Management and surgical treatment of parathyroid carcinoma: a 6-year experience of a single centre of endocrine surgery unit.

Background: Parathyroid carcinoma (PC) affects 0.1-0.3% of the general population and represents the rarest malignant neoplasms among endocrinological diseases, comprising less than 1%. The best therapeutic treatment and management methods are still debated in the literature. The aim of this study is to evaluate the management and surgical treatment of parathyroid carcinoma after 6 years of enrolment with the Endocrine Surgery Unit of the University Hospital of Bari. Materials and methods: A retrospective observational study was carried out using a prospectively maintained database of patients affected by primary hyperparathyroidism between January 2017 and September 2022. Consecutive patients over 18 years old with a final histopathological finding of PC were included in the study. Patients with secondary or tertiary hyperparathyroidism, parathyroid hyperplasia, and parathyroid adenoma were excluded. All patients underwent follow-up every 6 months for the first 2 years, and annually thereafter. Results: In this study, 9 out of 40 patients affected by hyperparathyroidism were included; 6 (66.6%) were female and 3 (33.3%) were male patients, with a median age of 59 years (IQR 46-62). None had a family history of PC. No mortality was recorded while the incidence of recurrence was 22.2%, with a disease-free survival of 8 and 10 months. Parathyroidectomy was performed in five patients, while four patients underwent parathyroidectomy with concurrent thyroidectomy for thyroid goitre. No intraoperative complications were recorded. Open parathyroidectomy was performed with a mini-cervicotomy in seven patients, while two patients underwent robotic surgery. All patients were discharged on the second postoperative day. Conclusion: PC represents a great challenge in terms of preoperative diagnosis, management and treatment. A surgical approach represents the first best option for PC in referral endocrine surgery units. The early identification of risky patients should be the dominant goal to plan an appropriate therapy and to perform adequate en bloc surgery.

The Italian Unitary Society of Colon-proctology (SIUCP: Società Italiana Unitaria di Colonproctologia) guidelines for the management of anal fissure.

INTRODUCTION: The aim of these evidence-based guidelines is to present a consensus position from members of the Italian Unitary Society of Colon-Proctology (SIUCP: Società Italiana Unitaria di Colon-Proctologia) on the diagnosis and management of anal fissure, with the purpose to guide every physician in the choice of the best treatment option, according with the available literature. METHODS: A panel of experts was designed and charged by the Board of the SIUCP to develop key-questions on the main topics covering the management of anal fissure and to performe an accurate search on each topic in different databanks, in order to provide evidence-based answers to the questions and to summarize them in statements. All the clinical questions were discussed by the expert panel in different rounds through the Delphi approach and, for each statement, a consensus among the experts was reached. The questions were created according to the PICO criteria, and the statements developed adopting the GRADE methodology. CONCLUSIONS: In patients with acute anal fissure the medical therapy with dietary and behavioral norms is indicated. In the chronic phase of disease, the conservative treatment with topical 0.3% nifedipine plus 1.5% lidocaine or nitrates may represent the first-line therapy, eventually associated with ointments with film-forming, anti-inflammatory and healing properties such as Propionibacterium extract gel. In case of first-line treatment failure, the surgical strategy (internal sphincterotomy or fissurectomy with flap), may be guided by the clinical findings, eventually supported by endoanal ultrasound and anal manometry.

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.

Results of sclerotherapy and mucopexy with haemorrhoidal dearterialization in II and III degree haemorrhoids. A 4 years' single centre experience.

