Postpartum thrombotic complication in a patient with paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal nonneoplastic hematopoietic stem cell disease characterized by an acquired mutation of the PIG-A gene with reduction or absence of CD55 and CD59. The absence of these proteins renders PNH erythrocytes susceptible to complement-mediated hemolysis. We report the case of a PNH patient before and during pregnancy until delivery. We observed and treated some postpartum thrombotic complications. Eculizumab should be used with caution in pregnancy. There are several reports supporting its use in these patients. This case should be considered paradigmatic of a series of clinical situations that may occur in the course of a pregnancy in patients with PNH: increased need for transfusion, need to increase the dose of Eculizumab, and insurgence of fetal sufferance. Moreover, after delivery, the patient, despite adequate prophylaxis with low-molecular-weight heparins, presented severe complications: development of pleural and peritoneal effusion, pulmonary embolism, bilateral upper limbs thrombophlebitis, and a possible abdominal angina with a transient paralytic ileus. All these complications were overcome and now the baby is healthy and the mother has returned to the usual therapeutic regimen.

Contrast-enhanced ultrasound for characterizing lymph nodes with focal cortical thickening in patients with cutaneous melanoma.

OBJECTIVE: The aim of this study was to ascertain the utility of contrast-enhanced ultrasound in assessing the significance of focal cortical thickening in the lymph nodes of patients followed up after surgery for cutaneous melanoma. MATERIALS AND METHODS: Ultrasound was used to examine 460 consecutive patients to identify nodes with focal hypoechoic cortical thickening. Patients whose nodes revealed these features underwent contrast-enhanced ultrasound and ultrasound-guided fine-needle aspiration cytology (FNAC) focusing on the area of cortical thickening. Enhancement in the arterial and parenchymal phases was evaluated: A generalized homogeneous or intense enhancement was considered benign and the presence of a perfusion defect was considered metastatic. RESULTS: After exclusion of 24 patients with frank signs of malignancy at gray-scale ultrasound, the study included 436 patients. Focal hypoechoic cortical thickening was seen in 44 of 436 nodes in as many patients. In 29 nodes, the area of focal thickening showed contrast enhancement similar to that of the remaining cortex on contrast-enhanced ultrasound. In 15 nodes, the area of cortical thickening was less well vascularized than the adjacent parenchyma in the arterial phase and there were areas with perfusion defects that were more evident in the parenchymal phase. FNAC focusing on the areas of focal cortical thickening identified 13 metastatic nodes and 31 nodes with benign features. Contrast-enhanced ultrasound compared with FNAC correctly classified 42 of 44 nodes, showing a sensitivity of 100% and a specificity of 99.5%. CONCLUSION: Although our findings need to be confirmed in larger series, they indicate that contrast-enhanced ultrasound can be useful in clinical practice for characterizing focal cortical thickening in lymph nodes. The exclusion or identification of regional lymph node metastases is of fundamental importance in oncologic staging because this issue directly influences both the prognosis and the choice of therapeutic strategy.

Fine-needle aspiration cytology and (99m)Tc-pertechnetate scintigraphy together in patients with differentiated thyroid carcinoma.

The aim of this study was to evaluate the usefulness of fine-needle aspiration cytology (FNAC) and (99m)Tc-pertechnetate scintigraphy (TS) together in patients with differentiated thyroid carcinoma. Data from a series of 357 patients (284 women and 73 men, median age 43 years, range 19-73) with solitary thyroid nodule and no signs of hyperfunction, who had undergone both FNAC and TS prior to surgery, were retrospectively reviewed. FNAC distinguished 3 groups of TN (benign, follicular neoplasm, cancer), while patients with 'cold' TN were considered at risk of having a thyroid tumor. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were: 95%, 21%, 20%, 95% and 34% for TS; 82%, 99%, 96%, 96% and 96% for FNAC; 98%, 99%, 97%, 98%, and 99% for TS and FNAC together, respectively. In conclusion, patients with 'cold' TN and FNAC suggesting follicular neoplasm should be considered at risk of having cancer.

Early (sentinel lymph node biopsy-guided) versus delayed lymphadenectomy in melanoma patients with lymph node metastases : personal experience and literature meta-analysis.

