Sinonasal Adenocarcinoma: Clinicopathological Characterization and Prognostic Factors.

Sinonasal (SN) malignancies are rare. Within SN adenocarcinomas, the most frequent are intestinal-type adenocarcinomas (ITACs). ITAC has been associated with wood and leather dust occupational exposure and TP53 mutations. Not much information is available regarding its characterization and treatment. The aim of this study is to characterize the clinicopathologic and prognostic factors of patients with sinonasal adenocarcinomas (SNACs) treated in our tertiary-level hospital. A retrospective, consecutive study including SNAC patients diagnosed between 2004-2023 was conducted. Clinicopathological data was collected, and p53 status was assessed in the tumor specimens. The association between p53 status and clinicopathological variables, as well as their impact on survival, was evaluated. In total, 35 were included, most of them having ITAC (91.4%) with papillary subtype (37.5%); the majority were subjected to occupational risk exposure (82.9%). Overexpression of p53 was identified in 48.6% of the tumors. Papillary and colonic subtypes were associated with higher median progression-free survival (mPFS) than mucinous and solid subtypes (mPFS 37 months, 95% CI, 20.0-54.0, vs. 9 months, 95% CI, 7.15-10.85, p=0.01); the former was also associated with higher median overall survival (mOS) (mOS 64 months, 95% CI, 37.18-90.81 vs. 14 months, 95% CI, 0-41.58, p=0.02). Histologic grade 1-2 and macroscopic complete resection were associated with higher PFS (PFS of five months of 90.9% vs. 33.3%, p=0.01; mPFS of 37 months, 95% CI, 4.93-69.07 vs. 10 months, 95% CI, 6.43-13.57, p=0.04, respectively). Disease recurrence with distant metastases was associated with lower OS (11 months, 95% CI, 6.1-15.9 vs. 53 months, 95% CI, 22.70-83.30, p=0.04). This study reinforces the importance of protective occupational measures. Future studies will be important to validate the best treatment strategy in the advanced stages of this disease and also to identify new prognostic and/or therapeutic target biomarkers in SNAC.

Baseline anaemia increases locally advanced rectal cancer mortality in older patients undergoing preoperative chemoradiation.

PURPOSE: The median diagnosis age of rectal cancer (RC) is 70 years old. The standard of care for locally advanced RC (LARC) is preoperative chemoradiation (CRT) followed by surgery. Anaemia is a frequent condition in older patients but is not a pure consequence of ageing. METHODS: The patients aged 65 years or over, with clinical stage II/III LARC, and treated with preoperative concurrent CRT were retrospectively reviewed. Baseline haemoglobin (Hb) levels were collected. RESULTS: One hundred and seven patients enrolled in this study, but 17 were excluded in relation with treatment disruption. Fifty-seven (63.3%) males and 33 (36.7%) females completed preoperative CRT whose median age at diagnosis was 73. Twenty-five (27.8%) patients presented with anaemia at rectal cancer diagnosis, and median Hb was 13.5 g/dL (IQR = 1.45) and 11.2 g/dL (IQR = 1.35), for non-anaemic and anaemic patients, respectively. For the enrolled older population, only 2 patients reported acute grade 3 toxicity. Baseline anaemia tended to decrease the LARC-free interval and was associated with a significantly higher hazard of all-cause and LARC mortality, approximately 5 times (HR = 5.25; 95% CI 1.48-18.66) and 10 times (HR = 10.09; 95% CI 2.40-42.48), respectively. Patients older than 75 presented a significantly negative impact on overall survival (OS) and LARC-specific survival (HR = 6.20, 95% CI 2.00-19.22; and HR = 7.61, 95% CI 2.08-27.87, respectively). Conversely, no significant impact was found for age-adjusted Charlson comorbidity index on OS, LARC-specific survival and LARC-free interval. CONCLUSIONS: Overall and LARC-specific survival were significantly lower for the baseline anaemic older patients and for those aged 75 years or over.

Ectopic Cushing's Syndrome Unveiling a Metastatic Parotid Carcinoma.

INTRODUCTION: Adrenocorticotropic hormone (ACTH) ectopic production is a rare cause of Cushing syndrome (CS). The most commonly associated tumours are small-cell lung carcinoma along with bronchial and thymic carcinoids. To date, only 5 cases have been published in the literature featuring ectopic ACTH secretion from metastatic acinic cell carcinoma (ACC) of the parotid gland. We hereby describe a very uncommon case of ectopic CS (ECS) unveiling a metastatic parotid ACC. CASE PRESENTATION: A 46-year-old man with hypertension and dyslipidemia diagnosed 4-months before, as well as new-onset diabetes mellitus unveiled 1-month earlier, was referred to emergency department for hypokalemia. Hormonal study and dynamic biochemical tests performed indicated ECS. Imaging and cytological findings pointed toward a likely primary right parotid malignancy with liver metastases. Somatostatin receptor scintigraphy has shown an increased uptake in the parotid gland and mild expression in liver metastasis. The patient underwent right parotidectomy, and histopathologic examination confirmed ACC. Meanwhile, hypercortisolism was managed with metyrapone, ketoconazole, and lanreotide. Despite chemotherapy onset, a rapid disease progression and clinical course deterioration was observed. CONCLUSION: The present report highlights a rare ECS, exposing a metastatic parotid ACC, with an aggressive and challenging clinical course, representing the first case whose diagnosis of ECS came prior to ACC.

