Isolated spinal artery aneurysms are extremely rare, and their pathogenesis, clinical presentation, and treatment strategies are poorly established. We report only the second case of a patient with an isolated posterior spinal aneurysm and concurrent left thalamic infarct and review the literature to help clarify treatment strategies of isolated spinal aneurysms.A 49-year-old patient presented with acute onset walking difficulty followed by diaphoresis, back and abdominal pain, and paraplegia. Imaging was notable for a hemorrhagic spinal lesion with compression at T12 through L4 and an acute left thalamic infarct. Surgical exploration revealed an isolated posterior spinal artery aneurysm. The aneurysm was surgically resected and the patient had partial recovery six months post-operatively.Isolated posterior spinal artery aneurysms of the thoracolumbar region are rare lesions that commonly present with abdominal pain, radiating back pain, and lower extremity weakness. Imaging may not provide a definitive diagnosis. The three primary treatment strategies are conservative management, endovascular treatment, or surgical resection. In patients with symptomatic cord compression, immediate surgical intervention is indicated to preserve neurologic function. In all other cases, the artery size, distal flow, morphology, and location may guide management. Spinal artery aneurysms are a rare condition typically associated with vascular anomalies that generate local flow alterations such as fistulas, arteriovenous malformations (AVM), and coarctation of the aorta. When found in the absence of these vascular pathologies, they are referred to as isolated spinal artery aneurysms and usually occur in the anterior spinal artery. Isolated spinal artery aneurysms arising from the posterior axis of the spinal cord are exceptionally rare with optimal medical and surgical management being poorly defined. We report a case of a patient with a spinal subarachnoid hemorrhage caused by rupture of an isolated posterior spinal artery aneurysm that was surgically corrected. The patient also presented with a concurrent thalamic infarct which has only been described in the literature once before .A 49-year-old patient with a history of vestibular schwannoma and epilepsy developed acute onset walking difficulty. Two days later, the patient presented to an outside hospital with diaphoresis, abdominal pain, back pain radiating to the thighs, and bilateral lower extremity paralysis. A brain MRI revealed an acute left thalamic infarct and a spine MRI found compression at T12 through L4 concerning for subarachnoid or subdural hemorrhage. The patient was transferred to our institution for further work-up and management. On our admission, examination revealed dense lower extremity paraplegia with anesthesia and absent rectal tone concerning for conus medullaris syndrome. Additionally, the patient had right hemi-body sensory loss of light touch and pinprick in the face, upper torso, and arm.MRI with MRA of the complete spine demonstrated an intradural cystic and hemorrhagic mass along the left ventrolateral pial surface at the T11-12 level. This lesion was associated with extensive intradural hemorrhage with inferior extension into the lumbar spine  presented with rupture [The natural history of isolated spinal aneurysms is unclear due to its rarity. Through a literature review, we found 39 cases of isolated spinal aneurysms from 2011 to 2019  surgical resection, (2) endovascular treatment, or 3) conservative management  conserva, 6. TheyDespite the successful cases with a wait-and-see approach, the overall occurrence of this condition is too limited to draw definite conclusions. There have been five reported cases of a patient dying without surgical intervention, with re-bleeding being the most common cause –14. EarlWhether the spinal aneurysm was the source of the left thalamic infarct or an incidental finding is difficult to determine. To the best of our knowledge, no cases in the literature have reported a spinal aneurysm as a cause of a cerebral infarct, although one case exists linking a subarachnoid hemorrhage with a spinal cord infarct . This suIn conclusion, isolated spinal artery aneurysms are an exceedingly rare occurrence, particularly in the posterior axis of the spine. Imaging with MRI/CT and angiogram may not provide a definitive diagnosis. We agree with previous authors that in a patient with a posterior spinal aneurysm and symptoms of spinal cord compression, prompt surgical intervention is warranted. Finally, further studies are needed to understand the possible interaction between subarachnoid blood products and cerebral infarction.


The most common mesenchymal tumours of the uterine corpus originate from smooth muscle cells. Leiomyomas arecommonly found in women of child bearing age; however, leiomyosarcomas occur later in life (50-55 years of age).Most uterine leiomyosarcomas occur de novo, but rare cases of leiomyosarcomas that arise from leiomyomas havebeen reported. We present two cases of fertile women with submucosal leiomyomas that became malignant and dis-cuss their pathologic features and immunohistochemistry studies for P16, P53 and Ki67. The most common mesenchymal tumours of the uterus arethe smooth muscle tumours . The majA 41-year-old woman  presentedto the gynaecology clinic with menometrorrhagia. Her pastmedical history and physical examination, including vaginalexam, were normal. On transabdominal ultrasound, theuterine size was 117x68 mm with a homogenous myometrialecho. A solid mass that measured 63x43x52 mm with mixedechogenicity filled the endometrial cavity and was suggestiveof a submucosal leiomyoma. The adnexa were normal.No other abnormal abdominopelvic findings were identified.The patient underwent a total abdominal hysterectomyand bilateral salpingo-oophorectomy. During the surgery, anomental adhesion was identified, which the surgeon decided to send for abdominal cytology. On gross examination of thespecimen in the uterus, a 7 cm diameter submucosal massand three  intramural masseswere found. The largest mass had a creamy cut surface withareas of haemorrhage and the smaller masses had homogenouscreamy cut surfaces . A cyst A 35-year-old woman  wentto the Gynaecology Clinic with abnormal uterine bleeding andspotting. Her drug history revealed consumption of a vaginalherbal suppository and oral contraceptive during the last year.Vaginal examination revealed a mass lesion that projected fromthe cervical canal. Transvaginal ultrasound showed a uterine thatwas 102x55x31 mm in size, endometrial thickness of 4 mm anda submucosal mass in the cervical canal that measured 54x46mm. No abnormality was identified in the adnexa. The patientunderwent a transvaginal myomectomy surgery. The surgicalspecimen was a round, polypoid creamy mass that measured5.5x4x2.5 cm. The cut surface was homogenous and creamywith a typical whorled pattern . MicroscTwo types of acceptable theories for the origin ofleyomyosarcomas are presented in the literature: a)uterine leiomyomas have no malignancy potential andleiomysarcomas are a de novo process without pre-existingneoplastic lesions  and b) rWe report two cases of uterine leiomyosarcomas thatcame from leiomyomas. The mean age of leiomyosarcomapresentation is 10 years more than leiomyomas (50-55years) . Like otThe parallel between the current case features andleiomyosarcomas shows the unpredictable appearancethat a leiomyoma undergoing malignant changes cantake. Fortunately, the limited number of reported cases(with the exception of a few) -8, had nThe accepted criteria for leiomyosarcoma consistsof high mitotic figures, nuclear atypia and coagulativenecrosis. In our cases, the necrosis was absent but thepresence of the two other features differentiated themfrom smooth muscle tumours of uncertain malignantpotential (STUMP) with regards to the current diagnosticcriteria . Under tIn 2009, Mittal et al.  performeThe investigation of p53 mutation in uterine smoothmuscle tumours was first conducted by De Vos et al. Theysequenced the P53 exons of eight cases of leiomyomasand eight cases of leiomyosarcomas. Point mutationswere observed in three cases from the leiomyosarcomagroup, whereas none of the leiomyomas showed anyalterations . SubsequThis study used immunohistochemistry for the P53, P16markers and the Ki67-index to confirm leiomyosarcomasthat arose from small submucosal leiomyomas in twoyoung women. These findings raise the possibilityof malignant transformation of very benign lookingleiomyomas.


Large-cell neuroendocrine carcinomas (NECs) of the colon are extremely rare aggressive tumors. A 79-year-old man presented at our hospital for muco-hematic diarrhea, weight loss and incarcerated hernia in his left groin. Colonoscopy revealed sigmoid stenosis. Computed tomography confirmed an incarcerated hernia containing sigmoid mass and massive abdominal adenopathy. In absence of colonic obstruction, the patient underwent elective palliative sigmoid resection and colostomy by laparoscopic approach, and direct hernia repair through inguinal access. Histopathological examination revealed a large cells sigmoid NEC. We report the first case of large-cell neuroendocrine colon cancer incarcerated in an inguinal hernia. Due to the advanced stage, we have performed a palliative laparoscopic resection in order to reduce surgical trauma, confirm pre-operative results and minimize post-operative complications, and direct hernia repair through inguinal access. The association between inguinal hernia and incarcerated colon cancer is a rare condition. Colon and rectal neuroendocrine carcinomas (NECs) represent less than 1–2% of colorectal cancers. Colon large cells neuroendocrine carcinomas (LCNECs) are rarer accounting for 0.25% of colorectal tumors [We report a case of sigmoid LCNEC incarcerated in an inguinal hernia.A 79-year-old man with a month’s history of muco-hematic diarrhea and weight loss underwent colonoscopy. He also had a left inguinal hernia which had become irreducible in the past 15 days. Colonoscopy showed lumen stenosis 30 cm from the anal edge with mucosal inflammation and erosion . The bioPhysical examination revealed a hernia incarcerated in the left groin involving the scrotum. Computed tomography (CT) of the abdomen demonstrated a huge left inguinal hernia containing the sigmoid colon . Sigma’sLaboratory tests showed a slight increase in C-reactive protein (39 mg/l). Neoplastic serum markers were normal except for Cancer Antigen 125 (CA-125) (131 U/ml). A sigmoid carcinoma imprisoned in an inguinal hernia was therefore suspected. The intervention was considered non-urgent in the absence of small-intestine occlusion and signs of ischemia of the sigmoid walls.In an elective setting, an explorative laparoscopy confirmed sigmoid colon cancer with mesenteric nodules  incarcerPathological examination showed a sigmoid mass invading the serosal appendage . HistopaLymph node metastases were found in 6 out 6 dissected lymph nodes. Immunohistochemistry confirmed tumor cells positivity for synaptophysin and Cg A  and negaThe patient started adjuvant chemotherapy with five fluorouracil cycles. Three months after surgery, a thoraco-abdominal CT scan revealed enlargement of the abdominal adenopathy and multiple metastases to the liver and chest. The patient’s condition rapidly deteriorated and he was therefore referred for palliative care.Inguinal hernia is a very common condition in the adult population. The association of tumors with inguinal hernia is less than 0.5% in the excised sac . Since tLCNEC belongs to the gastroenteropancreatic NECs (GEP-NECs). The GEP-NECs are poorly differentiated, high-grade malignant neoplasms (Ki 67 > 20% HPF), composed of small or large to intermediate cells .Usually, LCNECs are positive for synaptophysin, while CgA expression is often considered negative . The hisThe diagnosis of colon cancer in the incarcerated inguinal hernia is generally confirmed during an emergency operation or in histopathology, resulting in a non-optimal treatment from an oncological point of view. In patients with a long history of reducible inguinal hernia that becomes irreducible, the probable presence of neoplasm should be considered and more in-depth studies should be conducted prior to surgery . CT playHartman’s resection is preferred in case of perforation or strangulation, while colon resection and anastomosis should be considered in case of incarceration. If colon cancer was diagnosed pre-operatively, colectomy could be performed by laparotomy followed by normal inguinal repair through a separate inguinal incision .Laparoscopic treatment of colorectal cancer has numerous advantages in peri-operative recovery and could be considered safe and not inferior to open approaches in terms of cancer outcomes.The laparoscopic approach has only been described in four cases, all in an elective setting , 9, 10. To our best knowledge, this is the first report of a colorectal LCNEC incarcerated in an inguinal hernia. Considering the metastatic disease, the patient’s age and his vulnerability, we decided for a palliative intervention minimizing the risk of post-operative complications.None of the contributing authors have any conflict of interest, including specific financial interests or relationships and affiliations relevant to the subject matter or materials discussed in the manuscript.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available on request.M.G. collected information of patient, revised the literature and drafted the manuscript. N.I. collected information of patient and drafted case report. E.D.F., G.M.M., A.G. and U.C. revised the manuscript. D.M. performed the surgery and revised the manuscript.