BACKGROUND: It is debated whether patients with melanoma who undergo lymphadenectomy after a positive sentinel lymph node (SN) biopsy (SNB) have a better prognosis compared with patients who are treated for clinically evident disease. METHODS: The records of 190 patients with cutaneous melanoma who underwent radical lymph node dissection after a positive SNB (completion lymph node dissection [CLND]; n = 100) or who had clinically evident lymph node metastasis (therapeutic lymph node dissection [TLND]; n = 90) were analyzed. Moreover, the MEDLINE, EMBASE, and Cochrane databases were searched for studies that investigated the survival impact of SNB-guided CLND compared with TLND for clinically evident disease. Standard meta-analysis methods were used to calculate the overall treatment effect across eligible studies. RESULTS: In the authors' series, tumor characteristics did not differ significantly between patients who underwent CLND and those who underwent TLND. After a median follow-up of 52.6 months, the 5-year overall survival rate did not differ significantly between CLND patients and TLND patients (68.9% vs 50.4%, respectively; log-rank test; P = .17). In contrast, a meta-analysis of 6 studies (n = 2633) that addressed this issue (including the authors' own series) indicated that there was a significantly higher risk of death for patients who underwent TLND compared with that for patients who underwent CLND (hazard ratio, 1.60; 95% confidence interval, 1.28-2.00; P < .0001). CONCLUSIONS: Although no significant survival difference was observed in either series, the pooling of summary data from all the studies that dealt with this issue suggested that SNB-guided CLND is associated with a significantly better outcome compared with TLND for clinically evident lymph node disease.

Intraoperative modification of a supracricoid laryngectomy to a subtotal laryngectomy with tracheohyoidoepiglottopexy.

In recent decades, with the widespread diffusion of the supracricoid laryngectomy in the treatment of selected advanced laryngeal neoplasm, the indications to non-conservative surgery are less frequent than in past. We report the case of a patient in whom a planned supracricoid laryngectomy was intraoperatively converted in an extended partial laryngectomy with tracheohyoidoepiglottopexy for a tumor involving the cricoid cartilage.

Meconium periorchitis: a rare cause of fetal scrotal cyst--MRI and pathologic appearance.

A case of meconium periorchitis detected by fetal MRI and misdiagnosed during pregnancy as inguinoscrotal hernia is reported for the first time. A full-term black boy presented at birth with an asymptomatic, 'stony-hard', scrotal mass suggestive of an in utero testicular torsion or testicular/paratesticular tumor. Early surgical treatment resulted in the removal of paratesticular yellowish amorphous material. Histology was consistent with the diagnosis of meconium periorchitis, a rare and benign condition resulting from healed intrauterine bowel perforation.

Usefulness of combined sestamibi scintimammography, axillary ultrasonography and FNA cytology in reducing the number of sentinel node procedures in patients with early-stage breast cancer.

BACKGROUND: Intraoperative analysis of the sentinel lymph node (SLN) status is currently performed in patients with breast cancer (BC) undergoing surgery. Axillary node (AN) metastases are present in up to 60% of cases, but the risk is only 30% in patients with early stage (T1) BC. The aim of this study was to evaluate the usefulness of 99mTc-sestamibi scintimammography (SSM), axillary ultrasonography (US) and US-guided fine-needle aspiration (FNA) cytology together in detecting axillary metastases preoperatively and their potential role in reducing the number of SLN procedures. PATIENTS AND METHODS: A series of 86 consecutive women (median age 57 years, range 30-72) with confirmed BC and clinically negative nodes (T1N0) underwent both SSM and US prior to surgery. US-guided FNA cytology was performed in all the patients with suspicious AN on US, or positive SSM and ultrasonographically visualized enlarged nodes. RESULTS: Final pathology showed 4 pT1bN0, 1 pT1bN1, 60 pT1cN0 and 21 pT1cN1 BC. The sensitivity, specificity, and accuracy were 59.1%, 93.7% and 84.9% for SSM, 63.6%, 90.6% and 83.7% for US, and 72.7%, 97.8% and 90.7% for SSM and US together. Using FNA cytology the specificity reached 100%, but the sensitivity did not increase. A combined method using radioisotope and blue dye was used for SLN biopsy. The procedure was omitted in patients with FNA cytology showing AN metastases (N = 14, 16.3%) and they underwent level I-II axillary dissection, as well as those with positive SLN biopsy on frozen section (8 out of 72, 11.1%). CONCLUSION: In patients with BC, preoperatively selected by SSM and US in whom US-guided axillary FNA cytology has shown the presence of AN metastases, the SLN biopsy can be avoided and AN dissection should be the primary procedure.

