angiosarcoma is a highly aggressive soft tissue sarcoma. Responses to anthracyclines plus/minus ifosfamide, and taxanes alone or in combination with gemcitabine are well documented. Very few data are available on gemcitabine as a single agent. PATIENTS AND METHODS: We retrospectively reviewed all cases of advanced progressive angiosarcoma treated with gemcitabine as a single agent (1000 mg/m(2) i.v. every week for 3 weeks every 4 weeks), at Istituto Nazionale Tumori and within the Italian Rare Cancers Network from January 2008 to November 2010. RESULTS: Twenty-five patients [mean age: 52 years; radiation therapy (RT)-related: 8] received gemcitabine. Best tumor response by RECIST was as follows: complete response = 2, partial response = 14, stable disease = 2, progressive disease = 7 cases, for an overall response rate (PR + CR) of 68%. Six of eight post-RT angiosarcomas responded to treatment. Median overall survival (OS) was 17 months. Median progression-free survival (PFS) was 7 months (range 1-40 months). One patient with a locally advanced thyroid angiosarcoma became resectable after 5 months of gemcitabine, with <10% residual viable tumor cells seen on surgical specimen. Overall, gemcitabine was well tolerated. CONCLUSIONS: Gemcitabine is active in both RT- and non-RT-related angiosarcoma, with dimensional and possibly long-lasting responses. A formal phase II study on gemcitabine as a single agent is warranted.

Irinotecan, docetaxel and oxaliplatin combination in metastatic gastric or gastroesophageal junction adenocarcinoma.

This phase II study was designed to evaluate the activity and safety of a combination of irinotecan, docetaxel and oxaliplatin in metastatic gastric or gastroesophageal junction (GEJ) adenocarcinoma. Forty patients with measurable distant metastasis received irinotecan 150 mg m(-2) and docetaxel 60 mg m(-2) on day 1, and oxaliplatin 85 mg m(-2) on day 2. Cycles were repeated every 3 weeks. The primary end point was to demonstrate a 50% improvement in time-to-progression (TTP) over historical controls. All patients were evaluable. Median TTP was 6.5 months (95% confidence interval (CI) 5.6-7.4), the overall response rate was 50% (95% CI 35-65%) and the median overall survival was 11.5 months (95% CI 8.7-14.3). Grade 3/4 neutropaenia occurred in 47.5% of patients. There were four episodes of febrile neutropaenia in three patients. Other non-haematological grade 3 toxicities included diarrhoea in four patients (10%), vomiting in three patients (7.5%) and mucositis in two patients (5%). The irinotecan, docetaxel and oxaliplatin combination chemotherapy is an active and well-tolerated novel regimen for treating metastatic gastric or GEJ adenocarcinoma and deserves further evaluation in randomised trials and in combination with molecular targeting agents.

Epirubicin, cisplatin and docetaxel combination therapy for metastatic gastric cancer.

BACKGROUND: Docetaxel is a new agent with activity in metastatic gastric cancer. This phase II study was designed to evaluate the activity and safety of an epirubicin, cisplatin and docetaxel combination in patients with this disease. PATIENTS AND METHODS: Forty-six patients with gastric adenocarcinoma with measurable distant metastasis were eligible for the study. Patients received epirubicin 50 mg/m(2) and docetaxel 60 mg/m(2), on day 1, and cisplatin 60 mg/m(2) on day 2. Granulocyte colony-stimulating factor 300 mug/day subcutaneously was given on days 5 and 6. Cycles were repeated every 3 weeks for a maximum of eight courses. RESULTS: All patients were evaluable for response and toxicity. Two complete and 21 partial responses were observed, with an overall response rate of 50% [95% confidence interval (CI) 36% to 64%]. Stable disease was observed in 13 patients (28%) and progressive disease in 10 patients (22%). The median time to progression was 6 months (95% CI 5-7) and the median overall survival was 11.2 months (95% CI 8.5-13.9). Grade 3/4 neutropenia, thrombocytopenia and anemia occurred in 46%, 7% and 13% of patients, respectively. There were five episodes of febrile neutropenia in four patients. Other grade 3 toxicities included mucositis in three patients (6.5%), vomiting in four patients (8.7%) and diarrhea in one patient (2%). There were no cardiac toxicity, severe neurotoxicity or treatment-related deaths. CONCLUSIONS: The epirubicin, cisplatin and docetaxel combination is an active and well tolerated novel chemotherapy regimen for treating metastatic gastric cancer and deserves further evaluation in randomized studies.

Cerebral vasculitis in a patient with HCV-related type II mixed cryoglobulinemia.

