Primary Leiomyosarcoma of the Great Saphenous Vein: Case Report and Review of the Literature.

Soft tissue sarcomas are very rare tumors, representing less than 1% of all cancers. Leiomyosarcomas are a rare group of them representing about 6% of soft tissue sarcomas and they involve smooth muscles. Less than 2% of all leiomyosarcomas involves large blood vessels. Leiomyosarcomas of vein tunica media are very rare (1/100,000 malignant cancers) and only 10% of these originate from the great saphenous vein. In this article, we report a clinical case that occurred in our institution and review all the literature available at now.

B3 lesions of the breast and cancer risk. A single-centre experience.

BACKGROUND: Breast lesions of uncertain malignant potential, also known as B3 lesions, represent a heterogeneous group of tumors with variable malignancy risk. Surgical excision should be considered depending on clinical, radiological and histological features, family history and following informed consent. The aim of the present paper is to evaluate the positive predictive value (PPV) of diagnosis of malignancy in surgically excised B3 lesions in order to identify possible predictive upgrade criteria. We mainly focused on disclosing the concordance rate between tissue biopsy and final surgical pathology and correlation between radiology and pathology. METHODS: Between January 2018 and December 2021, 83 patients undergoing ultrasound guided tru-cut needle biopsy or VABB with a B3 diagnosis and surgical excision following multidisciplinary discussion were retrospectively reviewed in our Breast Unit. RESULTS: Out of a total of 83 cases with a B3 diagnosis before surgery, atypical ductal hyperplasia accounted for 29/83 cases(34.93%) and the most part of patients presented nodular lesions (n = 34/83, 40.96%). Among the 15 cases of malignancy detected after surgery, micro calcifications were registered in 53.3% of patients on mammography (n = 8/15). CONCLUSION: We assessed the correlation between radiological and pathological criteria in order to guide risk stratification and ensure adequate patient management. Correspondence between histological diagnosis, imaging and type of diagnostic biopsy were evaluated. No statistically significant predictors were identified for the parameters assessed in our study. KEY WORDS: B3 Breast Lesions, Lesions of Uncertains Malignant Potential, Mammografic Distortion, Screen Detected Breast Lesion.

Diffuse Leukoplakia of the Bladder Ostium-Sparing in Patient Treated with Leuprorelin for Breast Cancer.

CASE: A 55-year-old woman came to our attention in April 2020 referring haematuria, frequency and urgency. The patient referred previous treatment with leuprorelin 3.75 mg/2 ml for breast cancer three years ago. Urine culture was performed and resulted always negative for pathogens. Cystoscopy revealed a whitish plaque lesion on the fundus, dome, trigone, and left lateral wall of the bladder. Histology of the biopsy confirmed the diagnosis of leukoplakia of the bladder. The plan is to follow her up repeating a cystoscopy every three months and biopsy in 6 months. Literature search revealed very little information on pathogenesis and prognosis of this condition due to its rare occurrence. The main objective of our case study was to describe individual situation of a woman affected by diffuse leukoplakia of the bladder ostium-sparing with a previous treatment with leuprorelin 3.75 mg/2 ml for breast cancer and to show safety of follow-up by cystoscopy and biopsy. CONCLUSIONS: We showed a case of a woman treated with leuprorelin and with diffuse leukoplakia of the bladder. We support the recommended long-term follow-up and surveillance based on the literature review by cystoscopy with or without biopsy.

Metaplastic breast cancer. A case series.

Special type breast cancers display a wide range of different histological types in which clear recommendations on clinical and therapeutic management still lack and most of the information available derive from case report and case studies. In particular metaplastic breast cancer (MBC) is a rare and aggressive type of breast cancer accounting for around 1% of breast malignancies. We reported our experience in the management of five patients with MBC diagnosed and treated in our institution during the last few years (2016-2020). KEY WORDS: Metaplastic breast cancer, Special type breast cancers.

Advanced adult esthesioneuroblastoma successfully treated with cisplatin and etoposide alternated with doxorubicin, ifosfamide and vincristine.

