Giuseppina Parodo

Uneven hepatic iron and phosphorus distribution in beta-thalassemia

BACKGROUND/AIMS Determination of hepatic iron concentration is crucial in the evaluation of iron-storage disease. Iron content is normally determined in a part of a needle liver biopsy and the value obtained is considered to be representative of the iron concentration in the whole liver. To evaluate the reliability of this procedure, we studied iron distribution in the liver of two beta-thalassemic patients. Since the transport of intracellular iron is mediated by phosphates, we also studied the hepatic phosphorus distribution. METHODS At autopsy, a liver slice extending from the left to the right lobe was divided into 51 and 49 samples, respectively. Each specimen was subdivided into two parts: one of them was paraffin-embedded and utilized for the histochemical detection of iron; the second part was analyzed for iron and phosphorus content by induced coupled plasma atomic emission spectroscopy. RESULTS The histological picture of both livers was characterized by portal and periportal fibrosis associated with iron storage of different degree, without cirrhosis. The mean iron concentration of the liver was 20,631 +/- 4903 micrograms per g of dry tissue (micrograms/g dt) and 13,901 +/- 1976 micrograms/g dt, respectively. A striking variability in iron content between samples was also found: iron concentration ranged from 11,537 to 32,347 micrograms/g dt in the first case and from 6257 to 16,493 in the second case. We even observed regional differences in iron concentration, with a preferential peripheral accumulation in both cases and a tendency of the left compartment of the liver to accumulate more iron in the first case. Histochemical analyses confirmed the uneven iron distribution even at the acinar level, showing iron mainly being stored in hepatocytes and Kupffer cells of zone 1 of the acinus, with decreasing amounts of iron in zones 2 and 3. The mean hepatic phosphorus concentration was 6662 +/- 1300 micrograms/g dt (range: 4348-9947) and 7502 +/- 986 micrograms/g dt (range: 5844-90,282), respectively. The regional distribution of phosphorus was similar to that observed for iron. A strict correlation between iron and phosphorus content was also observed. CONCLUSIONS Our data show that: 1) iron and phosphorus are unevenly distributed in the beta-thalassemic liver, even in the non-cirrhotic stages; 2) a regional pattern of iron and phosphorus distribution is evident, characterized by higher concentrations at the periphery of the liver; 3) the observed uneven distribution of iron and phosphorus implies that their content determined in a small liver sample cannot be considered as absolutely representative of the mean hepatic iron concentration. Therefore, iron concentrations determined in a part of a needle liver biopsy should be interpreted with caution in monitoring the efficacy of the iron-chelating therapy in beta-thalassemic patients.

Uneven hepatic copper distribution in Wilson's disease

BACKGROUND/AIMS Determination of hepatic copper concentration is important in the diagnosis of Wilsons disease. We studied copper distribution in the cirrhotic liver of a patient who died of Wilsons disease. METHODS A liver slice extending from the left to the right lobe was divided into 38 samples. Each sample was analyzed for copper content by Induced Coupled Plasma Atomic Emission Spectroscopy. RESULTS The mean copper concentration in the liver was 1370 micrograms/g dt. A striking variability, up to 2-3-fold, in copper levels was observed between the samples: the copper concentration ranged from 880 to 2100 micrograms/g dt, with significant differences even between adjacent samples. Lobar differences were also observed, with a tendency of the right lobe to accumulate more copper than the left lobe. Histochemical analyses confirmed the uneven distribution of copper even at the acinar level. Copper was mainly stored in periportal hepatocytes (zone 1) and at the periphery of the regenerating nodules. Moreover, we observed some nodules with the majority of hepatocytes full of copper granules, adjacent to areas of parenchyma negative for copper stains. CONCLUSIONS Our data show that: 1) copper is unevenly distributed in Wilsons disease in the cirrhotic stage; 2) a lobar pattern of copper distribution is evident in this case, characterized by a higher copper concentration in the right lobe; 3) the observed lobar pattern is different from that described in the newborn liver, characterized by a higher copper content in the left compartment of the liver; 4) copper content determined in a small liver sample cannot be considered as absolutely representative of the mean hepatic copper concentration. From a practical point of view, our data show that sampling variability deserves more consideration in the diagnosis and in the monitoring of Wilsons disease. The use of hepatic copper concentration in monitoring the efficacy of the copper-chelating therapy may be unreliable, particularly in the cirrhotic stage, because of the patchy distribution of copper, as demonstrated in this study.

Lobular capillary hemangioma of the nasal cavity : A retrospective study on 40 patients

Background Nasal lobular capillary hemangioma (LCH) is a benign lesion of unknown etiology that must be included in the differential diagnosis of vascular lesions. Based on a large cohort of LCH patients, we retrospectively analyzed the clinical presentation, histological and radiological findings, and the treatment strategy. Methods Clinical records of 40 patients affected by LCH, treated in a 10-year period at two university hospitals, were reviewed. Data concerning symptoms, possible etiologic factors, endoscopic findings, imaging studies, and treatment were collected. Results Previous nasal trauma and pregnancy were identified as possible causes in six (15%) and two (5%) patients, respectively. The main symptoms were unilateral epistaxis (95%) and nasal obstruction (35%). Lesions ranged in size from 1 to 8 cm and mainly involved the nasal septum (45%) and the nasal vestibule (17.5%). In the four (10%) patients with a large lesion, radiological evaluation was helpful not only in assessing the extent, but also in suggesting the possible nature of the lesion. All patients underwent endoscopic resection under local (72.5%) or general (27.5%) anesthesia. At mean follow-up time of 53 months, no recurrence has been observed. Conclusion To the best of our knowledge, this is the largest series of patients with nasal LCH. When the mass is considerable in size, differentiation from other hypervascularized lesions may be intriguing. Under these circumstances, information obtained with imaging may sometimes suggest a correct diagnosis without resorting to biopsy. Endoscopic surgery is the treatment of choice even for large lesions, that do not require preoperative embolization.