Introduction: Haemorrhoidal disease (HD) affects a considerable portion of the adult population. The aim of this study is to confirm the safety and efficacy of the treatments and to report the long-term outcomes of Sclerotherapy (ST) and Mucopexy and Haemorrhoidal Dearterialization (MHD) performed over the last 4 years in a single tertiary centre. The secondary outcome is to evaluate the usefulness of both techniques and to demonstrate how those can be associated as a bridge to surgery. Materials and methods: Patients affected by second-third-degree haemorrhoids and undergoing ST or non-Doppler guided MHD between 2018 and 2021 were enrolled. Safety and efficacy, recurrence rate, Haemorrhoid Severity Score (HSS) and pain resulting from both techniques were evaluated. Results: Out of 259 patients, 150 underwent ST. Further, 122 (81.3%) patients were male and 28 (18.7%) were female. The mean age was 50.8 (range 34-68) years. Most of the patients (103, 68.6%) were affected by second-degree HD, while 47 (31.4%) were affected by third-degree HD. The overall success rate was 83.3%. The median pre-operative HSS score was 3 (IQR 0-4, p = 0.04) and at 2 year the median HSS was 0 (IQR 0-1, p = 0.03). No intraoperative complications and no drug-related side effects occurred. The mean follow-up for ST was 2 years (range 1-4; SD ±0.88). MHD was performed on 109 patients. In detail, 80 patients (73.4%) were male while 29 patients (26.6%) were female. The mean age in this group was 51.3 (range 31-69). Further, 72 patients (66.1%) were affected by third-degree HD and 37 (33.9%) by second-degree HD. The median HSS score was 9 (IQR 8-10, p = 0.001) preoperatively two years after treatment was 0 (IQR 0-1, p = 0.004). Major complications occurred in three patients (2.75%). The overall success rate was 93.5% (second degree 89.2% vs. third degree 95.8%). The mean follow-up for MHD was 2 years (range 1-4; SD ±0.68). Conclusions: The results confirm the usefulness of those techniques, which can be considered safe and easily repeatable procedures, with a low recurrence rate after 2 years of median follow-up.

Laparo-endoscopic management of chole-choledocholithiasis: Rendezvous or intraoperative ERCP? A single tertiary care center experience.

Introduction: The management of chole-choledocholithiasis remains a matter of debate to preserve minimal invasive management and different options have been proposed, with single- or two-stage approaches. Two techniques of single-stage approach are intraoperative ERCP and laparoscopic rendezvous, which have the great advantage of reducing the length of hospital stay with increased patient compliance. This retrospective study aims to evaluate and compare the efficacy and safety of intraoperative ERCP and rendezvous technique for more than 15 years. Materials and methods: Clinical records of 113 patients who underwent single-stage management for chole-choledocholithiasis between January 2003 and December 2020 were retrospectively reviewed using a prospectively maintained database. Patients were separated into two groups: those managed with intraoperative ERCP and those with rendezvous, and their intraoperative and postoperative parameters were compared. All patients were followed up for 6 months in an outpatient setting. Results: A total of 68 (60%) patients were treated with intraoperative ERCP, while the remaining 45 (40%) were treated with rendezvous. There were no significant differences in terms of comorbidities. ERCP was performed with a median operative time of 145 min (104-168) and an endoscopic time of 27 min (15-36). Meanwhile, rendezvous was performed with a significantly lower operative [120 min (94-147)] and endoscopic time [15 min (12-22)]. No intraoperative complications were recorded. Patients treated with rendezvous had a significantly shorter median hospitality stay [4 (3-5) vs. 3 (2-4) days, p < 0.05]. No hospital readmissions or mortalities were observed in either group after 30 days. Ten mild pancreatitis cases were observed, mainly in the intraoperative ERCP group (9 vs. 1, p < 0.05), and all were treated conservatively. Only two patients treated with intraoperative ERCP developed biliary complications later on. Conclusion: Laparoscopic rendezvous should be considered a preferable alternative to intraoperative ERCP for the treatment of patients with concomitant CBD stones and gallstones.

201 cases of foreign body ingestion in a Surgical Emergency Unit A 16-year retrospective study.

AIM: The aim of this study is to analyse clinical characteristics of FB ingestion and predictive factors for complications, in order to reduce mortality and morbidity. MATERIALS AND METHODS: A retrospective study of emergency surgical consultation records has been carried out from June 2005 through June 2015 yielded 201 episodes with the diagnosis of ingestion of foreign objects at the Surgical Unit of the University of Bari. RESULTS: Natural Removal in 44,8% of cases; Endoscopic retrieval in 42,4%, Surgical Procedures 4,4%. Statistical analysis was based on multivariate analysis and the model R2 of the Naegelkerke value. DISCUSSION: First of all, the approach to ingestion should be endoscopic. The second approach is surgical in selected cases. The most frequent site of impaction were oesophagus, stomach and right colon. An EGD proved to be the most used procedure with a no morbidity and no mortality. CONCLUSION: The ingestion of foreign bodies is a frequent, complex and expensive condition to treat. Observation and endoscopy are the most appropriate procedures to be considered to manage the ingestion of FB in Emergency Surgery Unit. KEY WORDS: Emergency surgery, Foreign bodies, Ingestion.

Immunity Profiling of COVID-19 Infection, Dynamic Variations of Lymphocyte Subsets, a Comparative Analysis on Four Different Groups.