FNA cytology and frozen section examination in patients with follicular lesions of the thyroid gland.

Patients with solitary thyroid nodules should have fine-needle aspiration (FNA) cytology as the initial screening test, but the most of those referred to a surgeon usually undergo frozen section examination (FS). The aim of this retrospective study was to assess the usefulness of FNA cytology and FS together in patients with a solitary thyroid nodule (TN). Two-hundred and ten patients with a TN and FNA cytology suggesting follicular neoplasm underwent intraoperative FS and subsequent hemithyroidectomy or total thyroidectomy. There were 47 (22.4%) men and 163 (77.6%) women, with a median age of 43 years (range 18-76 years). In all patients, ultrasound-guided FNA was successfully performed using 22-G needle prior to surgery. Smears of the FNA samples were stained by May-Grünwald-Giemsa stain and evaluated immediately by the cytologist. Final histology was follicular carcinoma in 23 (10.9%), follicular adenoma in 181 (86.2%), and hyperplasia in 6 (2.9%) patients. No difference (p=NS) in age of the patients, and greatest diameter on the TN was found between groups. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were 13.0%, 97.3%, 37.5%, 90.0%, and 88.1% for FNA cytology, and 17.4%, 100%, 100%, 90.8%, and 91.0% for FS, respectively. The combination of FNA plus FS did not significantly improve the results. In conclusion, both FNA cytology and FS are highly specific tests, but their sensitivity is low, even when they are used in combination. Thus, in patients with smears suggesting follicular neoplasm, FS should be considered unnecessary because it does not affect the intraoperative decision making. FS is most useful in those cases that are diagnosed as suspicious for papillary carcinoma by FNA.

Bisphosphonate-associated jawbone osteonecrosis: a correlation between imaging techniques and histopathology.

OBJECTIVES: Recently, jawbone osteonecrosis has been reported as a potential adverse effect of bisphosphonates administration. This paper considers and highlights histopathologic and radiologic features of this condition. STUDY DESIGN: Eleven patients, owing to unresponsiveness to conservative treatment and uncontrollable pain, underwent surgical resection of diseased jawbone after extensive hyperbaric oxygen therapy. A thorough clinical, laboratory, and imaging study was performed. Surgical specimens underwent histopathologic and immunohistochemical evaluation. RESULTS: Computerized tomography (CT) scans showed increased bone density, periosteal reaction, and bone sequestration in advanced stages. With magnetic resonance imaging (MRI), exposed areas showed a low signal in T1- and T2-weighted and inversion recovery images, which suggests low water content and is histopathologically correlated with paucity in cells and vessels (osteonecrotic pattern). Unexposed diseased bone was characterized by T1 hypointensity and T2 and IR hyperintensity, which suggests high water content and inflammation, associated with hypercellularity, osteogenesis, and hypervascularity (osteomyelitic pattern). CONCLUSIONS: Diseased bone extends beyond the limits of the bone exposed in the oral cavity. Histopathologic examination correlated well with CT and MRI, which are the choice for the evaluation of bisphosphonate-associated jawbone osteonecrosis.

High risk of malignancy in patients with incidentally discovered adrenal masses: accuracy of adrenal imaging and image-guided fine-needle aspiration cytology.

