Ovarian fibrosarcoma: case report and latest trends in diagnostic and therapeutic management.

The authors describe a rare case of primary ovarian fibrosarcoma and the latest trends in diagnosis and therapy. The rarity of this dis-ease and the scarce number of reported cases pose serious problems in differentiating it from other fibrous forms. A 58-year-old woman presented intermittent pelvic pain and a demarcated, mobile, and solid lump in the right adnexa. Diagnostic imaging revealed a solid- cystic inhomogeneous mass occupying the right adnexa and the CA125 level was elevated. The patient underwent total hysterectomy with bilateral salpingo-oophorectomy and infracolic omentectomy. Histological findings with immunomarkers led to the final diagnosis of low-grade malignant mesenchymal neoplasm derived from the ovarian stroma compatible with fibrosarcoma. Twenty-four months follow-up showed no recurrence of disease. Ovarian fibrosarcoma is very uncommon neoplasm with a poor prognosis. Despite the efforts of several authors in reporting morphological, histological, and immunohistochemical features of this neoplasm, nowadays, the diagnosis, treatment, and prognosis are unresolved issues. The present case highlights the important role of immunohistochemistry to define histological type and differential diagnosis. As demonstrated by the authors' experience, they believe that surgery is curative in the early stages with low immunohistochemical positivity for ki67 and that chemotherapy should be reserved in advanced stages with regimens in use for the treatment of sarcomas.

Supracricoid laryngectomies: oncological and functional results for 152 patients.

The purpose of this study was to evaluate the oncological and functional outcomes in patients who underwent supracricoid laryngectomies with a crico-hyoidopexy (SCL-CHP) or a crico-hyoido-epiglottopexy (SCL-CHEP) for the treatment of primary and reccurent laryngeal cancer. A retrospective study was conducted on 152 consecutive patients seen from January 1996 to December 2006. Overall survival (OS) and disease-free survival (DFS) were analysed using the Kaplan-Meier method, and were compared according to the type of surgery and clinical stage of the tumour. The mean period before decannulation, nasogastric tube (NGT) removal and recovery of a normal diet and speech were evaluated, and statistical analyses were performed regarding the association with the type of surgery and arytenoidectomy. The median follow-up period was 49.9 months (range: 10-110 months). The 3- and 5-year OS were 87.5 and 83.5%, respectively, and 3- and 5-year DFS were 78.3 and 73.7%, respectively. For patients with early stages tumours, the 5-year OS and DFS were 92.3 and 84.6% respectively, whereas for patients with locally advanced stage tumours, the OS and DFS were 74.3 and 62.2%, respectively. Significant differences in OS and DFS for patients who had early or locally advanced cancers were found (p = 0.0004 and p = 0.0032, respectively). The rate of overall local control was 92.1%, while the mean period until decannulation or NGT removal was 25.1 and 16.6 days, respectively. The mean period until NGT removal was significantly different according to the type of surgery (p = 0.0001) and whether arytenoidectomy was performed (p = 0.0001). The reliable oncological and functional results of SCL for early and locally advanced laryngeal cancers are confirmed by our series of patients.

Malignant melanoma metastasizing to the uterus in a patient with atypical postmenopause metrorrhagia. A case report.

UNLABELLED: The uterine metastases of melanoma are very rare. At the present time, only one case occurred in our department. CASE REPORT: a 54-year-old plurigravid woman showed a metrorrhagia of unknown origin. The patient underwent a diagnostic hysteroscopy and an endometrial biopsy, in order to investigate the symptomatic postmenopausal bleeding and exclude a neoplasia, such as the endometrial carcinoma. The patient was discharged with a diagnosis of uterine fibromatosis and called back to go through a complete laparoscopic hysterectomy and bilateral adnexectomy. During the operation, some metastases were found in the genital tract. An accurate physical examination allowed us to discover a cutaneous nevus, the excision and histology of which revealed its malignancy. The immunohistochemistry of the surgical sample was able to confirm the hypothesized relationship between the nevus and the metastases, thus leading to the diagnosis of malignant melanoma metastases, genital tract. It is important to make an accurate diagnostic passage to exclude tumoral pathology in patients with atypical uterine bleeding. Every uterine bleeding of the postmenopausal period (abnormal uterine bleeding, AUB) is considered atypical and it has to be early investigated, in order to exclude any endometrial cancer. The nature of the uterine bleedings can be ascribed to atrophy, dysfunctional matters (dysfunctional uterine bleeding, DBU), benign organic alterations, only in 7-10% of cases to endometrial cancer and more rarely to metastatic tumours, as well as this case of melanoma. Physicians should be aware of such unusual possibilities in order to look carefully for metastatic implants in adenomyomas.

Epithelioid hemangioendothelioma of the larynx.