BACKGROUND: A novel coronavirus (SARS-CoV-2)-induced pneumonia (COVID-19) emerged in December 2019 in China, spreading worldwide. The aim of the present investigation was to evaluate the immunological response and the clinical subset of peripheral lymphocyte subset alteration in COVID-19 infection. METHODS: the study was conducted on four different clinical groups (n = 4; total n = 138). Each individual was assigned to different groups based on specific criteria evaluated at the admission such as fever, dyspnea, arterial blood gas analysis (ABG), oral-nasopharyngeal swab/RT-PCR, and thoracic CT-scan. Treatment was performed only after blood samples were collected from each patient (PP and PP) at day 1. The blood samples were analyzed and tested the same day (CBC and Flowcytometry). The positive-positive group (PP n = 45; F = 18/ M = 27; median age = 62.33), comprised individuals affected by COVID-19 who showed fever, dyspnea (ABG = pO2 < 60), confirmed positive by oral-nasopharyngeal swab/RT-PCR and with CT-scan showing ground-glass opacities. The negative-positive (NP; n = 37; F = 11/M = 26; median age = 75.94) or "COVID-like" group comprised individuals with fever and dyspnea (ABG = pO2 < 60), who tested negative to nasopharyngeal swab/RT-PCR, with CT-scans showing ground-glass opacities in the lungs. The negative-affected group (NA; n = 40; F = 14/M = 26; median age = 58.5) included individuals negative to COVID-19 (RT-PCR) but affected by different chronic respiratory diseases (the CT-scans didn't show ground-glass opacities). Finally, the negative-negative group (NN; n = 16; F = 14/M = 2) included healthy patients (NN; n = 16; median age = 42.62). Data and findings were collected and compared. RESULTS: Lymphocytes (%) cells showed a decline in COVID-19 patients. The subsets showed a significant association with the inflammatory status in COVID-19, especially with regard to increased neutrophils, T-killer, T-active, T-suppressor, and T-CD8+CD38+ in individuals belong to the either COVID-19 and Covid-like NP group. CONCLUSIONS: Peripheral lymphocyte subset alteration was associated with the clinical characteristics and progression of COVID-19. The level of sub-set cells T-lymphocytes (either high or low) and B-lymphocytes could be used as an independent predictor for COVID-19 severity and treatment efficacy.

Sars-CoV-2 Virus Infection May Interfere CD34+ Hematopoietic Stem Cells and Megakaryocyte-Erythroid Progenitors Differentiation Contributing to Platelet Defection towards Insurgence of Thrombocytopenia and Thrombophilia.

To date, several cases of thrombosis have been confirmed to be related to Sars-CoV-2 infection. Multiple attempts detected the prolonged occurrence of Sars-CoV-2 viral RNA (long COVID) in whole blood suggesting that virus byproducts may remain within cells and tissues well over the disease has finished. Patients may develop severe thrombocytopenia, acute anemia of inflammation and, systemic thrombosis with the fatal course of disease, which is suggestive of further interferences of Sars-CoV-2 on hematopoietic stem cells (HSCs) within the differentiation process towards erythroid and megakaryocytic cells. Therefore, we speculated whether Sars-CoV-2 propagates in or compartmentalizes with hematopoietic progenitor, erythroid, and megakaryocytic cells as the main cause of thrombotic events in either COVID-19 patients or vaccinated individuals. Results: The Sars-CoV-2 RNA replication, protein translation and infectious particle formation as the spike proteins in hematopoietic cell lines take place via the angiotensin-converting enzyme 2 (ACE2) entry pathway within primary CD34+ HSCs inducing, ex vivo, the formation of defected erythroid and megakaryocytic cells that eventually become targets of humoral and adaptive immune cells. Conclusions: Viral particles from affected CD34+ HSCs or the cellular component of RBC units and eventually platelets, present the greatest risk for sever thrombosis-transmitted Sars-CoV-2 infections.

The THYCOVIT (Thyroid Surgery during COVID-19 pandemic in Italy) study: results from a nationwide, multicentric, case-controlled study.