AIMS AND BACKGROUND: The incidental finding of nonfunctioning adrenal masses (incidentalomas) is common, but no reliable criteria in differentiating between benign and malignant adrenal masses have been defined. The aim of this preliminary study was to assess the usefulness of adrenal imaging and image-guided fine-needle aspiration cytology in patients with nonfunctioning adrenal incidentalomas with the aim of excluding or confirming malignancy before surgery. METHODS: Forty-two consecutive patients (18 men and 24 women; median age, 54 years; range, 25-75 years) with incidentally discovered adrenal masses of 3 cm or more in the greatest diameter were prospectively enrolled in the study. All patients underwent helical computerized tomography scan and image-guided fine-needle aspiration cytology, 33 (78.6%) underwent magnetic resonance imaging, and 26 (61.9%) underwent norcholesterol scintigraphy before adrenalectomy. RESULTS: The revised final pathology showed 30 (71.4%) benign (26 adrenocortical adenomas, of which 3 were atypical, 2 ganglioneuromas, and 2 nonfunctioning benign pheochromocytomas) and 12 (28.6%, 95% CI = 15-42) adrenal malignancies (8 adrenocortical carcinomas and 4 unsuspected adrenal metastases). The definitive diagnosis of adrenocortical carcinoma was made according to Weiss criteria and confirmed on the basis of local invasion at surgery or metastases. The sensitivity, specificity and accuracy were 75%, 67% and 83% for computerized tomography scan, 92%, 95% and 94% for magnetic resonance imaging, 89%, 94% and 92% for norcholesterol scintigraphy, and 92%, 100% and 98% for fine-needle aspiration cytology. The sensitivity and accuracy of image-guided fine-needle aspiration cytology and magnetic resonance imaging together reached 100%. Immediate periprocedural complications of fine-needle aspiration cytology occurred in 2 (4.7%) patients: self-limited pneumothorax (n = 1), and severe pain (n = 1) requiring analgesic therapy. No postprocedural or late complications were observed. CONCLUSIONS: With the aim of selecting for surgery patients with a non-functioning adrenal incidentaloma of 3 cm or more in diameter, the combination of magnetic resonance imaging and fine-needle aspiration cytology should be considered the strategy of choice.

Automated quantitative evaluation of lymph node perfusion on contrast-enhanced sonography.

OBJECTIVE: The aim of this study was to assess the performance of experimental software (Qontraxt) intended to provide automated quantification of sonographic signal intensity, which is related to the contrast enhancement of lymph node tissue, to differentiate benign from malignant lymph nodes. SUBJECTS AND METHODS: In 31 patients (age range, 24-86 years; mean age +/- SD, 53.6 +/- 14.4 years) a single lymph node per patient was evaluated on sonography after the administration of sulfur hexafluoride-filled microbubbles. The stored sonographic images were analyzed and processed into chromatic maps that had numeric values related to the amount of contrast. The lymph node regions in which the increase of signal intensity values with respect to baseline were highest (maximum signal intensity value [SImax]) and lowest (minimum signal intensity value [SImin]) were identified, and the corresponding numeric data were stored. Statistical analyses were performed by means of the Student's t test; a p value of less than 0.05 was considered to be statistically significant. RESULTS: Histopathologic analysis revealed metastatic lesions in 12 of the 31 lymph nodes; the remaining 19 were benign (16 reactive lymph nodes, two cases of granulomatous lymphadenitis, and one case of tubercular lymphadenitis). Values obtained from the SImax regions showed no consistent difference between benign and malignant lymph nodes; on the other hand, values from the SImin regions comparing baseline and maximal contrast-enhanced values were significantly different in the two groups (p < 0.001). Confidence for characterization of malignancy was significant using the difference between values from SImax and SImin regions, with the higher diagnostic value from 24 to 31 inclusive: sensitivity, 92% (11/12); specificity, 89% (17/19); positive predictive value, 85% (11/13); and accuracy, 90% (28/31). CONCLUSION: The software being tested proved to be useful in differentiating benign from metastatic lymph nodes on the basis of the quantitative data it provided.

Is there a role for arthroscopic synovectomy in oligoarticular juvenile idiopathic arthritis?