Background: Primary squamous cell carcinoma of the thyroid (PSCCT) is an uncommon malignancy that is difficult to diagnose and differentiate. There is no consensus for the early clinical, radiological, or ultrasonic identification of PSCCT before pathological changes are observed in patients. There is also no suitable treatment due to the absence of a definite diagnosis.Case Presentation: A 76-year-old female patient complained about a rapidly growing cervical mass, dyspnea, dysphagia, and a change in her voice. Based on the results of thyroid ultrasound, fine-needle aspiration, and plain and enhanced CT, the patient was initially diagnosed with anaplastic thyroid carcinoma (ATC). Thereafter, we removed the mass that was the patient's main complaint. The gross examination of the patient's symptoms also supported our previous diagnosis. However, her disease was finally diagnosed as PSCCT, according to the histopathology and immunohistochemistry findings of the mass.Conclusion: Our case highlights the need for a comprehensive framework in the management of PSCCT. The more auxiliary examinations  we take, the more likely we are to identify this disease. Immunohistochemistry is currently the preferred examination for the diagnosis of PSCCT, while surgical resection combined with radio-sensitizing therapy and adjuvant chemotherapy is the main treatment method for PSCCT. Both primary squamous cell carcinoma of the thyroid (PSCCT) and anaplastic thyroid carcinoma (ATC) have a lower incidence rate in patients , 2; prevIn this study, we reported the case of PSCCT in a 76-year-old woman who was initially misdiagnosed with ATC, emphasizing the differential diagnosis of such diseases, and we aimed to recommend an optimal treatment for PSCCT.A 76-year-old female patient was admitted to our hospital as the result of progressive enlargement of a left cervical mass that the patient had over the past 2 months, accompanied by pain, dysphagia, and hoarseness. The patient initially found a “mung”-sized mass in her anterior region of the left neck, and her health was in good condition before the discovery of the mass. She had no history of smoking and alcohol consumption.During the physical examination, we found that her trachea deviated to the right side. A fixed and hard neck mass was also found in her left lobar thyroid. The mass had a smooth boundary and could be palpated without sensitivity. The size of her protuberance was ~2 × 2 cm. The mass could also move up and down when she swallowed and was accompanied by disorders of her vocal cord. There was no palpable cervical lymphadenopathy, and the examination results of her nervous system, heart, chest, and abdomen were normal.The thyroid ultrasound showed a well-defined, lobulated, heterogeneous hypoechoic of her left lobe, with a circular hyper-echogenicity measuring of ~1.5 × 1.2 cm. The thyroid capsule appeared to be intact and was labeled in TIRADS (Thyroid Imaging Reporting and Data System) as 3–4a . The ultThe patient's leukocyte count, serum T4, thyroid-stimulating hormone (TSH), thyroglobulin, and serum calcium were all within the normal range, and we were not able to detect the difference in the tumor markers in her blood serum except for elevation of CA199 by three times. Based on the abovementioned results, the patient's mass was considered as a rapidly growing single mass that was accompanied by the nerve recurrent laryngeal nerve invasion and internal necrosis and calcification. Thus, the mass was diagnosed as a poorly differentiated thyroid carcinoma, and we chose to remove it as a result of the abovementioned symptoms. During the course of the operation, there was a 5 × 3 × 2.5 cm mass with extensive adhesion of the topical surface, esophagus, and anterior cervical muscle in her left lobar thyroid . We alsoThe histopathological manifestations of the carcinomas were a prominent nesting pattern, with intercellular bridges and keratinization with a cancer pearl . This soDuring the follow-up, the patient's swallowing and phonation were stable, and she was discharged from the hospital on the third postoperative day. After 3 months, she revisited our hospital with a complaint about a pulmonary disease with dyspnea, chest discomfort, and coughing. The chest CT scan indicated multiple, unclear contour and clouding margin lung nodules, about 6–8 mm in size on both sides of her lungs. Along with increased bronchovesicular shadows, we did not observe any enlarged lymph nodes in her mediastinum. However, there was thickening soft tissue around the trachea that was included in the main CT results. After receiving short treatment for respiratory system diseases, the patient died from respiratory failure. CT report before death was as follows: a little inflammation in both lungs, nodules in both lungs, arteriosclerosis of aorta and coronary artery, and a little pericardial effusion, considered as pulmonary metastasis. The patient died due to old age, emaciation, and poor nutritional status.Intraoperative whole-body CT scan and gastroscopy showed no positive findings, and laryngoscopy showed no positive findings. Chest CT alone suggested small nodules in the lower lobes of both lungs . There wAn effective examination for diagnosis must follow the underlying differences in etiologies between PSCCT and ATC. The origin of the primary squamous cell carcinoma became a controversial matter due to the absence of squamous epithelium in the normal gland , 8. At pImaging using ultrasonography (US) to obtain a definite diagnosis of PSCCT has not been ruled out. However, in terms of previous US imaging findings, PSCCT was described as the eggshell calcification nodule, surrounded by soft tissues or the hypoechoic solid nodules with irregular margins , 10, shoConsidering the limitations of US in preoperative evaluations, CT was performed. In our CT findings, PSCCT was described as a huge mass containing focal cystic changes and the course/curvilinear or eggshell calcification, which can change the pressure of the trachea , 14. TheNo other obvious abnormality was found in the patient's blood, and the biochemical detection can suggest the characteristics of the tumors. Although there are many established biomarkers that are involved in malignant tumors in the head and neck , thyroglIn PSCCT, a pathological examination is often used to confirm its diagnosis. The microscopic description of PSCCT is composed of squamous cells, with prominent intercellular bridges in the different differentiation states . In PSCCCurrently, there is no standard treatment that can be used as a guideline for PSCCT due to the rarity of the cases , 6. The Given that PSCCT is insensitive to chemotherapy and is relatively resistant to radiotherapy, whether to use additional adjuvant therapy or chemotherapy during the perioperative period is controversial , 5, 6. TDifferentiating the difference between PSCCT and ATC in the preoperative diagnostic period is challenging since both diseases present similarities in the clinical symptoms . So far,All datasets presented in this study are included in the article/supplementary material.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.R-zZ conceived the idea and conceptualized the study. G-hH collected and analyzed the data. R-zZ and Y-jX drafted the manuscript. G-hH reviewed the manuscript. All authors read and approved the final draft.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Gastrointestinal cancer, gynecologic cancer and malignant peritoneal mesothelioma may disseminate widely to the surfaces of the abdomen and pelvis. In some patients this spread of the disease may find its way into a hernia sac.The Spigelian hernia is caused by a protrusion of the peritoneum of the anterior abdominal wall into the abdominal wall muscle. This peritoneal defect occurs through the semilunar line of Spiegel in or closely associated with the semicircular line of Douglas.A patient presented with a large (11 cm greatest diameter) mass in the left lower quadrant. The mass was composed of malignant peritoneal mesothelioma and was anatomically located within the Spigelian hernia belt. At the time of cytoreductive surgery numerous other sites of malignant peritoneal mesothelioma were present within the abdomen and pelvis.The left lower quadrant mass was a malignant peritoneal mesothelioma presenting within a Spigelian hernia defect. This phenomenon has not been previously reported. Hernia Many different types of hernia defects of the parietal peritoneum exist and result in a protrusion of the skin of the abdominal wall. Over a lifetime, the prevalence of inguinal hernias is approximately 7.3% of men. The prevalence in women is approximately 5% of that in men ,5.A rare malignancy occurring as a result of cancerous change of the lining of the abdomen and pelvis is malignant peritoneal mesothelioma. It occurs in the United States in approximately 800 patients per year . A new oTo my knowledge, the presenting sign of malignant peritoneal mesothelioma in a Spigelian hernia has not been previously reported. In this case report a 70-year-old woman presented with an abdominal wall mass of malignant peritoneal mesothelioma at the exact site where a Spigelian hernia would case a bulge. This is the first report of malignant peritoneal mesothelioma in a Spigelian hernia leading to the diagnosis and definitive treatment of this disease.www.researchregistry.com website with UIN 5346.Data on this patient was prospectively recorded and then retrospectively reviewed at academic institutions. This research work has been reported in line with the SCARE criteria . This st2In November 2002, a 70-year-old white woman complained of pain in a left lower quadrant abdominal mass. By physical examination a mass was fixed within the abdominal wall musculature within the left lower quadrant of the abdomen.On November 11, 2003, a CT was obtained which showed a mass within the anterior abdominal wall measuring 11 cm in diameter . A seconOn March 4, 2003, a 9-h surgical procedure was performed which removed all visible evidence of mesothelioma from abdominal wall, abdomen and pelvis. Resection of the mass from the left lower aspect of the abdominal wall required resection of the lower one-half of the rectus abdominus muscle and adjacent abdominal wall musculature to the iliac crest. The resected specimen measured 15 cm × 12 cm × 7 cm.Other sites of disease that needed to be removed in order to have a complete cytoreduction included a greater omentectomy and splenectomy, peritoneum from beneath the right hemidiaphragm, tumor nodules from small bowel and its mesentery, and complete pelvic peritonectomy with resection of rectosigmoid colon, uterus, Fallopian tubes and ovaries .After complete cytoreduction the patient was treated with hyperthermic intraperitoneal chemotherapy (HIPEC) .The patient’s postoperative course was uneventful. At 18 months postoperatively multiple lung metastases were seen by CT. The patient died of progressive disease in July of 2005, 29 months after her treatment by cytoreductive surgery and HIPEC.33.1A definitive determination of the pathophysiology of a malignant mesothelioma mass within the anterior abdominal wall in a 70-year-old woman is not obvious. Malignant peritoneal mesothelioma within the musculature of the abdominal wall does not occur unless there is a laparoscopy trochar site or a transabdominal biopsy of the malignant mesothelioma tumor . One may3.2There are several reports in the oncology literature regarding peritoneal metastases becoming symptomatic within a hernia sac. An early example of cancer cells and/or malignant ascites accumulating as a mass at the umbilical hernia defect was reported by Mayo et al. . The umb3.3In our patient, malignant peritoneal mesothelioma was widely distributed on the peritoneal surfaces of the abdomen and pelvis. By far, the largest tumor accumulation was within the Spigelian hernia sac. Tumor was present within the omentum in large volume likely because of phagocytic activity of omental macrophages. At other sites the malignant mesothelioma was layered out on the peritoneal surface of the right hemidiaphragm, surface of the spleen and pelvic peritoneal surfaces. The only large mass (15 cm × 12 cm × 7 cm) was within the hernia defect. Not surprising this painful mass in the left lower quadrant of the abdomen was this patient’s presenting complaint. Recently, we reported on a male malignant peritoneal mesothelioma patient whose presenting complaint was a left inguinal mass . This syPaul H. Sugarbaker has no conflicts of interest to declare.Foundation for Applied Research in Gastrointestinal Oncology.Data management and secretarial support provided by Local IRB-approval for this case report was not required:MedStar Health Institutional Review Board has determined that a case report of less than three [3] patients does not meet the DHHS definition of research (45 CFR 46.102(d)(pre-2018)/45 CFR 46.102(l)(1/19/2017)) or the FDA definition of clinical investigation (21 CFR 46.102(c)) and therefore are not subject to IRB review requirements and do not require IRB approval.This case report is of 1 patient.Informed consent was requested through surviving family members for publication of this case report and accompanying images.Paul H. Sugarbaker, MD: study concept or design, data collection, data analysis or interpretation, writing the paper.This study was registered as a case report on the www.researchregistry.com website with UIN 5346.Paul H. Sugarbaker, MDNot commissioned, externally peer-reviewed.


