Natale Pennelli

Cardiac conduction system abnormalities as a possible cause of sudden death in young athletes

Histologic examination of serial sections of the conduction system of the hearts of three young persons who died suddenly while engaged in active sports was performed. In no case were electrocardiogram tracings available. One patient, an 11-year-old girl, had micro-Ebsteins anomaly of the tricuspid valve associated with a septoseptal Kent fascicle through a wide gap of the central fibrous annulus and upper Mahaim fibers. Another patient, a 24-year-old football player, had a focal, 75 per cent obstructing atherosclerotic plaque in the proximal descending coronary artery, with scattered band necrosis and patchy myocardial fibrosis; upper and lower Mahaim fibers joined the atrioventricular node and the left bundle branch with the crest of the interventricular septum. The third patient, a 26-year-old cycling champion, had a conspicuous atrial fascicle of ordinary and transitional myocardium bypassing the atrioventricular node to anastomose with the His bundle (James accessory atrioventricular pathway). These findings indicate that atrioventricular conduction system abnormalities may play a fatal arrhythmogenic role in sudden death and raise questions regarding the prevention of electrical instability in young people engaged in active sports.

Chondrosarcoma of the larynx: Review of the literature and report of three cases

Three patients with laryngeal chondrosarcoma were observed at the ENT Department of Padua University from 1966 to 1983. Including the present observations, about 150 of these neoplasms have been described in the literature. The male to female ratio was 3:1, and the age at diagnosis ranged from 33 to 91 years (median, 66 years). The rate of metastasis was 8 per cent, and histologically positive lymph nodes developed in only five patients. Surgery is the treatment of choice for laryngeal chondrosarcoma. Radical neck dissection does not appear to be indicated, unless results of clinical examination suggest metastatic lymph nodal involvement.

Right Ventricular Myxoma: Review of the Literature and Report of Two Patients

The cases of 2 patients with right ventricular myxoma are reported, together with a review of the literature. In both patients the ultimate diagnosis was reached by means of angiocardiography, which revealed large filling defects in the right ventricle. In 1 patient, cardiac catheterization failed to record a transpulmonary gradient. Echocardiography, performed after hemodynamic investigation in both patients, revealed the usual pattern of abnormal echoes moving from the right ventricular cavity to the right outflow tract during the cardiac cycle. In both patients the tumor was successfully excised through a right atrial approach. This approach was preferred to the right ventriculotomy because it provides adequate surgical exposure, avoids undue trauma to the ventricular myocardium, and offers an easy way for inspection of the left heart.

Long-term survival in response to combined chemotherapy and radiotherapy in laryngeal small cell carcinoma.

Fourteen patients with small cell carcinoma of the larynx are studied. This represents the largest series, from a single institution, reported in the literature. This neoplasm is usually highly aggressive and the prognosis very poor, but, in our experience, combined chemo- and radiotherapy can significantly improve the clinical course of the disease. Three of six patients who received this combined modality treatment are still clinically disease-free more than six years after the initial diagnosis.

The extent of surgery for thyroid medullary cancer.

Aims Evaluation of the impact of the extent of primary surgery and reintervention on the outcome of patients with medullary thyroid carcinoma. Methods Seventy-two patients with medullary thyroid carcinoma (MTC) were surgically treated between 1967 and 1992. Results Fifty-five cases were sporadic, 5 patients had MEN 2A, 4 MEN 2B syndrome and 8 familial non-MEN MTC; 1 patient had stage I disease, 30 patients stage II, 36 stage III and 5 stage IV. Sixty-four had their initial treatment at our center, and 8 came for subsequent treatment. At first treatment, 8 patients were subjected to partial thyroidectomy, 10 to total thyroidectomy, 53 to total thyroidectomy with neck dissection, and 1 to only radical neck dissection; postoperative serum calcitonin (Ct) levels returned to normal in 3, 6 and 27 patients, respectively. In the patient with only radical neck dissection, Ct levels remained elevated. No patient with Ct normalization after surgery became responsive to pentagastrin in the follow-up. Thirteen patients had a reoperation due to nodal relapse. At a mean follow-up of 5.7 years (6-252 months), the 10-year survival rate was 84.5% with a significant difference between patients under and over 40 years of age (96.4 vs 57%), between stage I-II (100%) and stage III, IV (83.8%, 0% respectively). At the last follow-up, 36 (50%) patients were alive and disease free and 26 were alive with disease (15 with distant metastases). Of the 10 deaths, 7 were due to tumor recurrence, 3 to 120 months after surgery. Conclusions Data suggest that an earlier diagnosis rather than more extensive surgery could improve survival and reduce recurrences. However, the least treatment required is total thyroidectomy plus central neck and upper mediastinum clearance and in addition, according to the extent of nodal involvement, mono- or bilateral neck dissection. To avoid ineffective reoperation due to distant (mainly liver) micro-metastases, persistent residual microscopic disease requires a more aggressive restaging.

Cardiac ultrastructure in primary restrictive cardiomyopathy.

