Parathyroid Retrospective Analysis of Neoplasms Incidence (pTRANI Study): An Italian Multicenter Study on Parathyroid Carcinoma and Atypical Parathyroid Tumour

Background: Parathyroid cancer (PC) is a rare sporadic or hereditary malignancy whose histologic features were redefined with the 2022 WHO classification. A total of 24 Italian institutions designed this multicenter study to specify PC incidence, describe its clinical, functional, and imaging characteristics and improve its differentiation from the atypical parathyroid tumour (APT). Methods: All relevant information was collected about PC and APT patients treated between 2009 and 2021. Results: Among 8361 parathyroidectomies, 351 patients (mean age 59.0 ± 14.5; F = 210, 59.8%) were divided into the APT (n = 226, 2.8%) and PC group (n = 125, 1.5%). PC showed significantly higher rates (p < 0.05) of bone involvement, abdominal, and neurological symptoms than APT (48.8% vs. 35.0%, 17.6% vs. 7.1%, 13.6% vs. 5.3%, respectively). Ultrasound (US) diameter >3 cm (30.9% vs. 19.3%, p = 0.049) was significantly more common in the PC. A significantly higher frequency of local recurrences was observed in the PC (8.0% vs. 2.7%, p = 0.022). Mortality due to consequences of cancer or uncontrolled hyperparathyroidism was 3.3%. Conclusions: Symptomatic hyperparathyroidism, high PTH and albumin-corrected serum calcium values, and a US diameter >3 cm may be considered features differentiating PC from APT. 2022 WHO criteria did not impact the diagnosis

Open necrosectomy is feasible as a last resort in selected cases with infected pancreatic necrosis: a case series and systematic literature review

Acute pancreatitis is a common inflammatory pancreatic disorder, often caused by gallstone disease and frequently requiring hospitalization. In 80% of cases, a rapid and favourable outcome is described, while a necrosis of pancreatic parenchyma or extra-pancreatic tissues is reported in 10-20% of patients. The onset of pancreatic necrosis determines a significant increase of early organ failure rate and death that has higher incidence if infection of pancreatic necrosis (IPN) or extra-pancreatic collections occur. IPN always requires an invasive intervention, and, in the last decade, the advent of minimally invasive techniques has gradually replaced the employment of the open traditional approach. We report a series of three severe cases of IPN managed with primary open necrosectomy (ON) and a systematic review of the literature, in order to understand if emergency surgery still has a role in the current clinical practice

Parathyroid carcinoma in multiple endocrine neoplasm type 1 syndrome: case report and systematic literature review.

The aim of this report was to illustrate a case of parathyroid carcinoma (PC) in a patient with multiple endocrine neoplasia type 1 (MEN1) along with a comprehensive literature review. A 61-year-old man presented with 9-cm PC causing primary hyperparathyroidism (PHPT). His pre-operative corrected calcium and intact PTH serum levels were 2.92 mmol/L and 391.7 pg/mL, respectively. The neoplastic gland was removed in bloc with thyroid and central compartment lymph nodes. A literature review was run by searching PubMed MEDLINE from 1977 to 2018 for studies of all types, in the English language only, using the terms “Parathyroid, carcinoma, Multiple endocrine neoplasia, type 1, (MEN1).” Pathology confirmed PC. Post-operative calcium and PTH levels were normal. A diagnosis of MEN1 was established post-operatively. Seventeen cases of PC in patients with MEN1 have been reported in the literature. 59% of patients were men, and median age at diagnosis was 50 years, with median serum PTH of 379 pg/mL and median serum calcium level of 3.2 mmol/L. The occurrence of PC in the context of MEN1 is extremely rare. Diagnosis and treatment may represent a challenge, so opportune identification or suspicion of malignancy and adoption of correct surgical approach may offer affected patients the best outcome

Management of Mirizzi Syndrome in Emergency.