This report describes a case of solitary tracheal lymphoma in a 14-year-old alpaca mare.The alpaca was referred for dyspnea and inspiratory noise. The clinical examination included complete blood cell count, blood chemistry, endoscopy, ultrasound, radiographs, and computed tomography (CT). A solitary tracheal intraluminal and juxtatracheal lymphoma was diagnosed by fine needle aspiration (FNA). The owner requested euthanasia due to the uncertain prognosis. At postmortem examination, the presence of solitary lymphoma without involvement of other organs was confirmed. Immunohistochemical analysis confirmed a B-cell origin.Although multicentric lymphoma is the most commonly described neoplasia affecting South American camelids (SAC), solitary forms of the disease may occur. Vicugna pacos) mare diagnosed with solitary tracheal B-cell lymphoma.There are occasional reports of neoplasia in South American camelids (SAC), with multicentric lymphoma being the most commonly reported neoplasm described in llamas and alpacas –10. ThesA 14-year-old, 86 kg, domestic huacaya alpaca mare, was referred to the Clinic for Ruminants, Vetsuisse-Faculty, University of Bern, Switzerland for acute dyspnea and inspiratory noise. The mare had not shown any previous respiratory symptoms and had always a good appetite. The referring veterinarian did not initiate any treatment prior to referral.Upon clinical examination, the alpaca was bright, alert and in good general condition. Body condition was excellent  with a body condition score (BCS) of 3 out of 5. Rectal temperature was 37.6 °C (range 37.5–38.9 °C), heart rate 80 beats per minute (range 60–80 bpm) and respiratory rate 28 respiration per minute (range 10–30 rpm). The mare showed mild dyspnea with bilateral dilated nostrils and inspiratory stridor. There was no nasal discharge nor spontaneous or provoked cough. Auscultation of the lung fields revealed bilateral normal sounds, whereas tracheal auscultation revealed a high-pitched stridor. Mucosal membranes were pink and moist and oxygen saturation  was 95%. Complete blood cell count and blood chemistry were performed and all values were within normal limits.Resting endoscopic examination  of the upper airway revealed mild epiglottitis characterized by mucosal edema and reddening, and epiglottic retroversion where the epiglottis retroverted in the opening of the glottis during inspiration and returned to its normal position with each expiration  of the neck showed a wide-based soft tissue mass in the dorsal tracheal wall at the level of the 5th cervical vertebra (C5)  revealed the presence of intraluminal tracheal and left juxtatracheal homogeneous hypoechoic masses at the level of the caudal third of the neck Fig. . The extThe diagnosis of a tracheal mass was made and a computer tomographic (CT) examination  was performed under general anesthesia to evaluate the tracheal mass and the surrounding soft tissues in more detail. The CT examination confirmed the presence of an ovoid homogeneous soft tissue mass (LWH: 25.5 × 23 × 14.5 mm), originating from the left dorsal tracheal wall and maximally occluding +/− 90% tracheal lumen at the caudal aspect of the 4th cervical vertebra (C4)  of the extraluminal mass was therefore performed under ultrasound guidance. Smears were moderately cellular and contained predominantly medium to occasionally large lymphocytes, with a round to mildly indented nucleus (approximately 10 to 15 μm in diameter), coarsely stippled chromatin, occasionally 1–2 small, variably prominent nucleoli, and low to moderate amounts of mid to deeply basophilic cytoplasm .The postmortem examination revealed a 2 cm in diameter light pink, round, firm mass firmly attached to the left dorsal tracheal serosa, approximately 30 cm caudal to the larynx Fig. b. ImmediSamples of the tracheal masses and from the main internal organs were fixed in 10% neutral buffered formalin for histological examination. The samples were routinely processed, paraffin wax embedded, stained with hematoxylin and eosin (H&E), cut in 3 μm sections and observed in standard light microscopy.Histologically, the intraluminal tracheal mass consisted of a highly cellular, well demarcated, unencapsulated and infiltrative growing proliferation of neoplastic round cells within the lamina propria, which obstructed the tracheal lumen almost completely and whose surface was focal- extensive ulcerated. The neoplastic cells grew in sheets on a scant amount of highly vascularized fibrous stroma, displayed a small amount of eosinophilic, homogeneous cytoplasm, a round nucleus measuring 1–2 times the length of an erythrocyte with finely stippled chromatin and up to 4 round, basophilic nucleoli Fig. a. The anImmunohistochemical stains for CD20  and CD3  were then performed in order to further characterize these neoplastic lymphocytes. The spleen and a mediastinal lymph node from an alpaca that died due to cachexia and tissue sections from the intraluminal tracheal mass without primary antibody addition were used as positive and negative controls, respectively. The neoplastic lymphocytes in both masses showed a strong positive membranous staining in the CD20 stain Fig. b and e. Although multicentric lymphoma is the most commonly reported neoplasm described in SAC –10, thisImmunohistochemical staining using CD3, PAX-5, BLA36, CD 79 and CD20 , 12, 13 Primary tracheal neoplasia are uncommon in animals and humans. In humans, tracheal neoplasia account for up to 0.2% of all tumors, with most being squamous cell carcinoma or adenoid cystic carcinoma , 15. LymIn the present case, it could not be determined with certainty whether this neoplasia had an intraluminal or extraluminal tracheal origin. However, since both masses displayed similar histopathological and immunohistochemical characteristics, a common origin is assumed. In human medicine, intraluminal tracheal B-cell lymphomas have often been characterized as mucosa associated lymphoid tissue (MALT) lymphomas. These are low-grade malignant neoplasias, which often remain localized and are generally associated with a favorable outcome , 22. In Thoracic radiography is the initial imaging study being performed in humans presenting signs of dyspnea, wheezing and/or hemoptysis, suggestive of a tracheal neoplasia. This imaging modality, however, identifies only 18–28% of tracheal tumors . Neck raTreatment strategies in human medicine include surgical excision, chemotherapy and radiation therapy, alone or in combination. Reports of chemotherapy in SAC are rare. Cebra et al. reported an attempt using a combination of cyclophosphamide, vincristine and prednisolone for treatment of a llama diagnosed with a multicentric lymphoma, but with only a transient and short-duration response . RadiatiThis report describes the clinical, diagnostic imaging, and pathological findings of a solitary tracheal B-cell lymphoma in a geriatric alpaca mare. As SAC are constantly increasing in popularity as pets, information regarding uncommon diseases become important for the veterinarian. Although multicentric lymphoma is the most common neoplasm affecting SAC, solitary forms of the disease may occur. Further studies are needed regarding the therapeutic options of neoplasms in SAC.