Intraoperative sentinel lymph node detection by vital dye through laparoscopy or laparotomy in early endometrial cancer

Recent studies reported the feasibility of intraoperative lymphatic mapping in women with endometrial cancer but none of these studies compared the sentinel lymph nodes (SLNs) detection rates obtainable through laparoscopy or laparotomy. The purpose of this study was to address this issue.

The role of immunohistochemistry in the diagnosis of hyalinizing clear cell carcinoma of the minor salivary gland : a case report

A case of hyalinizing clear cell carcinoma (HCCC) of the minor salivary glands of the oral cavity is reported. A 52- year-old woman presented with a growing mass at the base of the tongue. The patient underwent complete resection of the tumour. The histological picture was characterized by trabeculae or solid nests of proliferating cells with a clear cytoplasm, surrounded by a hyalinizing stroma. Tumour cells were immunoreactive for Cytokeratins 5, 6, 7, 8, 14, 17 and 18. No reactivity was observed for cytokeratin 20, vimentin, S- 100 protein, smooth-muscle actin, muscle-specific actin, and calponin. These findings confirmed the diagnosis of HCCC of minor salivary glands of the oral cavity. The clinical presentation, the immunohistochemical pattern and the role of cytokeratins in the differential diagnosis of HCCC are discussed with a review of the literature.

Imaging of gynecological disease (6): clinical and ultrasound characteristics of ovarian dysgerminoma

To describe the clinical history and ultrasound findings in patients with ovarian dysgerminoma.

Serial ultrasonographic evaluation of a decidualized endometrioma in pregnancy.

results in the production of collagen and elastin with fibroelastic thickening of the endocardium, which leads to myocardial dysfunction. In the literature, endocardial fibroelastosis has generally been reported in young children or in secondand thirdtrimester fetuses. However, the condition is likely to develop earlier in pregnancy, as we have shown, and as was also demonstrated by Rustico et al.5 who reported a case of endocardial fibroelastosis with aortic stenosis at 14.5 weeks’ gestation. Our observation stresses the importance of first-trimester ultrasound examination, which can allow early termination for lethal diseases. Nevertheless, the benefits of such early systematic screening for heart diseases have to be weighed against the negative aspects6. When severe abnormalities are noticed so early it is likely that spontaneous miscarriage will ensue and parents should have the choice of continuing the pregnancy until it reaches its natural end.

Extragenital Endometrial Stromal Sarcoma Arising in Endometriosis

The diagnosis rate of deep pelvic endometriosis is increasing. Endometrial stromal sarcoma (ESS) is a rare neoplasm. Extragenital ESS is an extremely uncommon event. Very few cases of extragenital ESS have been reported to date. The diagnosis of this entity is very difficult in some instances. Knowledge about its management is also limited. In this paper, we review the current literature on the clinical management, histology, immunohistochemistry, treatment and outcome of ESS arising in pelvic endometriosis.

Parotid mass as an early sign of Kaposi's sarcoma associated with human herpesvirus 8 infection

Kaposis sarcoma of an intraparotid lymph node is extremely rare in non-immunocompromised human immuno-1 deficiency virus (HIV)-negative patients. We report a case of a left parotid mass as an early sign of Kaposis sarcoma-associated human herpesvirus 8 (HHV-8) infection in a 57-year-old patient. After subtotal parotidectomy and histopathological diagnosis of lymph node localization of Kaposis sarcoma, an accurate dermatological investigation revealed a solitary small lesion in the left foot. Chemotherapy with five cycles of vincristine gave a temporary response of the cutaneous lesion. Seven months later, a few small, firm, purplish-red lesions appeared in different areas of the body, but no adjuvant treatment was accepted by the patient since the lesions occasionally disappeared or remained stable in size. At four years follow-up, there has been no recurrence in the parotid region, and the patient is alive with cutaneous disease but in good general health. The problems related to the diagnosis, the management strategy of such a rare condition and the prognosis are also discussed.

Thyroarytenoid muscle invasion in T1 glottic carcinoma

We report the prevalence of thyroarytenoid (TA) muscle invasion in 109 consecutive patients with T1 glottic carcinoma submitted to endoscopic laser cordectomy between February 1997 and January 2006, in order to evaluate if routine resection of the TA is necessary. A total of 109 patients staged as T1 underwent endoscopic treatment with carbon dioxide laser (CO2), 36 cases were treated with type I or II cordectomies, and 73 patients underwent type III, IV or V cordectomies, with resection of all or part of the TA. Over a total of 109 patients, 6 (5.5%) cases staged as pT1 showed TA invasion. The endoscopic treatment of T1 glottic cancer should be as conservative as possible in terms of TA resection, since muscle invasion is rare. In many cases, type III and IV cordectomies can be regarded as excessive treatment. Muscle invasion found histologically after type II cordectomy can be managed by further excision.