The peripheral nervous system is often involved in patients with mixed cryoglobulinemia (MC), while there are few reports of central nervous system involvement. We describe a case of HCV-related type II MC with peripheral and central nervous system involvement. A 61-year-old woman, suffering from flaccid tetraparesis, was referred to our department because of an increasing disability. The presence of delirium prompted us to also investigate the central nervous system. MMSE, EEG, EMG, brain CT-scan, color-Doppler of neck vessels, retinal fluorangiography and brain MRI were performed. These investigations suggested a cerebral vasculitis. The finding of very low C4 serum levels, together with high rheumatoid factor serum levels, suggested the search for cryoglobulins. The laboratory findings showed a HCV-related type II (IgMk) MC. A marked improvement of symptoms and of laboratory data was obtained by treatment with methylprednisolone + cyclophosphamide.

Neoadjuvant M-VAC (methotrexate, vinblastine, adriamycin, and cisplatin) chemotherapy and bladder preservation for muscle-infiltrating transitional cell carcinoma of the bladder.

A group of 66 patients with locally advanced T2-T4 NOMO TCC of the bladder were treated with three cycles of neo-adjuvant M-VAC chemotherapy. Of 65 evaluable patients, 18 (28%) were T2, 22 (34%) were T3a, 21 (33%) were T3b, and 4 (6%) were T4a. Patients were restaged clinically by repeat CT scan and TURB and were to undergo pathologic staging. Partial cystectomy was to be performed in patients with initial monofocal lesions who responded to therapy. As the study evolved, many patients who responded to M-VAC underwent clinical restaging only. Clinical response incorporated the results of the CT scan, cytology, and TURB. The overall clinical response rate was 82%. A cCr was attained in 28 of the 65 (43%) patients, and 25 of the 65 (38%) patients attained a cPR; 7 patients (11%) had stable disease, and 5 (8%) had progression. The median follow-up is 36(+) months (6(+)-78(+) months). The overall survival for all patients is 82% at 2 years, and 3 year survival is 73%. Of 65 patients, 44 (68%) were managed with conservative therapy (TURB or partial cystectomy). Of 44, 34 (77%) are alive, 28 (64%) with a functional bladder. Patients who had downstaging of their tumors to absence of disease (TO) or superficial disease have 2 and 3 year survival of 86 and 83%. For patients with muscle-infiltrating tumors after M-VAC, 2 and 3 year survival is 89 and 32%. Of 65 patients treated in this study, 28 (43%) have conserved normal bladder function. Response to chemotherapy may be the most important predictor of survival. Although bladder conservation is feasible in selected patients, they remain at risk for recurrence.

Neoadjuvant M-VAC (methotrexate, vinblastine, doxorubicin, and cisplatin) for infiltrating transitional cell carcinoma of the bladder.

BACKGROUND: Based on the excellent results with combination chemotherapy such as M-VAC (methotrexate, vinblastine, doxorubicin, and cisplatin) in patients with advanced disease, neoadjuvant chemotherapy has been advocated to improve survival and in some cases to permit bladder conservation. METHODS: A Phase II study of neoadjuvant M-VAC chemotherapy was performed in patients with T2-T4N0M0 bladder tumors. After clinical staging, three cycles of M-VAC were given. After patients underwent postchemotherapy clinical restaging, pathologic restaging (partial or radical cystectomy) was planned. RESULTS: Forty-six patients are evaluable. A clinical response was attained in 78%. Six patients (13%) had stable disease, and four (9%) had progression. After chemotherapy, 17 patients underwent radical cystectomy, none of whom were pTO. In this group, 10 of the 17 (59%) are alive at a median follow-up of 37+ months (range, 8-62+ months). Eleven patients had a partial cystectomy; 7 of the 11 (64%) are alive, 6 (55%) with a preserved bladder. Eighteen patients had clinical restaging only, and did not have pathologic staging. Median follow-up for this group is 36+ months (11-65+ months). Twenty-one of the 29 (72%) patients managed with conservative surgery or transurethral resection of the bladder alone are alive with a functional bladder. Median survival for all patients has not yet been reached. Two-year survival is 82%, and 3-year survival is 70%. CONCLUSIONS: The current study is of interest in terms of bladder conservation. Assessment of the true success of any bladder-preserving treatment will require longer follow-up.

Carboplatin and 5-fluorouracil in poor performance status patients with advanced urothelial cancer.