Pulmonary hypertrophic osteoarthropathy (PHO) is a rare paraneoplastic syndrome that mainly occurs in patients with lung cancer. Most symptoms occur symmetrically, and unilateral symptoms without clubbing are infrequent. This report presents the case of a rare atypical symptom that was highly suspected of being PHO.A 77-year-old woman with swelling and severe pain in the left femur for 2 months was referred to our hospital. Radiography revealed a remarkably osteogenic thickening and sclerotic lesion in her distal femur. Preliminary diagnoses of malignant bone tumor and hematological tumor were made based on laboratory test results, radiological examination, and clinical manifestation. A needle biopsy was performed, which ruled out the diagnosis of malignant bone tumors. A low-grade bone tumor was still suspected. After that, en bloc resection was performed, followed by replacement of the femur with a mega-prosthesis. Pathological analysis revealed no malignant tumor cells, and immunohistochemical staining for CDK4 and MDM2 yielded negative results. Meanwhile, pulmonary large cell carcinoma was identified on biopsy. Based on published studies, a diagnosis of atypical PHO was made according to clinical and imaging manifestations.This is an infrequent case of PHO with unilateral symptoms in the left femur. When clinical manifestations and radiological findings are inconsistent with the pathological results, the possibility of benign lesions with malignant clinical manifestations or imaging features should be carefully considered. Hypertrophic osteoarthropathy (HOA) is a rare syndrome that can be divided into two categories: primary (hereditary or idiopathic) and secondary HOA. Pulmonary hypertrophic osteoarthropathy (PHO) can be secondary to HOA, which is a paraneoplastic syndrome. Most patients are complicated by PHO secondary to intrathoracic malignancies, lung or heart diseases. PHO predominantly usually occurs in middle-aged and elderly patients. The incidence of PHO is relatively low, and prior studies have reported incidences of PHO of 0.2–17% in lung cancer patients .Clinical symptoms of PHO usually appear symmetrically in the extremity and include clubbing, periostitis, arthritis, periosteal proliferation, and pain and swelling in the joints . RadioloAccording to prior studies, PHO with unilateral symptoms or without clubbing is relatively uncommon. This report presents a rare atypical symptom that was highly suspected of being PHO accompanied by unilateral pain, periostitis, and swelling. The clinical symptoms and radiological findings was similar to those of malignant tumor. This case will be expected to provide knowledge for the accurate diagnosis of atypical PHO with unilateral symptoms in the extremities.In January 2018, a 77-year-old woman was referred to our hospital with spontaneous pain and swelling of the left thigh. The patient noticed swelling of the left distal thigh, without pain, in 2015. In November 2017, she developed severe pain and consulted doctors at a general hospital. Radiography revealed a remarkably osteogenic thickening and sclerotic lesion in the distal femur , the T1-weighted image showed extremely low signals in the sclerotic lesion. The T2 fat-suppressed image showed heterogeneously signals in the sclerotic lesion and medullary cavity. Gadolinium contrast MRI images showed multiple high signals in the soft tissue and bone. The bone scintigraphy revealed significant uptake in the left femur  showed abnormally high accumulation in the lung, lung lymph nodes, cervical vertebra, thoracic vertebra, right third rib and left femur Fig. .Fig. 4ReThe clinical manifestations and radiological examination findings were inconsistent with the pathological results. However, a diagnosis of the low-grade malignant bone tumor was still suspected by the multidisciplinary team (MDT), and a treatment plan involving complete excision was done. Because of the older age, poor medical condition and suspicion of a low-grade tumor, neoadjuvant chemotherapy and open biopsy were not performed. En bloc resection was carried out, followed by the replacement of the distal femur with a mega-prosthesis Fig. . The finDue to the lack of support, all malignant tumors were ruled out. Combined with imaging diagnosis such as significant uptake in bone scan and PET-CT and sclerotic lesion in radiography, meanwhile patient was also diagnosed as pulmonary large cell carcinoma, PHO was finally diagnosed.Low-grade osteosarcoma and benign lesions are difficult to distinguish, especially osteoblastic lesions in the distal femur, due to its non-specific radiological findings and clinical manifestation. Since there are no atypical malignant tumor cells in the histological section, it is easy to be misdiagnosed, which leading to a poor outcome. Previous studies have reported that the misdiagnosis rate was close to 50% with a preoperative biopsy , 7. EvenHayashi et al.  reportedIn our study, the preliminary diagnoses were osteosarcoma and hematological malignancy based on clinical and radiographic characteristics. Subsequently, considering the PET-CT results, a metastatic tumor, osteomyelitis and a low-grade malignant bone tumor were suspected. However, although histopathological examination and bone-marrow biopsy excluded the above-mentioned diseases, the low-grade malignant bone tumor was still highly suspected. Initially, an open biopsy was planned to obtain more specimens to arrive at a definitive diagnosis. However, given the fact that multiple biopsies and extraction of the massive specimen might increase the risk of transmission of tumor cells, as well as, the patient having aplastic anemia and old age, there was a possibility that the patient could not tolerate multiple operations and anesthesia exposure. In addition, considering the patient had severe pain and poor function of the left femur. Therefore, we had to adopt a surgical plan of en-bloc resection of the tumor at the femur and followed by replacement with megaprosthesis.The resected specimen was then sent for histopathological examination to determine the definitive diagnosis. Unfortunately, the final histopathological analysis revealed massive fibrous tissue, granulation tissue, osteoblasts and no malignant tumor cells. Hence, the diagnosis of low-grade osteosarcoma was ruled out by the results of MDM2 and CDK4 on IHC staining.Consequently, the pulmonary large cell carcinoma was identified on biopsy. In addition, the pathologist also performed the IHC staining for CD3, CD20, CD34, and kappa and lambda light chains, which yielded negative results, ruling out the diagnosis of hematological malignancies. Therefore, malignant bone tumors, hematological malignancies, low-grade osteosarcoma, and metastatic tumors were all completely excluded. Nonetheless, it was noted that the severe pain and limb function significantly improved after the surgery.Due to clinical manifestations and radiological examination findings were inconsistent with the pathological result, it is difficult to make a final diagnosis in this case. Gene detection might help make a more accurate diagnosis according to relevant literature . UnfortuClinical manifestations of PHO include clubbing, periostitis, arthritis, periosteal proliferation, and pain and swelling in the joints, which appear symmetrically in the extremity . RadioloThe standard treatment for PHO is the removal of the primary lesion. Chemotherapy or radiotherapy are acceptable methods for control of the unresectable primary disease. Administration of adrenocortical hormone, nonsteroidal anti-inflammatory medications, and opioids have been reported to be useful in pain management. However, in this case, even if the diagnosis of PHO was confirmed in the patient before surgery, it would have been difficult to administer chemotherapy or palliative treatments because of the poor condition of the patient .This is a very rare case, and we can't make a definitive diagnosis. Due to the lack of typical symptoms, we combined the symptoms of the patient with the relevant literature. Finally, we had to highly suspect the diagnosis of PHO, although it does not have typical PHO symptoms. However, due to the result of the PET-CT examination, we did not examine some serum tumor markers. In addition, the patient had severe pain and poor function of the left femur, and we do not know if conservative treatment can relieve the symptom. We hope that this case can provide more ideas for the diagnosis of tumors in the future.In conclusion, we reported a case in which the clinical manifestations and radiological examinations results are inconsistent with the pathological results. We should carefully consider the clinical manifestations with atypical symptoms, radiological examination results, and pathological results between the benign lesion and malignant tumors. We believe that this case report has clinical applicability in making a differential diagnosis between a malignant bone tumor and a benign lesion.


Pericoronal radiolucent lesions are a common radiographic finding, but it is rare that they occur in multiple forms. Multiple calcifying hyperplastic dental follicles (MCHDF) are entities with few cases described to date; nevertheless, they appear to have a very particular phenotypic pattern.Case 1: A 10-year-old male was evaluated radiographically, revealing four impacted canines, each accompanied by unilocular pericoronal radiolucency. Case 2: A 16-year-old male was planning orthodontic treatment; following his radiological evaluation all third molars were found to be accompanied with pericoronal radiolucencies. Enucleation, and third molar removal along with the pericoronal tissue were the respective treatments. Microscopically, in both cases, the specimens shown odontogenic epithelium, and type I and II calcifications in the hyperplastic follicles, all these characteristics were consistent with MCHDF.Although MCHDF are a rare entity, they must be considered in the differential diagnosis of multiple pericoronal lesions. Under the light of the current evidence, the histological findings may be relatively heterogeneous, but their integration with both the clinical data, which are apparently particular, and with the radiographic characteristics, can lead to a definitive diagnosis. Pericoronal radiolucent lesions may have different origins, and the possibilities represent a wide range of entities Table . In geneA 10-year-old male with premature loss of the two primary mandibular canines and the primary right maxillary canine, was evaluated in a dental clinic Fig. . Short rFDI notation)  Fig. g. In allon) Fig. h. Finallon) Fig. i. The clon) Fig. . After 2A 16-year-old male went to a private clinic to plan his orthodontic treatment Fig. . During FDI notation)  Fig. d. Areas To date, there have been few cases of MCHDF reported, in this discussion we have considered those that cover the following parameters: three or more teeth involved and with no association with dental or syndromic abnormalities –14. A grFrom the radiographic point of view, MCHDFs are usually identified due to the lack of teeth eruption. In this sense, although the common trait of MCHDF is calcification, this is not radiographically evident in the early stages and is therefore insubstantial, but has been demonstrated to be very premature , 9. In pDiverse approaches have been made in their treatment, the most common being follicle removal, together with the associated dental organ. The single enucleation of the hyperplastic tissue has a variable success rate in inducing affected tooth eruption, and it is better to accompany it with orthodontic traction , 12. DurWith respect to pathological features, macroscopically, a grating sound during sectioning , 9, 14 aIn the cases of young patients (≤11 year old), there is a loose fibrous connective tissue which seems to become denser as age progresses , 13. In As for calcifications, in some cases a certain type predominates , 12, 14;Type II calcification has at least three arrangements: 1) a swirling spindle cell pattern intermixed with mineral deposit areas presenting tufting on borders, and well-defined interfacing with surrounding stroma ; 2) a wiType II calcification is fibrillar in nature, as demonstrated by polarized light , 12 and It has already been proposed that the possible origin of calcifications could come from mesenchymal cells, which can differentiate into cementoblasts or osteoblasts , 26, andAttempts have already been made on several occasions to clarify the difference between Calcifying or not, Hyperplastic Dental Follicles (HDF) with Simple or WHO type Central Odontogenic Fibroma (COF). The succinct difference in the COFs, in addition to the cementoid calcifications and the odontogenic epithelium, is the greater cellularity, greater vascularity and the occasional presence of dentinoid or osteoid material , 29, 30.The study of MCHDF is not without controversy. A few years ago, an interesting and provocative critique of an article was published, in which it was suggested that MCHDF may have descriptions dating back from 1945 . CriticsIn conclusion, a definitive diagnosis must be supported by clinical-radio-pathological correlation because there exist similar lesions such as multiple hyperplastic dental follicles  , 42 or s


A 32-year-old male presented with painless proptosis and diminution of vision in left eye. Imaging shows multiple well-defined masses, suggestive of cavernous haemangioma, in orbit and cranium with adjoining bones being thickened with cystic spaces. Histopathology proved the diagnosis. Cavernous haemangioma usually presents as a solitary intraconal and sometimes extraconal mass with vision usually preserved unless it extends to the apical portion. Here we report a rare case of multiple simultaneous locations of cavernous haemangiomas in orbit and cranium with significant diminution of vision. Cavernous hemangioma is the most typical orbital tumor in adults in their third or fourth decade. It usually presents as a solitary tumor rarely affecting the vision. Treatment is straightforward with complete excision by orbitotomy surgery. However, multiple cavernous hemangiomas of orbit and cranium are rare but possible presentations, making it a diagnostic dilemma and management difficulty.A 32-year-old male presented with left sided gradual onset painless progressive forward protrusion of eyeball associated with gradual onset diminution of vision in same eye since four years.On examination, the best-corrected visual acuity in the right eye (RE) was 20/20, while in the left eye (LE) was 20/400. Anterior segment and fundus examination of RE was within normal limits.Examination of LE shows the fullness of the upper and lower lid with prominent eccentric proptosis. The eyeball was protruded four mm axial and three mm downward displaced, as shown in Intraoperatively, we found multiple solid, solitary dark red colored lesions , which w1 Alan Mc Nab et al. states that it usually presents as a solitary intraconal lesion in 87% and sometimes as extraconal mass. In their study in 104 patients, all had single cavernous hemangioma in various parts of the orbit.2 A study also shows that 33% of patients have optic nerve dysfunction as well,3 which is likely in our case as well in view of reduced vision, RAPD, and disc pallor. Multiple cerebral cavernous hemangiomas have been reported in a child presenting with seizures, with most lesions located in supratentorial, frontal, and temporal lobes. These remain asymptomatic until intracranial hemorrhage occurs.4 Therefore, a close follow-up is required in a case like ours where intracranial hemorrhage may occur.Cavernous hemangioma is the most common benign vascular tumor of the orbit in adults.8 However, ours is the only case report with simultaneous presentation of multiple and multifocal presentations of cavernous haemangioma of orbit and cranium. According to a report by Brunereau L et al., our patient may have had the hereditary type of this disease given the tendency to form multiple lesions is seen in the hereditary rather than the sporadic type,8 however he had no family history. Complete surgical excision is a preferred treatment. Gamma knife radiotherapy and stereotactic radiation have also been tried for surgically challenging apical cases impeaching optic nerve with 76% reduction in the tumor volume.9 We would like to conclude by saying that cavernous haemangioma can rarely present multiple lesions in multiple sites in and outside the orbit.Multiple intraosseous cavernous haemangiomas similar to our patient is also reported by other studies in various locations like skull, spine, and inferior orbital rim.