The outbreak of the COVID-19 pandemic has led to a disruption of surgical care. The aim of this multi-centric, retrospective study was to evaluate the impact of the pandemic on surgical activity for thyroid disease among the Italian Units of Endocrine Surgery. Three phases of the pandemic were identified based on the epidemiological situation and the public measures adopted from the Italian Government (1st phase: from 9th March to 3rd May 2020; 2nd phase: from 4th May to 14th June; 3rd phase: from 15th June to 31st). The patients operated upon during these phases were compared to those who underwent surgery during the same period of the previous year. Overall, 3892 patients from 28 Italian endocrine surgical units were included in the study, 1478 (38%) operated upon during COVID-19 pandemic, and 2414 (62%) during the corresponding period of 2019. The decrease in the number of operations was by 64.8%, 44.7% and 5.1% during the three phases of COVID-19 pandemic, compared to 2019, respectively. During the first and the second phases, the surgical activity was dedicated mainly to oncological patients. No differences in post-operative complications were noted between the two periods. Oncological activity for thyroid cancer was adequately maintained during the COVID-19 pandemic.

Short-Term Results of Sclerotherapy with 3% Polidocanol Foam for Symptomatic Second- and Third-Degree Hemorrhoidal Disease.

BACKGROUND: Hemorrhoidal disease (HD) is defined as the symptomatic enlargement and/or distal displacement of anal cushions and is one of the most common proctological diseases. Sclerotherapy (ST) with 3% polidocanol foam induces an inflammatory reaction with sclerosis of the submucosal tissue and consequent suspension of the hemorrhoidal tissue. The aim of this study was to evaluate the short-term effectiveness and safety of ST with 3% polidocanol foam for the treatment of symptomatic second- and third-degree HD. METHODS: A total of 66 patients with symptomatic second- and third-degree HD underwent a single ST session between March 2017 and July 2018. A visual analog scale score was used to assess post-operative pain and patient satisfaction. The symptoms severity and anal continence were investigated through the Hemorrhoid Severity Score (HSS) and Vaizey score, respectively, at baseline, at 4 weeks and after 1 year. RESULTS: Fifty-seven out of 66 patients were male (86.3%), and the mean age was 52 (29-75; SD ± 12) years. The mean operative time was 4.5 (2-6; SD ± 1.23) minutes. No intraoperative complications and no drug-related side effects occurred. The overall success rate was 78.8% (52/66 patients) after a single ST session and 86% after two ST sessions (57/66 patients). The mean treatment effect, obtained comparing preoperative and 12 months symptom scores in each patient, showed a median change of 8 (p < 0.001). All patients resumed their normal daily activities the day after the procedures. CONCLUSIONS: ST with 3% polidocanol foam is a safe, cost-effective and repeatable conservative treatment.

Bilateral Phyllodes Giant Tumor. A Case Report Analyzed by Array-CGH.

The breast phyllodes tumor is a biphasic tumor that accounts for less than of 1% of all breast neoplasms. It is classified as benign, borderline, or malignant, and can mimic benign masses. Some recurrent alterations have been identified. However, a precise molecular classification of these tumors has not yet been established. Herein, we describe a case of a 43-year-old woman that was admitted to the emergency room for a significant bleeding from the breast skin. A voluminous ulcerative mass of the left breast and multiple nodules with micro-calcifications on the right side were detected at a physical examination. A left total mastectomy and a nodulectomy of the right breast was performed. The histological diagnosis of the surgical specimens reported a bilateral giant phyllodes tumor, showing malignant features on the left and borderline characteristics associated with a fibroadenoma on the right. A further molecular analysis was carried out by an array-Comparative Genomic Hybridization (CGH) to characterize copy-number alterations. Many losses were detected in the malignant mass, involving several tumor suppressor genes. These findings could explain the malignant growth and the metastatic risk. In our study, genomic profiling by an array-CGH revealed a greater chromosomal instability in the borderline mass (40 total defects) than in the malignant (19 total defects) giant phyllodes tumor, reflecting the tumor heterogeneity. Should our results be confirmed with more sensitive and specific molecular tests (DNA sequencing and FISH analysis), they could allow a better selection of patients with adverse pathological features, thus optimizing and improving patient's management.

Correction to: Emergency surgery due to diaphragmatic hernia: case series and review.

[This corrects the article DOI: 10.1186/s13017-017-0134-5.].

Efficacy and early results after combining laparoscopic harvest of double gastroepiploic lymph node flap and active physiotherapy for lower extremity lymphedema.