The esthesioneuroblastoma is a rare neuroendocrine tumor that derives from the olfactory cells. In the last 20 years, around 1,000 cases have been described, with an overall survival rate of 60-70% at 5 years. The most common symptoms are nasal bleeding, nasal clogging and, in locally advanced cases, signs/symptoms of intracranic hypertension such as papilla edema, cefalea, and vomiting. The standard treatments are surgery and radiotherapy. Chemotherapy can be used in an adjuvant/neoadjuvant setting and in the metastatic phase, even if its role is still not established with certainty. Here, the case is reported of a young man (38 years old) with a locally advanced esthesioneuroblastoma. Two months before coming to our clinic, he had been treated elsewhere with debulking surgery through bilateral frontal craniotomy. After surgery, MRI showed residual disease in the nasal cavities and in the medial wall of the orbits responsible for blindness and bilateral exophthalmos within a month: a very short time. Octreoscan and whole body CT scan confirmed a locally advanced disease, in the absence of metastases. Chemotherapy was begun with cisplatin and etoposide alternated with doxorubicin, ifosfamide and vincristine with granulocyte colony-stimulating factor (G-CSF) support after every cycle. Soon after the first cycle, an important reduction of pain and decrease of the exophthalmos and vertigos was observed. No improvement in blindness was seen. The patient is still stable after 24 months of follow up.

Gastrointestinal stromal tumours and other primary gastrointestinal neoplasms. A single-center experience.

BACKGROUND: In our surgical daily activity, we report the observation of rare tumour as Gastrointestinal Stromal Tumours (GIST). We report the incidence and behaviour of new cases of GIST operated in our Center during the last decade, from 2008 to 2018 and here we also describe the concomitant observation of a second gastroenteric tumor. METHOD: We have examined all the case files and histological examinations of patients with CD 117-positive GISTs treated in our Institute from 2008 to 2018. We have gathered data regarding clinical symptoms at the time of diagnosis, tumour site, type of surgery performed, tumour size, histopathological data and follow up data. RESULTS: We have analysed 950 cases of patients who underwent surgery for gastrointestinal neoplasia in our department from 2008 to 2018. We have found 12 cases affected by GIST and in 4 cases it was also a second tumour. In two cases GIST were incidentalomas and in the others two patients a second tumour was incidentally observed in primary GIST. CONCLUSION: Patients with GIST run the risk of developing a second neoplasm, nearly twice as high as the general population with a negative impact on survival; also, incidental GIST is often observed requiring a better molecular characterization for the high risk of developing second neoplasms with the aim of achieving an early diagnosis. KEY WORDS: Gist, Second neoplasm, Surgery.

Axillary treatment of patients with breast cancer and micrometastatic disease in the sentinel lymph node Our experience.

AIM: Since the introduction of the sentinel lymph node biopsy (SLNB) in patients with breast cancer, micrometastases are detected frequently in the sln. PATIENTS AND METHODS: Between July 2005 and June 2016, 1244 patients were submitted to surgery for breast cancer. 431 patients cT1-2 N0 underwent to sentinel lymph node (SLN) and micrometastases were found in 68 of 431 screen-detected patients. Nearly all patients with both micro and macrometastases had axillary lymph node dissection (ALND). RESULTS: The SLN was negative in 69% of patients (296 of 431), 121 patients (28%) instead turned positive for lymph node metastases and in 14 patients (3%) were identified isolated tumor cells (ITC). SLN micrometastases were detected in 15,7% of patients (68 of 431). All patients with micrometastases underwent a completion ALND. In 85% of cases, therefore, the sentinel node with micrometastases was the only site of nodal disease. Neither loco-regional recurrences or distant metastases occurred in any of the Patients with sln micrometastases. DISCUSSION: There is considerable interest in foregoing axillary dissection (AD) when the sentinel node (SN) is positive in early breast cancer, particularly when axillary involvement is minimal (micrometastases or isolated tumor cells). Several trials are addressing the problem. In breast cancer patients survival is not affected by the presence of micrometastatic lymph node involvement. CONCLUSION: In our experience we always underwent to ALND all patients with micrometastases. In the light of the results we observed our attitude no longer provides for the axillary lymphadenectomy. KEY WORDS: Axillary dissection ,Breast cancer, Sentinel Lymph Node Biopsy.

ALK rearrangement in specific subtypes of lung adenocarcinoma: immunophenotypic and morphological features.