Mesenteric lipodystrophy is a rare fibroinflammatory disease of unknown origin with clinical and radiological non specific findings.The case of a 42-years-old man affected by a pedunculated mesenteric lipodystrophy mimicking Meckel’s diverticulum is reported. Clinical, imaging and histological findings are discussed.Mesenteric lipodystrophy affects the mesenteric fat of the abdomen with a typical diffuse thickening of the mesentery, nodular thickening of the mesenteric root and presence of mass-like lesions. Ultrasound (US) and Multiphasic Computed Tomography (CT) represent the main imaging tools used for diagnosis. Clinical and imaging findings can mimick other pathological conditions affecting the mesenteric fat tissue. Contrast enhanced CT is the most accurate imaging technique for diagnosing mesenteric lipodystrophy due to the high panoramicity and accuracy with multiplanar imaging. Multiphasic technique helps to characterize the lesion and to recognize vascular anatomy. Oral administration of iodinated contrast medium may help to assess the relationship with bowel loops. All these diagnostic elements are crucial for the surgical timing and approach.Due to the heterogeneous mesenteric involvement, the nonspecific CT findings and the high number of diseases for differential diagnosis, the detection of mesenteric lipodystrophy is challenging and requires early clinical suspicion. An histological examination is always necessary. Nowadays most authors accept that these different entities represent a spectrum of the same disease.The etiology is unclear; however, previous abdominal trauma, surgery, mesenteric ischemia or infection have been proposed as potential causes .Clinical onset is subtle, with nausea, abdominal tenderness, fever, asthenia. Computed tomography (CT) represents the most accurat examination to achieve diagnosis being crucial also to define any surgical approach. However, CT findings are often nonspecific and need to be correlated to the clinical information and often requires biopsy and histological examination for a definitive diagnosis . The wor2A 42 years-old Caucasian man came to our emergency department with abdominal tenderness in the lower right abdominal quadrant and elevation of blood index of inflammation.An abdomino-pelvic ultrasonography (US) was performed. US shown a tubular structure in the right iliac fossa, with thickened wall and a central area of inhomogeneous echogenicity. Perilesional fluid layer was also detected .Fig. 1USThe US findings suggested an appendicitis, therefore the patient underwent CT examination.A 320-row CT scanner was used .CT acquisition was performed from the diaphragmatic domes to the pubic symphysis, using a triphasic technique after the intravenous injection of 1.5 mL/kg of Iopamidol  at 3.5 mL/sec through the ante-cubital vein using an automatic power injector.In order to evaluate bowel loops, a gastrografin solution, prepared by diluting 50 mL to one liter with water, was orally administered 20 min before the CT scan.All CT data were transferred to a workstation (HP XW 8600) equipped with dedicated software for image reconstructions .Abdomino-pelvic CT showed a normal retrocecal appendix.The sonographic finding corresponded to a well-demarcated hypodense tubular structure, 7 cm in length and 3 cm in width, strictly connected to a small bowel loop. After the intravenous injection of contrast medium, the lesion demonstrated hyperdense thickened walls. Perilesional fat strands were also detected into the adjacent mesentery. No other pathologic findings were identified in the abdomino-pelvic district .Fig. 2CoThe hypothesis of an inflamed Meckel’s diverticulum was performed, according to the tubular structure of the lesion, with hyperdense walls and perilesional mesenteric fat strands.The patient underwent surgery. After a laparotomic approach, the lesion was mobile in the peritoneal cavity, connected to the small bowel mesentery by a fibrovascular pedicle. The lesion appeared as a fibrolipidic mass, showing vascular congestion due to the torsion around its pedicle causing the clinical onset. Therefore, the pedicle was clamped and the mass was excised .Fig. 3SuPost-operatory course was uneventful and after a progressive improvement of the clinical condition, the patient was discharged after six days.Histological examination revealed fat necrosis with lipid-filled macrophages associated to a dense lymphocyte infiltration and fibroblastic proliferation.The CD117, SM-Actin, CD34, S-100 Protein markers were normally expressed, hence the pathological diagnosis was mesenteric lipodystrophy.3Mesenteric lipodystrophy is a rare fibroinflammatory condition of the mesenteric fat of the abdomen. Since its first description by the Italian surgeon Jura in 1924, variations of the same pathological process have been reported under many names: sclerosing mesenteritis, mesenteric panniculitis, retractile mesenteritis, mesenteric lipodystrophy, mesenteric lipogranuloma1.This ambiguous nomenclature reflects the progression from fat necrosis to fibrosis of the mesenteric adipose tissue.In 1997, after a review of 84 cases, Emory et al. concluded that these histological variants are parts of the same disease and that “sclerosing mesenteritis” would have been an appropriate common nomenclature [Mesenteric involvement can be divided into three different types: diffuse thickening of the mesentery, knotty thickening of the root of mesentery and mass-like lesion.Histologically, the mesenteric fat involved in this fibroinflammatory condition shows a progressive series of changes into three sequential phases. In the early phase of the process, the mesentery is characterized by fat necrosis with lipid-filled macrophage infiltration within the septa of the mesentery with absent or minimal inflammation, hence the name of mesenteric lipodystrophy .Then, as the inflammation process progresses, fat necrosis is associated to a dense inflammatory cell infiltration . This aspect suggests the name of mesenteric panniculitis.Finally, fibrosis occurs with consequent shortening and distortion of the mesentery. This final phase can be considered as an end stage process, a condition also known as retractile mesenteritis .Mesenteric lipodystrophy affects most commonly patients from the fifth to seventh decades of life, with a male-female ratio of 2:1.The incidence based on autopsy and CT findings varies from 0.4 to 1% .The etiology is unknown. However, several conditions are suggested as potential causes: prior abdominal trauma or surgery, mesenteric ischemia, autoimmune diseases, tuberculosis, pancreatitis, vasculitis, granulomatous diseases and malignancies  .The correlation between mesenteric panniculitis and a neoplastic condition represents a controversial topic in the medical literature. In fact, many authors report that a correlation is possible by interpreting the involvement of the mesentery as a paraneoplastic syndrome. Scheer et al.  describeOther authors do not recognize this correlation. About that, Gogebank et al., in a case–control study, refused the prevalent notion that mesenteric panniculitis is a paraneoplastic phenomenon and demonstrated that the degree and course of mesenteric panniculitis are not correlated with tumor stage or development . SimilarSclerosing mesenteritis is sometimes associated with a multisystem clinical syndrome affecting the lungs, liver, biliary tract, pancreas and kidneys; this condition has been described as “IgG4-related systemic sclerosing disease” due to the presence of IgG4+ plasma cells within involved tissues .The clinical presentation of sclerosing mesenteritis is heterogeneous and often non-specific, sometimes is totally asymptomatic and incidentally detected. Abdominal tenderness, nausea, vomiting, fever, fatigue are prevalent in the case of a diffuse mesenteric involvement; palpable abdominal mass and symptoms of bowel obstruction are described in the case of a mass forming process. If the mass is pedunculated, the torsion around the pedicle could be a fearing complication, compromising its vascularization and leading to ischemia ,13.Mesenteric lipodystrophy is often an incidental diagnosis; it shows 0.6% prevalence in patients undergoing abdominal computed tomography , which iMultiphasic CT with contrast enhanced technique is required in order to better characterize the lesion and to evaluate the degree of distortion of mesenteric vessels, which represent crucial informations for the surgical approach and for evaluating ischemic complications.In order to assess the dislocation and compression of bowel loops, an oral administration of hydrosoluble contrast medium 20 min before the scan could be useful.CT findings vary widely basing on the phase of fibroinflammatory process at the moment of diagnosis and on the main pathological aspects of mesenteric involvement.A diffuse thickening of the mesentery is associated to increased fat density, which can be diffuse (“misty mesentery”) or can envelope the mesenteric vessels preserving a normal fatty area around them (“fat ring sign”). Mesenteric increased fat density is not specific for mesenteric lipodystrophy and can mimic other condition such as edema, carcinomatous lymphangitis and inflammatory bowel diseases .A knotty thickening of the mesentery is associated to well-defined soft tissue nodules, usually less than 1 cm, associated to fat strand densities around the vessels. This condition must be differentiated from lymphadenopathies, tuberculosis, peritoneal carcinomatosis, mesothelioma and granulomatous diseases (Weber-Christian disease) .A mass-like lesion often involve the small bowel mesentery. It is characterized by a fibrolipidic mass, usually surrounded by an hyperdense pseudocapsule (60% of cases), which may also have a sovrafluid component as a result of intralesional fat necrosis. Usually, in the central necrotic portion of the mass, calcification may be present and may be related to the fat necrosis .These pseudotumors can grow into the mesentery or can be pedunculated; the latter are characterized by a fibrovascular pedicle and an esophitic growth into the peritoneal cavity. Torsion of the pedicle may cause severe ischemic complications .Depending from their size and location, these abdominal masses are associated to various grade of mesenteric vessel distortion and various grade of dislocation or compression of the bowel loops .Mesenteric lipodystrophy presenting as a mass lesion must be differentiated from a gastrointestinal stromal tumor (GIST), lymphoma, lipoma, liposarcoma, Meckel’s diverticulum.Meckel's diverticulum is an intestinal diverticulum that results from incomplete obliteration of the most proximal portion of the vitelline or omphalo-mesenteric duct during the fifth week of fetal development . The nonSonography isn’t useful for the study of mesentery; it can play a role only in detecting mesenteric mass, which are well defined and hyperechoic due to the fat content. The fatty content of the mass could reduce sonographic transmission, limiting the acquisition of information. If areas of necrosis occur, the mass shows central hypoecogenicity.Ultrasonography is less invasive and money saving, but the lacking of panoramicity and the poor diagnostic information strongly limit its role into a first level examination.As regards the therapy, there are not specific algorithms for mesenteric lipodystrophy; nowadays the treatment options are empiric and include the use of corticosteroids, colchicine, azathioprine, thalidomide, cyclophosphamide, tamoxifen and progesterone .Surgical approach is reserved for complications such as bowel obstruction and vascular distortion with consequent ischemia of the mesenteric mass or ischemia of bowel loops ,19.4Contrast enhanced CT is the most accurate imaging technique for diagnosing mesenteric lipodystrophy due to the high panoramicity and accuracy with multiplanar imaging. Multiphasic technique helps to characterize the lesion and to recognize vascular anatomy. Oral administration of iodinated contrast medium may help to assess the relationship with bowel loops.All these diagnostic elements are crucial for the surgical timing and approach.Due to the heterogeneous mesenteric involvement, the nonspecific CT findings and the high number of diseases for differential diagnosis, the detection of mesenteric lipodystrophy is challenging and requires early clinical suspicion. An histological examination is always necessary.None.The study is exempt from ethical approval at our institution.Written informed consent obtained for case report and image publication.All authors conceived and designed the study, acquired the data, analysed and interpreted the data and drafted the manuscript. All authors revised the manuscript. All authors read and approved the final manuscript.This is not a first-in-man study.Alessandra Mirabile Marco Moschetta.Not commissioned, externally peer-reviewed.None.