BACKGROUND: Lymph node flap transfer has gradually gained popularity for the treatment of upper and lower limb lymphedema. The aim of this study is to present the outcomes of an integrated treatment protocol based on double gastroepiploic lymph node flap (DG-VLN) and active physiotherapy in patients affected by Stage II and III lower extremity lymphedema. METHODS: All Stage II and III lower limb lymphedema patients operated between September 2015 and December 2017 were retrospectively identified and only those treated with an integrated approach of DG-VLN flap and active physiotherapy were included. Outcomes were assessed clinically with limb circumference measurement and radiologically with lymphoscintigraphy. Flap viability was evaluated through indocyanine green lymphography. Lymphedema related quality of life was evaluated preop and at 1 year follow up through LYMQOL questionnaire. RESULTS: Sixteen patients met inclusion criteria. Mean follow up was 26.2 months. Significant reduction in lower limb volume was observed for all patients from pre to post intervention. At 3 months of follow up, the mean CRR was 42.4% at below knee (BK) level and 25.4% at above knee (AK) level. At 12 months of follow up, the mean CRR was 58.3% at BK level (p = .001*) and 43.4% at AK level (p < .04*). LYMQOL metrics showed significantly better scores in all domains. CONCLUSIONS: Patients with lower limb lymphedema can benefit from combined DG-VLN flap and active physiotherapy, as this approach seem to fasten the onset of improvement and to have a positive impact on patients' quality of life.

Transaxillary robotic-assisted thyroid surgery: technique and results of a preliminary experience on the Da Vinci Xi platform.

BACKGROUND: Robotic thyroidectomy by transaxillary approach (RATS) is regarded as a feasible and safe alternative procedure in selected patients with benign disease or thyroid cancer of low risk, facilitating thyroidectomy with respect to conventional endoscopic approach and offering improved cosmetic results. The Da Vinci Xi surgical system (Intuitive Surgical, Sunnyvale, CA, USA) presents technical advantages over its previous generations, including overhead docking, more compact robotic arms, extended range of motion, and ability for camera to be docked in any arm. This construct supports dissection in smaller spaces with less arm interference and improved view. We present an initial experience of RATS on DVSS Xi in an academic Centre in Italy. METHODS: We conducted a prospective observational study, involving patients with thyroid disease and treated between April 2016 and January 2018. A modified thyroidectomy retractor (Modena retractor, CEATEC Medizintechnik, Germany) was used to lift a musculocutaneous flap and operate gasless. Instrument placement was recorded for each procedure. Each procedure description was broken down into three phases, creation of working space, machine docking with instrument positioning and endoscopic operating technique. Duration of cases was recorded. Patients selected were young women, BMI < 30, thyroid nodule < 5 cm, cytology TIR2 to TIR4 (TIR4:only nodules < 1 cm diameter). RESULTS: Twelve RATS were performed within the learning curve for the robotic technique, 10 lobectomies and 2 total thyroidectomies. No patients required reintervention. Mean duration of surgery was 198.9 min for lobectomy and 210 for thyroidectomy. The same surgical team performed all procedures. No patients presented surgery-related complications, mean stay was 3 days. Decrease in operating time was observed after 8 cases along with more precise preparation of working space. Four arms were used in the first 10 procedures then only three. No recurrent laryngeal nerve dysfunction, no seroma or haematoma were recorded. One patient had transient hypocalcaemia after total thyroidectomy. CONCLUSIONS: Since the early phases of a preliminary experience RATS appeared a safe alternative to open thyroidectomy. Uptake of technique was quick on Xi platform with few technical tweaks over techniques described for Si machines. Careful patient selection is crucial. TRIAL REGISTRATION: Retrospectively registered on 20 july 2018 . TRIAL REGISTRATION NUMBER: researchregistry4272. The Research Registry: https://www.researchregistry.com/browse-the-registry#home/registrationdetails/5b517f08dbc2045aefd7f9b4/.

A method to repair the recurrent laryngeal nerve during thyroidectomy.

Summary: Vocal cord palsy (VCP) is one of the most frequent complications following thyroidectomy. We evaluated the outcomes of intraoperative reconstruction of the recurrent laryngeal nerve (RLN). Of 917 patients who underwent thyroid surgery in a single high-volume general surgery ward between 2000 and 2015, 12 (1.3%) were diagnosed with RLN injury and were retrospectively categorized into 2 groups: group A (n = 5), with intraoperative evidence of iatrogenic transection or cancer invasion of the RLN, and group B (n = 7), with postoperative confirmation of VCP. In group A, immediate microsurgical primary repair of the RLN was performed. Postoperative assessment included subjective ratings (aspiration and voice quality improvement) and objective ratings (perceptual voice quality according to the grade, roughness, breathiness, asthenia and strain [GRBAS] scale, and direct laryngoscopy). In group A, roughness, breathiness and strain were significantly lower at 9 months than at 3 months (p < 0.05). Although larger, multicentre studies are needed, the results suggest potentially excellent postoperative phonatory function after immediate RLN reconstruction.