OBJECTIVE: To evaluate the longterm efficacy and safety of arthroscopic synovectomy (AS) in children with oligoarticular juvenile idiopathic arthritis (JIA). METHODS: Patients with oligoarticular JIA and persistent monoarticular involvement, refractory to nonsteroidal antiinflammatory drugs (NSAID) and/or intraarticular corticosteroid (IAC) treatment underwent AS followed, one month later, by IAC. The efficacy of AS was prospectively evaluated, and a good response was defined as absence of synovitis or > or = 60% decrease in articular score from baseline. Clinical, laboratory, and radiological variables (radiographs, ultrasound, magnetic resonance imaging) were noted to examine possible factors predictive of the result. RESULTS: Twenty-two patients with JIA (15 female, 7 male) entered the study. Age at disease onset was 77 months (range 13-168). Mean disease duration at the time of AS was 50 months (3-324). Nineteen knees, 2 temporomandibular joints, and one shoulder were treated; the mean followup was 57 months (12-168). Thirty-six percent of patients relapsed within 12 months of the procedure, 14% within 24 months, and 14% thereafter. Eight patients (36%) remain in remission after a mean 65 months' followup. Variables found to be predictive of good response were persistent monoarticular course (p = 0.004), short disease duration at the time of AS (p = 0.03), and normal erythrocyte sedimentation rate and C-reactive protein at baseline (p = 0.008 and 0.01, respectively). CONCLUSION: AS is a safe but only partially effective procedure in patients with oligoarticular JIA. Best results are achieved early in the disease course in children with persistent monoarticular involvement and no evidence of systemic inflammation.

Sensitivity and positive predictive value of CT, MRI and 123I-MIBG scintigraphy in localizing pheochromocytomas: a prospective study.

AIM: To establish a standardized non-invasive imaging protocol for patients with pheochromocytoma undergoing surgery. METHODS: A series of 32 consecutive patients (16 men, 16 women; median age 43 years, range 15-71 years) with biochemically confirmed pheochromocytoma underwent computed tomography (CT) scanning, magnetic resonance imaging (MRI) and meta-[I]iodobenzylguanidine (MIBG) whole-body scintigraphy prior to adrenalectomy or excision of extra-adrenal tumour (paraganglioma). RESULTS: At final pathology no malignant pheochromocytomas were found. The tumour was right-sided in 16 (50%) patients, left-sided in 13 (41%), extra-adrenal (sympathetic ganglia, upper abdomen) in two (6%) and bilateral in one (3%) patient. Overall, the median greatest diameter (size) of the tumour was 35 mm (range, 15-90 mm). The sensitivity of CT, MRI and MIBG scintigraphy was 90%, 93% and 91%, and the specificity was 93%, 93% and 100%, respectively. The three patients with false negative scintigraphy had an intra-adrenal tumour, ranging from 20 to 50 mm in size. The presence of necrosis within the mass might justify the lack of significant uptake of radiopharmaceutical in two patients, and the small size (15 mm) of the mass in the other. There were two false positive results with both CT and MRI, and no false positive MIBG scintigraphy, which had the highest (100%) positive predictive value. The combination of MRI+MIBG scintigraphy reached 100% sensitivity and positive predictive value. CONCLUSION: Our data suggest that this imaging protocol should be used in all patients with biochemically confirmed pheochromocytoma.

Role and cost-effectiveness of adrenal imaging and image-guided FNA cytology in the management of incidentally discovered adrenal tumours.

Incidentally discovered adrenal masses (incidentalomas) are relatively frequent and unsuspected incidentalomas (AI) of more than 1 cm in size may be found in 1-5% of patients who have undergone abdominal or chest computed tomography (CT)-scan for unrelated reasons. Once an AI is detected, the two major questions are whether the patient has biochemical evidence of adrenal hyperfunction, and whether the mass is an adrenal metastasis or a malignant adrenal tumour. In most cases (>90%) AI are non-functioning, with a low (<10%) risk of being malignant, and an estimated cumulative risk of malignant transformation of less than 1:1000. However, all patients with non-functioning AI usually undergo several imaging studies, but the impact of imaging techniques and image-guided fine-needle aspiration cytology (FNAC) on the cost-effectiveness in the management of patients is not well established. A single test for disease probabilities is not always more cost-effective than two-test approaches and it has been shown that the cumulative sensitivity and accuracy of both FNAC + magnetic resonance imaging (MRI) and FNAC + norcholesterol adrenal scintigraphy reach 100%, at a similar cost-to-accuracy ratio (7.5 vs. 7.0), whilst the strategy CT-scan + MRI together is less sensitive at a lower cost-to-accuracy ratio. In conclusion, the significance of AI, as well as the optimal management approach to treatment, is still under discussion. However, image-guided FNAC in conjunction with MRI as the exclusive imaging test has the major role and cost-effectiveness in the management of patients with AL, and should be considered the strategy of choice in distinguishing between benign and malignant non-functioning adrenal masses of more than 2 cm in diameter.