Epithelioid hemangioendothelioma is an uncommon vascular tumor of the soft tissues. Several reports have described this tumor in visceral locations such as liver, lung, and brain. To the best of our knowledge this is the first report of an immunohistochemical and ultrastructural study of a primary epithelioid hemangioendothelioma occurring in the larynx. Difficulties concerning the differential diagnosis and clinical problems arising from surgical therapy in the laryngeal area are also discussed.

Morphometric analysis of AgNORs in uveal malignant melanoma.

OBJECTIVE: To define the value of silver-stained nucleolar organizer regions (AgNOR) in the evaluation of uveal malignant melanomas (MM). STUDY DESIGN: Morphometric analysis of AgNORs was performed on 30 uveal MM of spindle A, B, epithelioid and mixed cell types. Findings were compared with cytotype, largest tumor dimension (LTD) and clinical outcome. RESULTS: AgNOR mean perimeter (MP) was 3.76 microns (SD 1.21, range 1.79-8.51 microns) in A, 5.05 microns (SD 1.96, range 1.79-16.41 microns) in B and 6.15 microns (SD 3.86, range 1.79-33.80 microns) in epithelioid MM; mean area (MA) was 0.86 micron 2 (SD 0.60, range 0.20-2.78 microns 2) in A, 1.58 microns 2 (SD 1.11, range 0.20-9.63 microns 2) in B and 2.34 microns 2 (SD 2.10, range 0.20-15.27 microns 2) in epithelioid MM; the aspect ratio (AR) was 3.10 (SD 0.19, range 2.90-3.35) in A, 5.80 (SD 2.07, range 3.77-9.32) in B and 12.22 (SD 2.84, range 8.11-15.81) in epithelioid MM. From comparing MA and RA with follow-up, it seemed that MM with a good prognosis (spindle A and a subgroup of B tumors, B1) exhibited the lowest MA and AR values, while MM with poorer clinical behavior (epithelioid and a second subgroup of spindle B MM, B2) showed the highest. No correlation was found between AgNORs and LTD. CONCLUSION: AgNORs may contribute to the prognostic evaluation of uveal MM.

An unusual case of primary systemic amyloidosis.

Here we report an unusual case of primary systemic amyloidosis. The cutaneous lesions were polymorphic and included involvement of both external auditory canals. The visceral involvement was covert. Mapping of amyloid deposits was performed using scintigraphy with technetium-99m (V) dimercaptosuccinic acid ([99mTc (V)] DMSA). Therapy with melphalan, prednisone and colchicine resulted in considerable improvement.

Warthin tumor of the lacrimal gland.

PURPOSE: To describe a case of Warthin tumor involving the lacrimal gland. METHODS: Case report. The patient underwent a lateral orbitotomy to remove a nodular lesion involving the lacrimal gland. The specimen was submitted for histopathologic examination. RESULTS: The lesion was characterized by epithelial columnar cells arranged in solid nests or lining cystic spaces containing an exudative fluid and by pronounced lymphoid infiltrate with focal follicular formation. CONCLUSIONS: To our knowledge, this is the first case of Warthin tumor involving the lacrimal gland. Management requires complete excision of the lesion.

Granular cell basal cell carcinoma of the skin. Report of a case with immunocytochemical positivity for lysozyme.

Granular cell tumors have been described in many sites throughout the body. To our knowledge, few cases have been described in the literature. We report here the sixth case, in which positivity for lysozyme was also observed for the first time. The problems of the histogenesis and differential diagnosis of this rare variant of basal cell carcinoma are also discussed.

The prognostic significance of tumor angiogenesis in nonaggressive and aggressive basal cell carcinoma of the human skin.

Basal cell carcinoma (BCC) is currently the most common cutaneous cancer found in humans. Although it generally shows a relatively benign course (BCC1), some cases of BCCs show "aggressive" behavior, rapidly infiltrating deeper structures, or metastasizing (BCC2). Until now, the traditional histological diagnostic criteria have failed to discriminate unequivocally between BCC1 and BCC2. Therefore, there is still a need to find reliable prognostic indicators that correlate with outcome and may detect patients at high risk for BCC recurrence, or metastasis and death. Recent studies have suggested that there is a significant correlation between tumor angiogenesis, expressed as the microvessel density within and toward a tumor, tumor aggressiveness, and the overall survival of patients with solid tumors. In this study, the authors examined the angiogenic rate in human cutaneous BCCs, to establish if it correlates with their biological behavior. Vessels were highlighted by immunocytochemical staining for FVIII-related antigen in formalin-fixed, paraffin-embedded tissues. All BCC2 samples of this series showed a significantly higher microvessel count than did BCC1. The existence of a significant discrepancy between the neovascularization in BCC1 and BCC2 suggests that the angiogenetic process may be an important step in the acquisition of the aggressive (malignant) phenotype in human. BCCs. The findings of the present study seem to establish a correlation between tumor vascularization and clinicobiological parameters of aggressiveness in BCC. Considering the emerging studies on the possible clinical use of substances interfering with the angiogenetic process, it is possible that the local therapy for BCCs could become less destructive, with consequent improvement in the quality of life of these patients, apart from the prolongation of the overall survival. From this view-point, the assay of microvessel density might be helpful in selecting patients with cutaneous BCCs at high risk for recurrence or metastasis, who could benefit ab initio from additional therapies and closer follow-up.

