Uncommon syndromes and treatment manifestations of malignancy: Case 1. Unusual association of lupus and sarcoma.
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Adolescent↗
Biomedical subjects
M Lucioni
Publications and source records attributed to M Lucioni.
Lymph node reticulum cell neoplasm with progression into cytokeratin-positive interstitial reticulum cell (CIRC) sarcoma: a case study.
AIMS: To detail on sequential biopsies the morphological and immunohistochemical features of a case of primary lymph nodal fibroblastic reticulum cell (FBRC) tumour which progressed into a clinically aggressive cytokeratin-positive interstitial reticulum cell (CIRC) sarcoma. METHODS AND RESULTS: A 70-year-old female underwent surgical excision of an enlarged submandibular lymph node. The nodal architecture was effaced by a neoplastic proliferation of medium to large cells, round to oval to spindle in shape, growing in a storiform pattern. The tumour stained for vimentin, CD68, factor XIIIa, alpha1-antitrypsin, fascin and actin. Dendritic and endothelial cell markers were negative. A diagnosis of FBRC tumour was made by combining pathological and clinical data. The patient received no therapy but 5 months later the tumour relapsed, exhibiting a deceptively pleomorphic cytology, phenotypic changes (strong cytokeratin positivity), intense p53 expression and aggressive clinical course with fatal outcome. In-situ hybridization for Epstein-Barr virus was negative. CONCLUSIONS: We speculate that the morphological changes and p53 expression of the relapsing neoplasm might reflect tumour cell dedifferentiation, in keeping with the aggressive clinical course. The intense p53 expression suggests that this oncoprotein might also play a role in reticulum cell tumorigenesis.
Aged↗
[Castleman's disease: update].
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Castleman Disease↗
Plastic tracheostomal-widening procedure: the "petal" technique.
Severe tracheostomal stenosis after total laryngectomy may require the permanent use of a tracheostomy tube which undoubtedly represents a personal and social handicap (cosmetic impairment, accumulation of sputum, noisy breathing, cough stimulation, tube management). In patients with voice prostheses, this is a major obstacle to phonation and device maintenance. Stenosis is so pronounced, in some cases, as to cause dyspnoea. The main causes of stenosis are perichondritis of the upper tracheal rings or, more frequently, a defect in the tracheostoma preparation. All such cases require surgical revision of the tracheostomal diameter, for which numerous procedures have been described in the literature. The "petal" technique, adopted by the Otorhinolaryngology O.U. in Vittorio Veneto, for four years, has been used in 59 patients. The technique is described and results of retrospective study, to assess outcome, are outlined. In 40 cases, outcome was immediately satisfactory, while recurrence of stenosis was observed in 19 patients, 9 of whom preferred to accept tube dependence while 10 were reoperated, with permanent successful results in 6 cases. In our opinion, since this is an easy surgical procedure to perform and, in the majority of cases, is carried out under local anaesthesia with good patient compliance, absence of complications and good long-term results, this should be considered the method of choice for surgical widening of permanent tracheostomas.
Adult↗
Nijmegen breakage syndrome-associated T-cell-rich B-cell lymphoma: case report.
In 1981 Weemaes et al. first described the Nijmegen breakage syndrome (NBS), a rare autosomal recessive disorder characterized by stunted growth, microcephaly, immunodeficiency, spontaneous chromosome instability, and a peculiar predisposition to cancer development. Most NBS-related malignancies are lymphomas, but their pathologic features have rarely been specified. We report here the case of a northern Italian 8-year-old child who, 2 years after the diagnosis of NBS, developed a diffuse large B-cell lymphoma (T cell-rich B-cell lymphoma variant). The histological and immunobiological features of the lymphoma population are analyzed and discussed in detail.
Antigens, CD20↗
[Treatment of laryngeal adenoid cystic carcinoma].
Laryngeal localization (nearly always hypoglottic) of adenoid cystic carcinoma is quite rare (0.6% of all malignant laryngeal tumors). In most cases the local development of the neoplasm is slow, of an infiltrating and highly invasive nature (affecting the cartilage, thyroid gland, esophagus). On the other hand in some cases, particularly in the laryngeal-tracheal localization, the tumors take on an exophytic, sessile, polypoid aspect. Although the evolution is quite slow prognosis is poor due to the latent spreading of distant metastasis (average survival is 8 years). Prognosis is further worsened by delay in diagnosis due to the poor, aspecific initial symptoms. C.A.T. and N.M.R. have made a marked contribution to improved diagnostic definition, especially in terms of tumor extension, which is of great importance in therapeutic staging. Given the poor results obtained with radiotherapy and chemotherapy the only possible treatment is surgery which, in most cases, must be quite radical. Only in those forms which are still localized (with endoluminal exophytic development) is it possible to perform conservative surgery. Two paradigmatic cases are reported here indicating two different tumor extensions: in the first case total laryngectomy was performed and extended to the thyroid gland and the upper portion of the trachea; in the second, a partial glottic-subglottic laryngectomy was performed with functional restoration of laryngeal function. Conservative surgery is only possible if the hypoglottic neoformation is limited to the larynx, if it is mainly exophytic in nature and if histological examination reveals a low degree of malignancy (a high degree of cell differentiation). Since this type of carcinoma tends to give rise to latent distant metastases prognosis is, in all cases, poor no matter what surgical technique is employed for the primary tumor. Therefore it is imperative that a conservative laryngectomy be performed in all cases where it proves oncologically possible and particular attention should be paid to the quality of the patient's post-surgery life.
Carcinoma, Adenoid Cystic↗
[Three-quarter laryngectomy. Our experience: indications, surgical technique, results].
Three-quarter laryngectomy is regarded as a supraglottic laryngectomy which has been extended on one side to the glottic plane. Indications are those for supraglottic laryngectomy at the vestibular level and of cordectomy extended to the arytenoid at the glottic level (undamaged lower paraglottic space, preserved motility). Surgical technique includes total removal of the laryngeal vestibule together with the hyoid bone, pre-epiglottic space and one supracricoid hemilarynx (vocal cord, arytenoid, medial wall of the entrance of the pyriform fossa) through an internal, subperichondrial approach. Reconstruction of a new cord on the hemilaryngectomy side is through a triangular strip of the exterior thyroid perichondrium. From 1975 to 1989 44 patients underwent a three-quarter laryngectomy in the authors' department. Functional results were positive in all cases. The oncological results were as follows: 6 recurrences (3 local, 2 locoregional lymph node recurrences and 1 distant metastasis) and 4 primary secondary tumors. 9 patients expired (6 of T, 2 of secondary tumor, 1 of other causes). The actuarial 5-year survival rate for the tumor was 79%.
Adult↗