Large desmoid tumors in familial adenomatous polyposis: a successful outcome

Desmoid tumors develop from connective tissue, fasciae, and aponeuroses, and may occur in the context of familial adenomatous polyposis or may arise sporadically; also, they may be extra-abdominal, intra-abdominal, or located in the abdominal wall. These benign tumors have a great aggressiveness with a high rate of local recurrence. Familial adenomatous polyposis is an inherited condition with autosomal dominant transmission, and is characterized by the development of multiple colonic and rectal adenomatous polyps, as well as desmoid tumors. We present the case of a 54-year-old woman with germline APC gene mutation, who underwent a total colectomy, subsequently developing two large infiltrative solid intra-abdominal lesions consistent with desmoid tumors. Medical treatment with Cox-2 inhibitors was initiated without result. She was submitted to resection for intestinal obstruction, but developed local recurrence. The lesions were also unresponsive to tamoxifen, and chemotherapy was initiated with dacarbazine plus doxorubicin, switching to vinorelbine plus methotrexate, achieving a good response in all lesions after 12 months. The approach to these intra-abdominal lesions should be progressive, beginning with observation, then a medical approach with non-steroidal anti-inflammatory drugs or with an anti-hormonal agent. Afterwards, if progression is still evident, chemotherapy should be started. Surgery should be reserved for resistance to medical treatment, in palliative situations, or for extra-abdominal or abdominal wall desmoids tumors.

Treatment of Head and Neck Cancer with Photodynamic Therapy with Redaporfin: A Clinical Case Report.

Advanced head and neck squamous cell carcinoma, after locoregional treatment and multiple lines of systemic therapies, represents a great challenge to overcome acquired resistance. The present clinical case illustrates a successful treatment option and is the first to describe the use of photodynamic therapy (PDT) with Redaporfin, followed by immune checkpoint inhibition with an anti-PD1 antibody. This patient presented an extensive tumor in the mouth pavement progressing after surgery, radiotherapy, and multiple lines of systemic treatment. PDT with Redaporfin achieved the destruction of all visible tumor, and the sequential use of an immune checkpoint inhibitor allowed a sustained complete response. This case is an example of the effect of this therapeutic combination and may provide the basis for a new treatment modality.

Large desmoid tumors in familial adenomatous polyposis: a successful outcome.

Desmoid tumors develop from connective tissue, fasciae, and aponeuroses, and may occur in the context of familial adenomatous polyposis or may arise sporadically; also, they may be extra-abdominal, intra-abdominal, or located in the abdominal wall. These benign tumors have a great aggressiveness with a high rate of local recurrence. Familial adenomatous polyposis is an inherited condition with autosomal dominant transmission, and is characterized by the development of multiple colonic and rectal adenomatous polyps, as well as desmoid tumors. We present the case of a 54-year-old woman with germline APC gene mutation, who underwent a total colectomy, subsequently developing two large infiltrative solid intra-abdominal lesions consistent with desmoid tumors. Medical treatment with Cox-2 inhibitors was initiated without result. She was submitted to resection for intestinal obstruction, but developed local recurrence. The lesions were also unresponsive to tamoxifen, and chemotherapy was initiated with dacarbazine plus doxorubicin, switching to vinorelbine plus methotrexate, achieving a good response in all lesions after 12 months. The approach to these intra-abdominal lesions should be progressive, beginning with observation, then a medical approach with non-steroidal anti-inflammatory drugs or with an anti-hormonal agent. Afterwards, if progression is still evident, chemotherapy should be started. Surgery should be reserved for resistance to medical treatment, in palliative situations, or for extra-abdominal or abdominal wall desmoids tumors.

Small cell neuroendocrine carcinoma of the nasopharynx: a rare case report.

Small cell neuroendocrine carcinoma rarely appears primarily in the head and neck and exhibits aggressive behavior with a poor prognosis. The pathologist has a significant role in the diagnosis, and a consensual treatment still does not exist. The authors report the case of a middle-aged male patient who presented repeated episodes of massive epistaxis. The diagnostic work-up disclosed the diagnosis of small cell neuroendocrine carcinoma of the nasopharynx. The patient was treated with chemotherapy followed by radiotherapy. Imaging examinations performed after the end of treatment showed apparent complete remission of the disease. The patient was kept under active surveillance with no signs of local relapse or distant metastasis after 4 years of follow-up.

Small cell neuroendocrine carcinoma of the nasopharynx: a rare case report

Small cell neuroendocrine carcinoma rarely appears primarily in the head and neck and exhibits aggressive behavior with a poor prognosis. The pathologist has a significant role in the diagnosis, and a consensual treatment still does not exist. The authors report the case of a middle-aged male patient who presented repeated episodes of massive epistaxis. The diagnostic work-up disclosed the diagnosis of small cell neuroendocrine carcinoma of the nasopharynx. The patient was treated with chemotherapy followed by radiotherapy. Imaging examinations performed after the end of treatment showed apparent complete remission of the disease. The patient was kept under active surveillance with no signs of local relapse or distant metastasis after 4 years of follow-up.

Colon stenosis in a patient with ulcerative colitis as a manifestation of mixed müllerian tumor of the peritoneum.

We report the case of a 56-year-old woman followed for ulcerative colitis since 1987. In 1993, she underwent a total hysterectomy with bilateral salpingo-oophorectomy. In 1996, a narrowing of the lumen at 20 cm of the anal margin was observed and she was put under endoscopic observation. In 1998, a mass in the stricture was observed, with significant narrowing of the lumen. Histologic examination revealed a biphasic tumor with features of malignant mixed müllerian tumor. Five years after surgery and chemotherapy with paclitaxel and carboplatinum, the patient remains well.