A five-year clinical follow-up and the results of myocardial biopsies are described in a patient with primary restrictive cardiomyopathy. Histologic examination of a right ventricular endomyocardial biopsy taken early in the course of the illness was not contributory. Histologic examination of a left ventricular endomyocardial biopsy five years later showed hypertrophy and disarray of myocytes, thickening of the endocardium, and interstitial fibrosis. Connective tissue was compact and regularly oriented in the endocardium, but tangled and irregularly oriented in the interstitium. It is concluded that the irregular network of collagen fibrils and elastic fibers limits diastolic relaxation and prevents ventricular dilatation; that the coexisting hypertrophy results from an attempt to maintain normal pump function; and that the myocyte disarray is a consequence of abnormal mechanical forces generated under conditions of severe fibrosis.

Reproducibility in the diagnosis of needle core biopsies of non-palpable breast lesions: An international study using virtual slides published on the world-wide web

Zito F A, Verderio P, Simone G, Angione V, Apicella P, Bianchi S, Conde A F, Hameed O, Ibarra J, Leong A, Pennelli N, Pezzica E, Vezzosi V, Ventrella V, Pizzamiglio S, Paradiso A & Ellis I
(2010) Histopathology 56, 720–726
Reproducibility in the diagnosis of needle core biopsies of non‐palpable breast lesions: an international study using virtual slides published on the world‐wide web

Androgen producing adrenocortical carcinoma.

Two cases of androgen secreting adrenocortical carcinoma have been described by light and electron microscopy. The histological and ultrastructural features of the tumour cells were similar to those of compact cells of zona reticularis and to those described in virilizing adenomas. They possess numerous mitochondria with lamellar and tubular cristae, abundant smooth endoplasmic reticulum, lipofuscin bodies and scanty lipid. Irregularly shaped, crenated mitochondria, with outpouchings of the outer limiting membrane have also been observed. The clusters of neoplastic cells were surrounded by basement membrane which demonstrated a focal discontinuity, probably reflecting malignancy of the tumours. Hyperplasia of smooth endoplasmic reticulum and the presence of outpouchings of the mitochondrial outer limiting membrane might be the morphological manifestation of endocrine activity of the tumours.

[Biological, histological and ultrastructural aspects of lymphomas of the Hodgkin type in SJL-J mice].

Spontaneous lymphoma developed in 57 % female SJL/J mice, with a mean latent period of 58 weeks and in 33 % male SJL/J mice with a mean latent period of 72 weeks. In (CBA x SJL) F1 and F2, the incidence in females was 79 % and 67 % at a mean age of 107 and 100 weeks respectively while in males it was 53 % and 41 % at 111 and 106 weeks. In reciprocal F1 and F2 hybrids, an incidence of 62 % and 37 % was observed in females at 118 and 109 weeks of age, while in males it was 33 % and 43 % at the mean ages of 72 and 90 weeks. Gross lesions were usually restricted to mesenteric, cervical, axillary and inguinal lymph nodes, Peyers patches and spleen. Less frequently, lungs, liver, kidney and thymus were also involved. Histological examination of nodes in the pre-neoplastic stage revealed follicular hyperplasia with prominent germinal centers, sinus hystiocytosis, precence of pyroninophilic cells within the paracortical area and medulla, medullary plasmocytosis and epithelioid sarcoid-like noduli. Nodes, spleen and other affected organs examined during overt disease showed a pleomorphic pattern characteristic of type B reticulum cell neoplasm according to Dunn and contained predominantly plasma cells, giant cells or reticulum cells. At the electron microscope, these different cell types were detected within the neoplastic tissue. In addition, cells resembling immunoblasts were frequently observed, which suggests that they might represent proliferating tumor cells capable of differentiating into plasma cells. Type C-immature and A intracisternal particles were repeatedly observed, but no type C-mature particles were seen. Attempts to transplant neoplastic tissue in syngeneic hosts were successful. Cell-free extracts from primary SJL/J lymphomas were injected in newborn RFM and CBA mice. 4 out of 9 RFM mice developed SJL/J-like lymphoma at 64–70 weeks of age, while no lymphomas were found in 8 CBA mice over 70 weeks of age. Freund adjuvant injected intraperitoneally in (CBA x SJL/J) F1 mice did not modify the incidence or the latency of the lymphomas when compared with control animals.

Tumor Induction by Murine Leukemia/Sarcoma Viruses: Morphological and Immunological Studies

Murine sarcoma virus (MSV), injected in immunocompetent adult mice, has the peculiarity of inducing tumors which have a high incidence of complete regression. However, when this virus is injected in newborn mice or in adults, immunosuppressed by various treatments (1,2,3), the induced sarcomas grow progressively and ultimately cause the host’s death. Since neoplastic cells release infectious virus and possess a strong antigenicity, it is generally agreed that tumor regression is mediated by an immunological reaction against tumor-specific virus and/or cellular antigens. The operational factors responsible for this regression are controversial. Therefore, we have elected to study the relationship between morphological and biological data on tumor growth and regression in mice injected with MSV. Moreover, preliminary data obtained on the effects of adoptive immunization and pre-infection with murine leukemia virus (MLV) on the oncogenicity of MSV will also be reported.