Intestinal obstruction caused by a tumor is very rare in newborns, and the preoperative diagnosis is difficult. We herein report a rare case of neonatal colonic obstruction due to solitary intestinal myofibroma with characteristic findings on gastrografin enema and the surgical strategy.A 4-day-old female infant presented to our neonatal intensive-care unit with abdominal distention and bilious vomiting after feeding. A gastrografin enema showed that the transverse colon near the hepatic flexure was not delineated at the oral side. When pressure was applied, a small amount of contrast material moved into the mouth in the form of threads. Microcolon was not observed, and stenosis of the transverse colon was found 9 cm from the Bauhin valve. Partial resection and end-to-end anastomosis were performed. A pathological examination of the resected specimen suggested gastrointestinal stromal tumor (GIST). After obtaining a second opinion, the histology and immunohistological markers were deemed characteristic of infantile myofibroma.If string sign and a napkin ring appearance are found in a case of neonatal intestinal obstruction, surgery should be performed with a tumor in mind. In cases of neonatal intestinal obstruction caused by a tumor, the lesion should be resected with a sufficient surgical margin before the pathological examination. Neonatal intestinal obstruction may be due to a variety of conditions, including atresia and stenosis, annular pancreas, malrotation, duplication cyst, meconium ileus, meconium plug syndrome and neonatal small left colon syndrome, Hirchsprung’s disease, tumor, trauma, and other rarer causes . HoweverInfantile myofibromatosis, although a rare disorder, is a common tumor of infancy and childhood . The disWe herein report a rare case of neonatal colonic obstruction due to SIM with characteristic findings on gastrografin enema and the surgical strategy.The present patient was a 4-day-old female infant. The mother had had a natural pregnancy. The patient had been born by vaginal delivery at a gestational age of 39 weeks 3 days with a birthweight of 3124 g. The passage of meconium was observed soon after the birth. The patient presented on day 4 of age to our neonatal intensive-care unit with abdominal distention and bilious vomiting after each feed. Her vital signs were stable, and she was slightly inactive and mildly dehydrated. Upper gastrointestinal series showed a normal appearance. Gastrografin enema showed that the transverse colon near the hepatic flexure was not delineated on the oral side. When pressure was applied, the string sign was observed . A further consideration, including an immunohistochemical analysis revealed positive staining for SMA, CD34, DOG-1, and SDHB and negative staining for c-kit, S-100, β-catenin, pan-tropomyosin resepter kinase (pan-TRK), analplastic lymphoma kinase (ALK), and c-ros oncogene 1 (ROS1). The histology and immunohistological markers were characteristic of infantile myofibroma.The patient had an uncomplicated postoperative course and was discharged home after 3 weeks. Whole-body magnetic resonance imaging (MRI) and gastrografin enema performed 6 months after the operation showed no recurrence of the lesion and no other new lesions.Neonatal intestinal obstruction occurs 1 in 1500 live births . IntestiOur case showed characteristic findings on gastrografin enema. Although the preoperative findings were atypical for intussusception, emergency surgery was performed. The intraoperative findings showed a napkin ring appearance. A “napkin ring” appearance is used to describe the morphology of the gastrointestinal tract in cases of circumferential stenosis forming a ring , and it Initially, GIST was suspected on the pathological examination of this case. Additional resection was, therefore, considered. After obtaining a further consideration, including an immunohistochemical analysis, the final diagnosis of SIM was made. Additional resection is highly invasive in all patients, not just neonates, but the risk of short bowel syndrome is increased in neonates compared with older patients. In a single-center review, the ratio of benign to malignant pediatric gastrointestinal tumors was 9:4 . In geneThe prognosis for solitary myofibroma cases is good with complete resection . No repoWe herein report a rare case of neonatal colonic obstruction caused by SIM. This is the first case in the world in which a “napkin ring” was confirmed by neonatal gastrografin enema. If neonatal intestinal obstruction includes enema findings of string sign and the “napkin ring” appearance, a tumor should be considered. In cases of neonatal intestinal obstruction caused by a tumor, the lesion should be resected with a sufficient surgical margin before the pathological examination.


Liposarcomas are exceedingly rare entities that evoke malignant transformation of connective tissue and fat cells. These tumours occur throughout the soft tissues of the body, afflicting a myriad of regions. In the adult population, liposarcomas represent the most prevalent subtype of sarcomas, and often arise de novo. Retroperitoneal liposarcomas (RLS) are a ubiquitous subset of sarcomas that, due to their deep location in the hollow abdomen, can grow to astronomical proportions before manifesting any noticeable symptoms; a prompt diagnosis of RLS is therefore often rendered dilatory. We hereby delineate the case of a 43-year-old woman who presented with vague left hemiabdominal distention and discomfort. A subsequent computed tomography scan divulged a giant retroperitoneal growth impaling on and thus displacing the pancreas. A compartmental, en bloc resection was performed, with subsequent histopathology of the excised specimen revealing a well-differentiated liposarcoma. The surgical intervention was curative and led to an uneventful recovery. This paper highlights the pertinence of surgical management as an appropriate treatment modality for a complete resection of RLS.  Liposarcomas are malignant tumours of mesenchymal cells that can infiltrate a plethora of soft tissues in the body. Retroperitoneal sarcomas encompass tumours that are mesenchymal in origin and arise from the retroperitoneum. Of all retroperitoneal sarcomas, retroperitoneal liposarcomas (RLS) are established to be the most prevalent . Due to We delineate the case of a 43-year-old female who presented to our department with a two-year history of persistent discomfort in her left abdomen and associated vague abdominal heaviness. The patient had no comorbid conditions, and her past medical and surgical histories were unremarkable. Since the inception of her symptoms, she had experienced concomitant constipation for which she had seen a physician in her remote village. Due to an acute presentation, and no associated red-flag signs, the physician attributed the constipation to the patient’s history of consuming a diet bereft of high-fibre foods. In the six months that followed, the patient began to appreciate a painful, poorly demarcated abdominal lump, for which she ultimately underwent an ultrasound one month prior to presenting to us. The ultrasound divulged an abnormal growth in the left hemiabdomen; further imaging, however, was deemed necessary in order to yield a definitive diagnosis. At presentation, the patient had a huge, palpable abdominal mass that was firm and non-tender. The mass was noted to be in the left hemiabdomen, and appeared to extend from her left hypochondrium to the left iliac fossa, ostensibly traversing the midline. Thereafter, the patient was advised to under undergo a computed tomography (CT) scan of her chest, abdomen, and pelvis. The contrast-enhanced CT scan revealed a giant retroperitoneal mass, measuring 50 x 40 x 30 cm. The CT scan showed that the giant retroperitoneal growth was boring behind the pancreas, displacing it supero-anteriorly. Furthermore, the tumour was also noted to be impaled on the splenic hilum, thereby displacing the spleen laterally Figure .The CT scan further revealed that the retroperitoneal tumour was in fact completely encasing the left kidney, displacing it medially and across the midline. Moreover, based on the CT findings, it was aptly construed that the massive retroperitoneal growth was indeed crossing the midline itself, entirely encasing the splenic flexure and the descending colon Figure . A subsequent CT-guided core biopsy revealed a well-differentiated liposarcoma. Due to the extensive encasement of adjacent structures by the tumour, the case was discussed at a multidisciplinary team meeting (MDT), which involved the pathologist, an oncological surgeon, general surgeon, and the radiologist. A surgery was promptly planned, and an en bloc compartmental resection approach for the excision of the tumour and the encased structures was performed Figure . A successful en bloc resection of the mass, with the encased kidney and involved colon, yielded the specimen Figure . AdditioThe excised sample was subsequently sent for histopathology, which revealed fibroadipose and fibromuscular tissue infiltrated by an adipocytic lesion composed of variably sized mature adipocytes and bands of fibrotic stroma containing atypical spindle to stellate stromal cells. Enlarged, hyperchromatic nuclei were appreciated with prominent nucleoli and scant mitoses. The spindle cells, in particular, demonstrated mild atypia on a background of present lipoblasts. Sections examined revealed that both the attached bowel and kidney wall were tumour-free.Post-operatively, the patient's recovery was uneventful and the patient was discharged on the fourth day post-operatively. To date, at one-year follow-up, the patient continues to thrive and do well, with no recurrence(s). Liposarcomas are specific subtypes of sarcomas that involve the soft tissues . AlthougRLS generally present with a progressive increase in abdominal girth that does not subside ,5. PatieDespite the requirement of negative surgical margins involving resection of the pseudocapsule, en bloc resection presents a surgical quandary owing to the close proximity of the tumour to vital organs, such as the great vessels . As a reDue to their location in the hollow abdomen, RLS often present late in the disease course, remaining clinically silent and thus evading prompt detection. Clinical manifestations elicited by retroperitoneal sarcomas are often vague, and most often present only with exorbitant tumour growth and subsequent encasement of the adjacent structures. En bloc resection is thus warranted, presenting an exacting surgical challenge.