A Prospective, Comparative Evaluation on Totally Implantable Venous Access Devices by External Jugular Vein versus Cephalic Vein Cutdown.

The request for totally implantable venous access devices (TIVADs) has rapidly grown up through the last decades. TIVADs are implanted by direct vein puncture or by surgical approach with vein cutdown. The authors present a comparative prospective study evaluating external jugular vein (EJV) and cephalic vein cutdown techniques. Two hundred and fifteen patients were consecutively submitted to TIVAD implantation to perform chemotherapy. Patients were divided in two groups, depending on the implantation technique. Group A patients (106) underwent implantation via EJV cutdown and group B (109) patients underwent implantation by cephalic vein cutdown. The following variables were investigated: operating time, need for conversion to other approaches, complications, and intraoperative and postoperative pain. In Group A patients, the success rate of the procedure was 100 per cent, whereas in 11 patients (10.1%) of Group B, a modification of the initial approach was needed. Mean operative time was 23.9 ± 9.2 minutes in Group A and 35.4 ± 11.9 in Group B, and this was statistically significant (P < 0.05). Complication rates at 30 days were similar. Considering intraoperative pain, a difference was found between the two groups because the mean value of pain in Group A was lower than that in Group B (4.13 ± 0.3 vs 5.22 ± 1.24), even if not significant. External jugular vein cutdown approach is quick and safe and allows a very high success rate with very low risk of complications. For these reasons, this approach could be considered as a first choice in TIVAD placement.

Congenital diaphragmatic disease: An unusual presentation in adulthood. Case report.

INTRODUCTION: Congenital diaphragmatic disease is a quite common condition that usually occurs in the neonatal period, and the diagnosis of congenital diaphragmatic disease in adulthood is rare. CASE PRESENTATION: A 64-years-old Caucasian woman was admitted in emergency at our Department, due to a bowel obstruction and dyspnea. A CT-scan showed a diaphragmatic herniation in the left area, with malposition of dilated transverse and descending colon in the chest. An emergency laparatomy was performed, showing a toxic megacolon, in the absence of a true diaphragmatic hernia, and a left diaphragm and left liver hypoplasia. An intraoperative bronchoscopy revealed concomitant hypoplasia of the left lung. A subtotal colectomy with ileo-rectal anastomosis was performed. The postoperative course was uneventful. Histological examination demonstrated hyperplasia of the muscularis mucosae of the colon and cytoplasmic vacuolization of the Auerbach plexus ganglia. The karyotype genetic analysis excluded concomitant microdeletion or duplication syndromes. DISCUSSION: To our knowledge, this seems to be the first reported case of toxic megacolon in a patient with congenital hypoplasia of the left bronchial-lung system, of the left liver, and of the left diaphragm. CONCLUSION: The correct development of the diaphragm is essential for the neighboring organs. The observed clinical pattern could be related to a partial modification of neural crest cell detachment or migration, which could be responsible for bowel and diaphragm defects, even though it was not included in typical neural crest cell syndromes. Further researches should be performed in order to define the sporadic or syndromic source of these multiorgan defects.

Extralymphatic Disease Is an Independent Prognostic Factor in Hodgkin Lymphoma.

PURPOSE: To identify the characteristics and outcomes of patients with extralymphatic Hodgkin lymphoma. PATIENTS AND METHODS: We performed a retrospective single-institution study of 341 cases comprising 207 male (61%) and 134 female (39%) subjects with a median follow-up of 44 months. RESULTS: Fifty-five patients (16%) had extralymphatic disease. The sites were lung in 29 patients (44%), bone in 22 (33%), liver in 12 (18%), and kidney in 3 (5%). In 46 patients (86%) only one organ was involved, while in 7 patients (13%) extralymphatic disease was present in 2 sites and in 2 patients (3%) in 3 sites. The extralymphatic disease group had a poorer prognosis than the lymphatic disease group. Complete remission rates in the extralymphatic and lymphatic patient subsets were 65% and 82% (P = .043), respectively. CONCLUSION: Extralymphatic disease in patients with Hodgkin lymphoma is a rare occurrence (16%) associated with poor clinical outcome.