Lung adenocarcinomas are characterized by a variety of genetic and epigenetic changes that lead to activation of specific signaling pathways. This allowed the classification of lung adenocarcinomas according to genetic alterations and the clinical development of novel anticancer agents that affect the activity of specific oncoproteins. In such a context, chromosomal rearrangements that cause constitutive activation of ALK gene define a category of lung adenocarcinomas that is amenable to targeted therapy with ALK inhibitors. Thus, a major issue of current research is to define the morphological and immunophenotypic features of lung ALK-rearranged adenocarcinomas to improve the selection of tumors suitable for molecular genotyping. ALK status was determined, by immunohistochemistry and fluorescence in situ hybridization, in 94 surgically resected lung adenocarcinomas and correlated with histomorphological parameters. Indeed, ALK rearrangement was observed in 10/94 (11%) lung adenocarcinomas and enriched in tumors with a predominant mucinous (46%; p < 0.05) and solid (29%; p < 0.05) pattern. By contrast, it was lacking or sporadically observed in lung adenocarcinomas with predominant acinar, papillary or lepidic pattern. Moreover, the presence of signet-ring cells was predominantly observed in ALK-rearranged tumors (47%; p < 0.05). These data suggest that ALK rearrangement is associated with specific and distinct clinical-pathological characters compared to other genotypes. Thus, the knowledge of these characteristics can improve the diagnostic accuracy and lead to a better understanding of the behavior of ALK-rearranged NSCLC.

Validation of vacuum-based refrigerated system for biobanking tissue preservation: analysis of cellular morphology, protein stability, and RNA quality.

Biobanks of fresh, unfixed human normal and malignant tissues represent a valuable source for gene expression analysis in translational cancer research and molecular pathology. However, the success of molecular and cellular analysis in both clinical and translational research is strongly dependent on the collection, handling, storage, and quality control of fresh human tissue samples. The aim of this study was to evaluate an innovative vacuum-based refrigerated system, as a logistically feasible technology to increase the collection of tissue specimens, preserving the integrity of cellular and molecular components. We tested randomly-selected tissues stored under vacuum at 4°C by using endpoints important for research and diagnosis, including tissue morphology, epitope stability, and RNA integrity. Gene expression was evaluated by qualitative and quantitative RT analysis of selected housekeeping and tissue-specific genes. Tissue morphology and overall protein stability were generally well preserved, being compromised only in gallbladder tissue. By contrast, phosphoprotein and RNA analysis demonstrated a time-dependent degree of degradation, with progressive loss of stability from 24 to 72 hours. However, this reduction in RNA quality did not represent a limitation for successful expression analysis of selected genes. Indeed, a comparative qualitative and quantitative RT-PCR analysis showed that RNA extracted from tissues stored under vacuum is suitable for gene expression profiling, but requires highly sensitive technologies, such as quantitative RT-PCR. These data suggest that the refrigerated vacuum-based system represents a suitable and feasible technology for routine transport of fresh specimens from surgery to biobanks, thus increasing the opportunity to collect biospecimens.

An urologic face of chronic lymphocytic leukemia: sequential prostatic and penis localization.

We report a patient with chronic lymphocytic leukemia (CLL) in whom a leukemic involvement of prostate and penis occurred in the advanced phase of his disease. Obstructive urinary symptoms were indicative of prostatic CLL infiltration, followed by the occurrence of an ulcerative lesion on the glans. Histologic examination confirmed the neoplastic B-cell infiltration. Both localizations responded to conventional treatments. A review of the literature confirms that leukemic involvement of the genito-urinary system is uncommon in CLL patients. However, it should be considered in CLL patients with urologic symptoms and a long history of the disease.

Mast cell sarcoma of the scalp: the first sign of undisclosed systemic mastocytosis?

Mastocytosis is a neoplastic disease of mast cells and their CD34+ precursors, including a heterogeneous group of disorders. It is characterized by abnormal growth and accumulation of mast cells in one or more organ systems. Mast cell sarcoma is an extremely rare and aggressive disease characterized by local proliferation of atypical mast cells, destructive growth and poor prognosis, without systemic involvement. Very few clinical cases describing this entity have been reported in the literature. In this paper, we report a case of a mast cell sarcoma, localized in the scalp of a 63-year-old woman; it appears to be the first manifestation of undisclosed systemic mastocytosis.