Hepatocellular carcinoma (HCC) arising from hepatic adenoma is an infrequent situation. Only a few cases were reported in the literature. We present a rare case of hepatocellular carcinoma arising from HA in a young woman with no medication history of oral contraceptives. Surgical resection is the only available treatment. Hepatocellular carcinoma (HCC) arising from hepatic adenoma is an infrequent situation. Only a few cases were reported in the literature. We present a rare case of hepatocellular carcinoma arising from HA in a young woman with no medication history of oral contraceptives. Surgical resection is the only available treatment. Most of these tumors are detected incidentally during imaging for not related pathology. However, patients may present with abdominal discomfort or pain, hepatomegaly, and intraperitoneal hemorrhage in case of spontaneous tumor rupture.2A 35‐year‐old married housewife, who lives with her husband and her three children in the city, was referred to us because of right upper quadrant abdominal discomfort and intermittent fever for the last 6 months. There was no history of drug or alcohol intake. Her background was significant for cesarean section, with no history of any medication or oral contraceptive use. On examination, an immobile and tender palpable epigastric mass was found, the temperature was 37.2°C, blood pressure was 120/80 mm Hg and pulse was 70 bpm. Abdominal ultrasound revealed a 15 × 9 cm sized epigastric mass  levels about two times the upper limit of normal . Gamma‐glutamyl transferase (GGT), aspartate aminotransferase (AST), alanine aminotransferase (ALT), and total bilirubin levels were normal. Other parameters, including leukocytes, hemoglobin, platelet counts, and renal function tests, were normal. Serum tests for human immunodeficiency virus (HIV) and hepatitis C virus were negative. Hepatitis B markers were positive for hepatitis B surface antigen and hepatitis B core antibody. The viral load was undetectable. Alpha‐fetoprotein level was very high . Tests for serum tumor markers were negative for CEA and CA19‐9. Fine needle aspiration cytology showed typical features of trabecular hepatocellular carcinoma including liver cell cords that were one to three cells thick and nuclear atypia Figures  associat3We reported a 35‐year‐old woman with no medical history of oral contraceptives use, who presented with abdominal pain and a palpable abdominal mass. Gadoxetate‐enhanced MRI revealed a 15 × 11 × 9 cm heterogeneous encapsulated lesion arising from the left hepatic lobe. Pathological analysis of the tumor revealed HCC arising from hepatic adenoma. The patient underwent a left hepatectomy with no complications. Clinical and abdominal ultrasonography follow‐up revealed no evidence of recurrent disease. The originality of this case report stems also from the fact that hepatic adenoma malignant transformation occurred in a hepatitis B inactive carrier with no cirrhosis.Hepatic adenomas are uncommon, rare benign liver neoplasms that occur more frequently in young women with a medication history of oral contraceptives. The incidence of HAs is 3‐4 per 100 000 women.Although HAs are benign lesions, they do have a potential of malignant transformation. The adenoma‐carcinoma sequence is defined by the presence of foci of dysplasia within adenomas, which predispose to carcinoma in situ and epithelial cancers.4Hepatic adenomas are uncommon, benign liver lesions typically affecting young women of childbearing age. Despite the fact that they are benign neoplasms, they can become malignant. Rare cases of hepatocellular carcinoma arising from hepatic adenoma with no history of oral contraceptives use were reported in the literature. Surgical resection of hepatic adenoma is the only treatment available for this type of tumor.None declared.Nadia Maamouri: contributed to concepts; Haythem Yacoub, Nadia Maamouri, and Hela Kchir: contributed to design; Haythem Yacoub, Nadia Maamouri, Slim Haouet, and Hela Kchir: contributed to definition of intellectual content; Haythem Yacoub: contributed to literature search; Haythem Yacoub, Nadia Maamouri, and Asma Ayari: contributed to manuscript preparation; Nadia Maamouri and Haythem Yacoub: contributed to manuscript editing; Haythem Yacoub, Hajer Hassine, Nadia Maamouri, Hela Kchir, Slim Haouet, Habiba Mizouni, Mohamed Taher Khalfallah, and Sabeur Mannai: contributed to manuscript review; Haythem Yacoub served as guarantor.Yes.


Aspergillosis, aka fungal ball (FB), is classified as a type of non-invasive fungal rhinosinusitis, which usually occurs unilaterally in the maxillary sinus of an immunocompromised patient. Its diagnosis is complex and depends on the association between clinical, imaging, and histopathological exams. There are many treatments for fungal infections of the paranasal sinuses, so early diagnosis is extremely important to determine the appropriate treatment. This paper reports an unusual clinical case of aspergillosis present bilaterally inside the maxillary sinuses of a healthy patient, associated with mucous retention cysts, whose imaging exams and transnasal endoscopy were not sufficient to precisely identify the lesion. Its diagnosis and definitive treatment were obtained only after orthognathic surgery and integration between a multidisciplinary team. Key words:Aspergillosis, communicable diseases, fungal ball, infectious disease, orthognathic surgery. The Fungal Ball (FB), or aspergillosis of the maxillary sinuses, is classified as a non-invasive fungal infection caused mainly by the species Aspergillus fumigatus ,2. This The infection occurs in the maxillary sinuses of immunocompromised patients and is usually exclusive (without associated pathologies) and unilateral, with rare bilateral lesions ,3,4. CliThe treatment of this pathology is surgical, either by nasal endoscopic surgery or by direct access to the lesion. The complete removal of the fungal ball is sufficient to allow natural sinus drainage; however, the use of antifungal medication is also indicated ,9,10. ThA 42-year-old female patient was referred to the Oral and Maxillofacial Surgery team, with an indication for orthognathic surgery. During anamnesis, she reported a history of chronic sinusitis, which triggered headaches, facial pain, constant episodes of rhinorrhea, and breathing difficulties. The patient also reported having undergone treatment with an otorhinolaryngologist for six years. During this period, several antibiotic prescriptions and nasal endoscopy exams were performed Fig. A,B, howeThe collected material was sent for histopathological analysis, which confirmed the diagnosis of fungal balls (predominantly Aspergillus sp) Fig. B,C and mThe sinus fungal ball, usually found in the maxillary sinus, can develop via respiratory or odontogenic routes . From a Aspergillosis is generally unilateral, with a prevalence of approximately 94% . In addiComputed tomography (CT) is an important complementary exam to assist in the differential diagnosis, however, alone, it is not enough to generate the definitive result. Hypotheses such as anthrolytes, osteomas, mucoceles, B cell lymphoma, squamous cell carcinoma, cystic adenoid carcinoma, and inflammatory myofibroblastic tumors are lesions of possible similarities in imaging exams . AuthorsAs for the treatment of the fungal ball, the surgical techniques of Caldwell-Luc and endoscopic techniques may be used, aiming at the complete removal of the infection ,10. In oAlthough there are reports in the literature of orbital impairment by aspergillosis, the development of epiphora due to nasolacrimal duct obstruction is a rare symptom, and when it occurs, it tends to be due to progression of aspergillosis to the sphenoid sinuses and in cases of invasive arpergillosis . In the It is worth mentioning the importance of early diagnosis and the correct classification of fungal sinusitis since the management and prognosis are very different . Therefo


Elastofibroma dorsi (ED) is a benign connective tissue tumor that most commonly occurs on the inferior pole of the scapula. It can be found incidentally on radiologic imaging or due to clinical symptoms. Patients may become apprehensive due to it mimicking new malignancy or recurrence of prior malignancy. Treatment is only recommended in symptomatic cases and biopsy is usually unnecessary. We present a case of a 70-year-old female status-post lung cancer resection who was found to have a lump at the inferior pole of her right scapula. She was seen by multiple different specialties and subsequently, a biopsy confirmed her mass to be consistent with ED. Since ED is a benign soft tissue tumor, educating physicians is of utmost importance to avoid pursuing unnecessary diagnosis and to thereby decrease the cost of care to the patient. Therapeutic excision should only be performed in symptomatic patients and observing these lesions in asymptomatic patients would be sufficient. Elastofibroma dorsi (ED) is a rare benign connective tissue tumor that is typically found on the inferior pole of the scapula . First dA 70-year-old Caucasian female with a past medical history significant for severe chronic obstructive pulmonary disease (COPD), lung cancer status-post resection 15 years prior, and osteoporosis presented to the urgent care in March of 2019 complaining of a lump on the right side of the upper back. This happened to be the at the site of a previous right-sided thoracotomy where she underwent segmentectomy of the right upper lobe, and a right upper lobe lobectomy in 2004. No radiation or chemotherapy was administered at the time of surgery and she had remained cancer free with no reoccurrence based on her yearly pulmonary follow ups. Upon initial presentation, she stated that she had noticed the mass over the past year and although it was painless, it had raised concern due to its progressive size. She denied fever or recent history of trauma. On physical examination, the surgical scar from the thoracotomy appeared well healed with no signs of infection. A non-tender mass was palpated just under the surgical scar and at the time of exam, measured approximately 2-3 cm in diameter but became more prominent with movement of the scapula. U/S showed a 3 cm complex mass and magnetic resonance imaging (MRI) was recommended for further evaluation (Figure Her MRI showed a heterogeneously enhancing mass along the right posterior lateral chest wall Figure . The masThe patient was evaluated by oncology and at that time, the differential diagnosis included elastofibroma, desmoid tumor, sarcoma, and recurrent lung cancer. A CT-guided biopsy was performed, and the pathology report was consistent with a diagnosis of elastofibroma. The patient's oncologist recommended no surgical intervention of the mass due to its benign nature.During the 12th Histopathology Scandinavian Congress in 1959, ED was first described. It was postulated that this soft tissue growth was due to degenerative and regenerative processes along the thoracic fascia. Later in 1969, Jarvi et al. published an in-depth manuscript and confirmed that scapular movements along the thoracic fascia cause friction which leads to the development of the mass. Gross histopathological features also provide sufficient reasoning for the slow growth of this mass . In 1987Women over the age of 50 have a higher occurrence of ED -10. The Diagnostic modalities include ultrasonography, MRI, and CT. U/S can be an extremely useful modality in conjunction with an astute clinician. No further imaging is necessary if the mass has regular boarders, is not cystic and fits the classic presentation of ED: a slow-growing subscapular mass in a woman over the age of 50 with or without previous risk factors ,15. On UED is a benign soft tissue tumor which occurs most commonly in elderly female patients. Clinicians with awareness of this tumor can save time and avoid pursuing extensive work up by using an U/S and a thorough physical exam. Rarely is it found to be symptomatic and even then, it merely causes discomfort with mobilization. If asymptomatic, close observation of the mass is sufficient.