Introduction: Mirizzi syndrome (MS) is a rare complication of cholelithiasis. Despite the success of laparoscopic cholecystectomy as a minimally invasive approach to gallstone disease, MS remains a challenge, also for open and robotic approaches, due to the subverted anatomy of the hepatocystic triangle. Moreover, when emergency surgery is needed, the optimal preoperative diagnostic assessment could not be always achievable. We aim to analyze our experience of MS treated in emergency and to assess the feasibility of a diagnostic and therapeutic decisional algorithm. Methods: From March 2006 to February 2016, all patients with a preoperative diagnosis, or an intraoperative evidence of MS, were retrospectively analyzed at our Academic Hospital, including patients operated on in emergency or in deferred urgency. Eighteen patients were included in the study using exclusion criteria and were treated in elective surgery. Results: The patients were distributed according to modified Csendes' classification: type I in 15 cases, type II in 2, type III in 0, type IV in 1, and type V in 0. In the type I group, diagnosis was intraoperatively performed. Laparoscopic approach was performed with cholecystectomy or subtotal cholecystectomy, when the hepatocystic triangle dissection was hazardous. Patients with preoperative diagnosis of acute abdomen and MS type IV were directly managed by open approach. Conclusions: Diagnosis of MS and the therapeutic management of MS are still a challenge, mostly in an emergency setting. Waiting for standardized guidelines, we propose a decisional algorithm in emergency, especially in nonspecialized centeres of hepatobiliary surgery

Severe anemia in a patient with vulvar melanoma

We report the case of a 78-year-old woman with a history of surgical excision of vulvar melanoma (VM; stage IV c-Kit ex-17 mutated) who developed lung and nodal metastases, achieving 9-month complete response from anti-PD1 MoAb. She gave written consent for this publication; ethics committee approval was waived because of the case report format. Subsequently, the onset of fatigue associated with severe anemia (up to Hb 7.5 g/dL) occurred. Gastroscopy, colonoscopy, and hematologic investigations, such as bone marrow biopsy, were negative. The computed tomography scan showed an unspecific dilatation of the small bowel loops without air fluid levels. Based on these findings, she underwent video capsule endoscopy, showing an irregular, hyperemic, bloody, and pigmented ulcer of the first jejunal loop

Introducing routine intraoperative nerve monitoring in a high-volume endocrine surgery centre: a health technology assessment

We sought to analyse the effect of the introduction of intraoperative nerve monitoring (IONM) in our routine surgical practice and to provide a circumstantial analysis of direct costs of IONM in total thyroidectomy and of indirect costs associated with vocal fold palsy, as centred in the health care system of Italy. We retrospectively compared outcomes of 232 total thyroidectomies performed between November 2017 and October 2019, respectively, before (109 TT-Group A) and after (123 TT-Group B) adopting IONM technology in November 2018. We analysed the costs of IONM per procedure and rate and costs of vocal fold palsy events (temporary and permanent). Overall, there were 61 thyroid cancers (32 in Group B) and 171 multinodular goitres (91 in Group B). We recorded 5 cases of vocal fold palsy (4.6%-4 transient, 1 permanent) in Group A and none in Group B (p = 0.016). IONM consumables cost 219 eur per case. Healthcare and social cost of Vocal fold palsy ranged between 3200 eur (function recovery < 1 month postoperatively) and over 32,000 eur (permanent event). When only direct costs are considered, IONM can hardly be cost effective. In this study, cost of IONM implementation was offset by the absence of complications attributable to recurrent laryngeal nerve dysfunction

Intraabdominal sporadic desmoid tumors and inflammation: an updated literature review and presentation and insights on pathogenesis of synchronous sporadic mesenteric desmoid tumors occurring after surgery for necrotizing pancreatitis

Sporadic intra-abdominal desmoid tumors are rare and known to potentially occur after trauma including previous surgery, although knowledge of the underlying pathogenetic mechanism is still limited. We reviewed the recent literature on sporadic intraabdominal desmoids and inflammation as we investigated the mutational and epigenetic makeup of a case of multiple synchronous mesenterial desmoids occurring after necrotizing pancreatitis. A 62-year-old man had four mesenteric masses up to 4.8 cm diameter detected on CT eighteen months after laparotomy for peripancreatic collections from necrotizing pancreatitis. All tumors were excised and diagnosed as mesenteric desmoids. DNA from peripheral blood was tested for a multigene panel. The tumour DNA was screened for three most frequent beta-catenin gene mutations T41A, S45F and S45P. Expression levels of miR-21-3p and miR-197-3-p were compared between the desmoid tumors and other wild-type sporadic desmoids. The T41A CTNNB1 mutation was present in all four desmoid tumors. miR-21-3p and miR-197-3p were respectively upregulated and down-regulated in the mutated sporadic mesenteric desmoids, with respect to wild-type lesions. The patient is free from recurrence 34 months post-surgery. The literature review did not show similar studies. To our knowledge, this is the first study to interrogate genetic and epigenetic signature of multiple intraabdominal desmoids to investigate potential association with abdominal inflammation following surgery for necrotizing pancreatitis. We found mutational and epigenetic features that hint at potential activation of inflammation pathways within the desmoid tumor