[Desmoplastic ameloblastoma. Report of a case]. / Ameloblastoma desmoplastico. Presentazione di un caso clinico.

The authors reported a case of desmoplastic variant of ameloblastoma. The patient showed a swelling in right emi-mandible in the premolar region with radiologic features of fibro-osseous lesion (mixed radiolucent-radiopaque appearance). The histological diagnosis has been the only identifications instrument.

A primary, lateral-cervical medullary thyroid carcinoma: a case report.

Medullary thyroid carcinoma (MTC) arises from the parafollicular cells of the thyroid and occurs in a sporadic or in an inherited form. We present a case of an aberrant MTC in a patient with a functioning thyroid gland. At surgical dissection, the thyroid was present in its anatomical site with a nodule in the upper one third of the right lobe. A mass was also found in a lateral-cervical position distinct from the thyroid gland. Histological examination showed the mass to be the primary MTC, whereas the thyroid nodule was a follicular adenoma. Analysis of DNA extracted from the MTC, from the adenoma, and from peripheral blood revealed a mutation within exon 16 of the RET proto-oncogene only in the DNA from the tumor. The reported case represents a sporadic MTC in an aberrant localization, probably originating from a developmental abnormality of the primordial C cells. This event might have occurred during the migration and/or differentiation of the C cells and might be related to, or caused by, the mutated RET proto-oncogene.

Histiocytosis X of the thyroid gland.

Histiocytosis X rarely involves the thyroid gland and occurs as one of the unusual manifestations of systemic diseases. Diagnosis is based on the identification of the Langerhans' cells in an appropriate background. We report a case of histiocytosis X confined to the thyroid gland that represents the primary manifestation of the disease. A careful histological, immunohistochemical and ultrastructural study has been performed and problems of differential diagnosis are discussed.

Cutaneous metastases from gastric adenocarcinoma. Report of two cases and review of the literature.

The skin is rarely involved by metastatic tumors. Breast and pulmonary carcinoma are the most frequent primary lesions that may spread to the skin. Head and neck, chest and abdominal wall are the commonest sites of cutaneous metastases. The authors report two cases of cutaneous metastases from gastric adenocarcinoma, one of which is particularly interesting as it developed on the extremities, a very rare site for skin metastasis. The authors also stress the differential diagnosis with primary cutaneous adenocarcinomas.

Primary malignant melanoma of the ovary.

Primary malignant melanoma of the ovary is an exceedingly rare pathologic event: only eight cases have been reported in literature and in every case they arised in a cystic mature teratoma. The differential diagnosis of primary malignant melanoma is a challenge for pathologist: in fact it must be differentiated by metastatic melanoma and by numerous primary ovarian tumours. We report a rare case of primary malignant melanoma of the ovary with immunohistochemical and ultrastructural study and with a review of the literature.

Primary mucinous carcinoma of the skin. Report of case.

The authors report a case of a primary mucinous carcinoma of the skin. This is a rare adnexial tumor arising from the eccrine secretory coils. The histologic characteristic finding of this lesion is the heap of abundant mucinous material (sialomucin) in the dermis in which rare epithelial islands are free-floating. In this study we also value the importance of a correct diagnosis for an appropriate therapy.

Juvenile hyaline fibromatosis. A case report of a localized form?

Juvenile hyaline fibromatosis is a rare soft-tissue, tumor-like condition that usually arises in children. We report a case characterized by localized lesions and very slow progression. We postulate the existence of two distinct forms of juvenile hyaline fibromatosis--a localized form with very slow growth and a diffuse form with large and rapidly growing tumors.

Smooth muscle tumors of the breast: clinicopathologic features of two cases.

Two cases of smooth muscle tumor of the breast with high tendency to local relapses are reported. The common cytohistologic feature of these infrequent neoplasms is interfacing bundles with spindle-shaped cells showing eosinophilic cytoplasm and blunt-ended nuclei. In estimating the prognosis of these lesions, the mitotic index must be evaluated together with evidence of necrosis and hypercellularity. The present cases suggest that, in the absence of the ancillary features, a mitotic rate of 1-3 x 10 HPF might define a "grey zone" in the spectrum of lesions between leiomyomas and leiomyosarcomas: the smooth muscle tumors of indeterminate prognosis (low risk lesions).