Usefulness and limits of ultrasound-guided hook-wire positioning for localisation of soft tissue lesions prior to surgery.

PURPOSE: The aim of our study was to assess the usefulness of positioning metal wires under ultrasound guidance for localising soft tissue lesions in the preoperative phase. MATERIALS AND METHODS: We studied superficial soft-tissue lesions in 12 patients, using hooked mammographic wires of different lengths. One patient had a multifocal growth of disease which required a double localisation procedure. Correct positioning of the wire was confirmed by ultrasonography. All patients underwent surgery within five hours of hook-wire positioning. RESULTS: Correct wire position was confirmed at surgery in 12 out of 13 procedures. In one case the hook-wire reached the margin of the lesion. In all cases, the preoperative localisation procedure facilitated identification and resection of the masses. CONCLUSIONS: In our experience, the main indications for hook-wire positioning before surgery are: marking of small lesions, localisation of lesions in anatomic areas structurally subverted by previous surgery and consequently difficult to detect, guidance for surgical sectioning in order safeguard the noble structures close to the lesions.

Usefulness of 99mTc-pertechnetate scintigraphy and fine-needle aspiration cytology in patients with solitary thyroid nodules and thyroid cancer.

The aim of this study was to evaluate the accuracy of fine-needle aspiration (FNA) cytology and thyroid scintigraphy (TS) in patients with solitary thyroid nodules. We retrospectively reviewed a series of 657 consecutive patients (531 (80.8%) women and 126 (19.2%) men, median age 45 years, range 16-81 years) with solitary thyroid nodules. Prior to surgery, all patients underwent FNA cytology whilst 99mTc-pertechnetate TS was performed in 496 (75.5%) patients. Final histopathology showed 533 (81.1%) benign nodules, including 251 (38.2%) follicular adenomas and 124 (18.9%) thyroid carcinomas. The sensitivity, specificity, positive predictive value, negative predictive value and accuracy in the detection of thyroid cancer were 95.8%, 21.1%, 22.6%, 95.5% and 35.7% for TS, and 81.4%, 99.4%, 97.1%, 95.8% and 96.0% for FNA cytology. The presence of thyroid cancer was confirmed in 92 out of 407 (22.6%) patients with a "cold" nodule, in 4 (4.5%) patients who had normal or increased uptake on TS, in 101 out of 104 (97.1%) patients with smears suggesting malignancy, and in 3 out of 533 (0.6%) smears suggesting benign lesions. In conclusion, the specificity of TS is low and this technique should be abandoned as a routine test in patients with nontoxic thyroid nodules. However, a more careful evaluation should be suggested for patients with cold thyroid nodules and a FNA cytology that reads follicular neoplasm.

Progressive bilateral sensorineural hearing loss probably induced by chronic cyclosporin A treatment after renal transplantation for focal glomerulosclerosis.

OBJECTIVE: The immunosuppressive agent cyclosporin A (CsA) has contributed to the success of organ and bone marrow transplantation. CsA-related neurotoxicity is a well-known occurrence. Sensorineural hearing loss (SNHL) due to initiation of CsA treatment is an extremely rare finding. MATERIAL AND METHODS: A 32-year-old man who had undergone technically uneventful cadaveric renal transplantation for focal glomerulosclerosis when 25 years old was evaluated as the result of a 10-month history of bilateral hearing loss. The patient had been taking only CsA (150 mg twice daily) and methylprednisolone. RESULTS: Progressive bilateral SNHL was confirmed by an audiological examination. Eight months after dose reduction of CsA, pure-tone audiometry excluded progression of hearing loss. CONCLUSIONS: To the best of our knowledge, only rare cases of CsA-related hearing loss have been reported, and none after long-term CsA treatment. Audiological findings confirmed the cochlear origin of SNHL in our patient. The action of CsA on the blood-inner ear barrier has recently been demonstrated but the mechanism of cochlear damage is still unknown. A prospective study to determine the incidence of CsA-induced hearing loss has been instituted in our department.