Prognostic and Therapeutic Role of Angiogenic Microenvironment in Thyroid Cancer

Thyroid cancer is the most common endocrine malignancy, with a typically favorable prognosis following standard treatments, such as surgical resection and radioiodine therapy. A subset of thyroid cancers progress to refractory/metastatic disease. Understanding how the tumor microenvironment is transformed into an angiogenic microenvironment has a role of primary importance in the aggressive behavior of these neoplasms. During tumor growth and progression, angiogenesis represents a deregulated biological process, and the angiogenic switch, characterized by the formation of new vessels, induces tumor cell proliferation, local invasion, and hematogenous metastases. This evidence has propelled the scientific community’s effort to study a number of molecular pathways (proliferation, cell cycle control, and angiogenic processes), identifying mediators that may represent viable targets for new anticancer treatments. Herein, we sought to review angiogenesis in thyroid cancer and the potential role of proangiogenic cytokines for risk stratification of patients. We also present the current status of treatment of advanced differentiated, medullary, and poorly differentiated thyroid cancers with multiple tyrosine kinase inhibitors, based on the rationale of angiogenesis as a potential therapeutic target

Rare gastrointestinal metastases from primary lung cancer. Two case reports of patients managed with emergency surgery

Background: Lung cancer represents the second leading cause of death in US and the high mortality rate could be related to the diagnosis, usually, performed in an advanced metastatic stage. Gastrointestinal metastases from non-small cell lung cancer, are extremely rare and only few cases complicated by digestive haemorrhage and/or perforation have been reported in literature. Material and methods: We report two cases of gastrointestinal metastases and their rare clinical onset with haemorrhage and perforation of the digestive tract. Both patients were admitted in an emergency setting. The first case was a 59-year-old man complained of abdominal pain and massive gastrointestinal haemorrhage. An upper gastrointestinal endoscopy revealed an ulcerated gastric mass and an emergency CT-scans showed a right lung mass with biopsy conclusive for a large cell lung cancer. The second case was a 62-year-old man with abdominal pain and shock due to gastrointestinal bleeding. He was submitted to an emergency CT-scan showing two lung nodules (1.0 and 3.5 cm of diameter) as well as widespread metastases, intraperitoneal free air and fluids. Results: Both patients were surgical managed in emergency and pathology revealed the metastatic origin from an unknown large-cell lung cancer and a rare lung adenocarcinoma in the second one. Conclusions: Despite the rare clinical condition, in patients with a diagnosis of lung cancer managed in emergency for gastrointestinal complication, gastrointestinal metastases should be taken into account, and referred to the primary disease in order to tailor the best approach. Key words: Gastrointestinal metastases, Lung cancer, Tailored surgery

Current concepts in parathyroid carcinoma: a single Centre experience

BackgroundParathyroid carcinoma is a rare neoplasm that may present sporadically or in the context of a genetic syndrome. Diagnosis and management are challenging due to the lack of clinical and pathological features that may reliably distinguish malignant from benign disease.MethodsFrom January 2013 to December 2017, from 358 consecutive patients affected by parathyroid diseases, 3 patients with parathyroid carcinoma were treated at our academic Department of General Surgery. We present our experience as illustrative of the different features of clinical presentation of parathyroid carcinoma and review its management considering the recent relevant literature.ResultsCase 1: A 62-year-old man was hospitalized for left-sided palpable neck mass, hypercalcemia and elevated PTH. US-guided FNA was suspect for parathyroid carcinoma. A large cystic mass was excised in bloc with total thyroidectomy and central neck dissection. Genetic studies framed a pathologically confirmed parathyroid carcinoma within MEN1 syndrome. Case 2: A 48-year-old woman with hypothyroidism had total thyroidectomy performed for a suspect for right follicular thyroid lesion. Pathology revealed parathyroid carcinoma. Case 3: A 47year-old man was admitted for hypercalcaemic crisis and renal failure in the context of PHPT. A lesion suggestive on US and MIBI scan for parathyroid adenoma in the right lower position was removed by mini-invasive approach. Pathology revealed parathyroid cancer and patient had completion hemythyroidectomy and central neck dissection.ConclusionParathyroid cancer is a particularly rare endocrine malignancy, however it should be suspected in patients with primary hyperparathyroidism when severe hypercalcemia is associated to cervical mass, renal and skeletal disease. Parathyroid surgery remains the mainstay of treatment. Radical tumour resection and expedited treatment in a dedicated endocrine Center represent crucial prognostic factors