BACKGROUND: In view of the difficulties in administering aggressive treatment to elderly patients, frequently with concomitant medical problems, a treatment program with the combination of carboplatin and 5-FU for advanced urothelial tumors was designed. The aim was to maintain an efficacious therapeutic schedule while minimizing toxicity. PATIENTS AND METHODS: Twenty-three patients with advanced bidimensionally measurable urothelial carcinoma were given carboplatin 100 mg/m2 and 5-fluorouracil 500 mg/m2 days 1-3 which was escalated to carboplatin 125 mg/m2 and 5-fluorouracil 625 mg/m2. 5 patients were > 70 years, the ECOG performance status was 2-3 in 10 patients (43%), and the creatinine was > 2.0 mg/dl in 3 patients (13%). Five patients (22%) had pre-existing cardiac disease, and 1 had hepatopathy. Nine patients (39%) had prior cisplatin. RESULTS: Ten patients remained at level 1, and 12 others had the dosage escalated to level 2. Twenty-one patients are evaluable for response. Response was observed in 5 of 21 (24%) evaluable patients (95% confidence limits 15%-33%), only at dose level 2. There was 1 CR (5%) and 4 PR (19%). There were no responses in patients who had prior DDP versus 5 of 13 (38%) responses in patients who had not had prior DDP. The median time to response was 2 months. The median duration of response was 8 months. At level 2 myelotoxicity was significant, and led to a return to level 1 in 2 patients. Nine of 12 patients (75%) treated at level 2 had grade 3 leukopenia, and 1 patient had nadir sepsis. 4 patients (33%) had grade 4 thrombocytopenia. CONCLUSIONS: Moderate activity was shown with this regimen in untreated patients at level 2. This regimen presents a feasible outpatient alternative for patients who are unable to undergo more aggressive chemotherapy.

Recombinant tumor necrosis factor for superficial bladder tumors.

Twenty patients with histologically documented superficial bladder cancer (Ta, T1, Tis) were treated with intravesical administration of TNF 400-1800 micrograms. Of 18 patients with a marker lesion, 2 obtained a complete response for 8+ and 18 months. Two had a partial response and were given other intravesical therapies after 5 and 7 months. No or minimal systemic absorption of TNF was observed and documented in 4 of 20 patients by pharmacokinetic studies, and no patients developed antibodies to intravesically administered TNF. TNF was well tolerated in doses up to 1800 micrograms. No systemic or local side effects were observed. Modest activity was attained with intravesical TNF, even in pretreated patients.

Voiding disorders in patients with cerebrovascular disease.

Eighty patients affected by ischemic cerebrovascular disease (ICVD) in stable conditions were studied: brain CT scan was performed in all patients to evaluate site/extension of brain injury, while urodynamic tests were employed in those patients who showed urinary bladder symptomatology (n = 30). Twenty-six complained of urgency and urge incontinence, only 4 patients showed urinary retention. Micturition abnormalities seem to occur mostly in patients with multiple infarcts and cerebral atrophy and particularly among those with bilateral lesions.

Cluster-like headache and a median intracranial calcified lesion: case report.

A 69 year old man complaining of a cluster-like headache is reported. CT scan showed a median intracranial calcified lesion. Two main considerations are: 1) the importance of searching for an underlying structural lesion in patients with an "atypical" cluster headache; 2) the possible significance of a lesion localized close to the midline structures in the pathogenesis of cluster headache.

Fluctuations of plasma beta 2-microglobulin, soluble interleukin 2 receptor and interferon-gamma concentrations after adoptive immunotherapy with high-dose interleukin 2 and lymphokine-activated killer cells.

We report the serum levels of soluble interleukin 2 receptor (sIL2R), beta 2-microglobulin (beta 2-M) and interferon-gamma (IFN-gamma) in patients undergoing adoptive immunotherapy with rIL2 and lymphocyte-activated killer (LAK) cells. Our results indicate that rIL2 induced a marked increase of the serum concentration of these markers, although this increase varied considerably for different individuals. Parallel studies with the same patients also showed a marked rise in the number of IL2R+ lymphocytes: the IL2Rs expressed on these cells were mainly of the "low affinity" type. We suggest that evaluation of these markers may allow the monitoring of immune system activation induced by rIL2 in patients undergoing adoptive rIL2 and LAK cell immunotherapy.

"Enuresis risoria": evaluation and management.

"Enuresis risoria" or "giggle incontinence" is a particular condition characterized by a sudden, involuntary, uncontrollable and complete emptying of the bladder during giggling or hearty laughter. We had under observation a 15-year-old girl affected by this condition. The tests she underwent did not reveal anatomic or functional alterations. We were able to control her symptoms with Imipramine. We can thus assume that laughter reacts as a trigger that activates micturition reflex through the intermediation of the limbic system.