Acute mesenteric ischemia (AMI) refers to the sudden onset of intestinal hypoperfusion that can also result from splanchnic venous occlusion. The portomesenteric venous system (PMVS) is an unusual site of thrombosis in patients with protein S deficiency and its obstruction is a rare cause of AMI. Aim of this report is to illustrate a successful strategy in a case of massive small bowel infarction managed with an open abdomen (OA) approach.A 64 year-old woman presented to the emergency department with acute abdominal pain, rectal bleeding, diarrhea and vomiting. Contrast-enhanced computed tomography (CECT) showed small bowel ischemia and the complete occlusion of all the PMVS branches. Surgery was performed with an OA approach and anticoagulation was immediately begun. Further workup revealed isolated protein S deficiency and history of atrophic gastritis. Thromboprophylaxis with warfarin was started on discharge and no recurrence of thrombotic events was recorded during the one-year follow-up.PMVS thrombosis related to protein S deficiency is a rare condition that can rapidly lead to an acute abdomen. CECT is the gold standard, because it detects splanchnic thrombosis and its possible complications, like bowel ischemia. In case of surgery, a planned second-look operation is the best strategy to assess bowel viability and possible ischemic progression.OA management plays a fundamental role in case of resection for bowel ischemia. Patients with thrombosis at an uncommon site should be further investigated for prothrombotic states. The repThe clinical presentation is variable, ranging from asymptomatic to non specific abdominal pain to acute abdomen.The gold standard for its detection is Contrast-Enhanced Computed Tomography (CECT), which also can reveal other associated conditions like bowel ischemia .PMVS thrombosis is generally caused by the association of inherited or acquired prothrombotic conditions and local causes, but sometimes its origin remains idiopathic.Protein S is a vitamin K dependent anticoagulant. Its deficiency can present with recurrent episodes of venous thromboembolism and its prevalence in patients with splanchnic vein thrombosis is 2,6 % . TrombopIn this paper, is presented a case of massive small bowel infarction due to acute PMVS thrombosis in a 64 year-old woman with history of chronic athrophic gastritis and protein S deficiency, successfully treated with a multidisciplinary approach based on the surgical open abdomen management.This study has been reported in line with the SCARE criteria .2A 64 year-old woman presented to the emergency department complaining of severe diffuse abdominal pain, associated with multiple episodes of vomiting, rectal bleeding and diarrhea. Pain was acute, dull, non-radiating, increasing in severity in the last 24 h. She had no relevant medical history except for a microcytic anemia and an atrophic gastritis with chronic Helycobacter Pylori (HP) infection. The physical examination revealed abdominal distention and rebound tenderness in epigastrium and right quadrants, positive Blumberg’s sign and absence of bowel sounds.3, an hemoglobin of 8,5 g/dL with a mean corpuscolar volume of 582 fL, prothrombin time/international normalized ratio of 17,8 s/1,3, protein level of 3,5 g/dL and albumin of 21 g/L.Blood tests showed a white blood cell count of 13,600/mmArterial blood gas analysis showed 2,7 mMol/L  lactate acid level.An US examination showed free abdominal fluid and portal vein thrombosis. Hence a CECT was performed and revealed a massive thrombosis of right intrahepatic portal branches, portal vein, superior and inferior mesenteric veins with associated extended acute venous ischemia of the small bowel, colonic wall thickening with normal contrast enhancement and abundant peritoneal effusion .Fig. 1A,The patient was referred to the emergency surgery department. An explorative laparotomy revealed the presence of abundant bloody ascitic fluid and edema of the mesentery and of the entire small bowel; furthermore evidence of venous infarction of about 130 cm of the terminal ileum was found, and thus surgical resection was performed. The bowel was left in discontinuity and an open abdomen management was adopted to better assess intestinal viability. After surgery, the patient was transferred to the Intensive Care Unit (ICU). Antibiotic prophylaxis, blood transfusions, total parenteral support nutrition, as well as anticoagulation with low-molecular-weight heparin infusion were immediately initiated. The second look surgery was planned 48 h after the first operation and a terminal ileostomy was performed. The patient was extubated the following day and discharged from ICU two days after extubation.The following work-up showed no evidence of malignancies or other acquired risk factors for PMVS thrombosis except for an atrophic gastritis with chronic HP infection. Investigations to detect any prothrombotic disease revealed only a mild protein S deficiency.The patient was discharged from the hospital on the 15th postoperative day, after switching anticoagulant therapy on warfarin; new oral anticoagulants could not be administered for an acute kidney injury likely due to dehydration because of the ileostomy. Besides, the patient had begun the HP eradication therapy.After three months, the PMVS thrombosis was completely resolved  and the At the 1-year follow-up, the patient was in good clinical condition, with no further episodes of thrombosis, HP eradication confirmed at gastric biopsy and improvement of microcytic anemia and creatinine levels.3The PMVS refers to the vessels involved in the drainage of the gastrointestinal tract and spleen and it’s composed by superior and inferior mesenteric veins, portal vein and splenic vein.PMVS thrombosis is a rare disease, with a global mortality rate of 20–50 % [CECT is the technique of choice in detecting both filling defects inside the venous vessels and ischemic changes in the bowel .PMVS thrombosis is often the result of a combination of local and systemic risk factors that could be divided into inherited or acquired .Table 1PMost common inherited risk factors for PMVS thrombosis have a prevalence >2% in general population and carry a relatively low risk of splanchnic thrombosis, while more rare conditions, like protein S deficiency, with a prevalence <0,4 %, are associated with a higher relative risk of thrombosis .Protein S is a plasma serine protease involved in coagulation, inflammation and apoptosis and its deficiency results in thrombophilia. This condition is usually congenital, caused by more than 200 different mutations in the PROS1 gene , leadingIn the presented case, the patient's medical history and the diagnostic tests allowed to excludemalignancies or other acquired risk factors, except for the presence of a microcytic anemia probably due to HP-induced chronic atrophic gastritis.The patient underwent a gastroscopy that confirmed a chronic gastritis with glandular atrophy, but that also revealed angiodysplasia of all the gastric mucosa and fundic gastric polyps.Besides the known anemia, the initial blood tests showed also reduced protein levels, in particular albumin, probably due to the protein-losing gastropathy.With regard to systemic risk factors, only the free protein S deficiency was found; this condition can be both congenital or acquired. In this particular case, it is unlikely that this deficiency was determined only by a congenital mechanism, considered the patient’s age and the absence of previous major thrombotic episodes. The deficit may be rather the consequence of a combination of two conditions, namely a mild congenital protein S deficiency worsened by the protein-losing gastropathy, as also described in Greenblatt and Nguyen’s case report .With regard to PMVS thrombosis, the utility of thrombophilia testing is still a matter of debate . ScreeniAnticoagulant therapy should be continued for at least six months in patients without thrombogenic risk factors and be lifelong in those with persistent risk factors .Quality improvement in imaging, a better understanding of the disease and early detection of acute SPMV thrombosis have reduced the need for surgery and, consequently, its mortality rate .Indications for the surgical management of SPMV thrombosis are clinical findings of bowel infarction, perforation and peritonitis.Intestinal infarction leads to bowel wall and mesenteric edema, as well as ascites production; reperfusion of the bowel can worsen parietal edema and ascites, increasing the risk for abdominal compartment syndrome.For this reason, OA use should be considered after bowel reperfusion in AMI , in partIn the case reported here, a bowel resection was needed because of massive intestinal infarction.Moreover, due to the certain in diagnosis at CT scan, a laparoscopic exploration was avoided in order to promptly perform resection and offer the patient a damage control resuscitation strategy in the ICU. The surgical strategy involved a second-look operation 48 h after the open abdomen management, as already described by Occhionorelli  and Burc4PMVS thrombosis is a rare condition that can lead to severe consequences like bowel infarction. It is usually determined by the combination of inherited and acquired risks factors, that should be carefully investigated to understand its pathogenesis and establish the adequate therapy.The presence of intestinal infarction represents a mandatory indication for surgery. A planned surgical second-look, 24−48 hours after the open-abdomen management can be useful in assessing viability of the remnant bowel and feasibility of the anastomosis.Patients should undergo lifelong anticoagulant therapy whenever in presence of persistent risk factors.NoneNone.Retrospective case reports do not require ethical approval.Written informed consent was obtained from the patient for publication of this case report and accompanying images.Not essential identifying details were omitted.Annamaria Di Bella, MD: study concept and design, data collection, data analysis and interpretation, writing the paper, approval of the final version of the manuscript.Alessandro Bruscino, MD: first operator in the surgical treatment described, study concept, data collection, approval of the final version of the manuscript.Giovanni Alemanno, MD: study design, data analysis, approval of the final version of the manuscript.Carlo Bergamini, MD: study concept, data analysis and interpretation, approval of the final version of the manuscript.Paolo Prosperi, MD: data interpretation, approval of the final version of the manuscript.Retrospective not “first-in-men” case reports do not require registration.NAAlessandro Bruscino, MD.Paolo Prosperi, MD.Not commissioned, externally peer-reviewed