Papillary fibroelastoma (PFE) is a benign cardiac tumor usually incidentally found on cardiac imaging. They are typically located on the left-sided heart valves and are concerning primarily due to their risk for embolization into the periphery. Right-sided PFE, however, is very rare and their management is not well known. We present a 66-year-old male with a past medical history of HIV on antiretroviral therapy presenting with new exertional dyspnea. Upon workup, he was found to have a mass on the tricuspid valve seen on echocardiography which was ultimately resected and found to be a tricuspid fibroelastoma. The clinical management of right-sided PFE is poorly documented. Treatment of PFE in an asymptomatic is dependent on characteristics such as location, mobility, and risk of embolization. Echocardiography has made the incidental diagnosis of PFE a common issue in asymptomatic patients such as ours. Primary cardiac tumors are extremely rare, and over 75% of primary cardiac tumors are benign. Papillary fibroelastoma (PFE) is a rare, primary cardiac tumor that is more commonly associated with left heart cardiac valves and is most commonly incidentally found on echocardiography. These tumors typically have sizes that can range between 2 and 70mm, and over 80% of PFEs are typically found on left-sided heart valves ,2. UnlikA 66-year-old male with a past medical history significant for HIV diagnosed in 1995 managed on antiviral therapy, Kaposi’s Sarcoma of the right foot, diabetes mellitus, hypertension, hyperlipidemia, and hypothyroidism presented to the cardiology clinic for new-onset dyspnea over the last few months. He was primarily asymptomatic prior, but now he was only able to walk 40 blocks over 1 hour prior to getting fatigued. Otherwise, he denies having chest pain, palpitations, dizziness, orthopnea, or leg swelling. He also denied toxic habits and prior cardiovascular history. His vital signs and physical exam were unremarkable.Initial EKG showed normal sinus rhythm with no ST-T-wave abnormalities and Qtc 417 ms. An exercise transthoracic echocardiogram (TTE) was obtained due to exertional dyspnea and strong premature coronary artery disease history in his family. Resting imaging showed a left ventricular ejection fraction of 60%, normal left and right ventricular chamber size and function, and mildly dilated left atrium. The incidental finding of the stress TTE was the presence of a round, mobile echo-dense mass attached to the septal leaflet of the tricuspid valve (TV) on the atrial side measuring 1.7cm x 1.3cm with mild tricuspid regurgitation (TR) and a patent foramen ovale (PFO) concerning for vegetation or possible tumor , normal white blood cell count, and no fever. Multiple sets of blood cultures were obtained at the same time from different locations were found to be all negative thus making infective endocarditis less likely. After negative infectious workup, a cardiac MRI (cMRI) to further characterize the right-sided mass showed a small mass on the atrial side of the septal leaflet of the TV measuring 0.9cm x 0.5cm in the short-axis orientation. The mass was found to be hyperintense on T2-weighted imaging, mildly hyperintense on T1-weighted imaging, and enhanced on post-contrast findings which are concerning for the presence of a tumor rather than vegetation or thrombus Figures .The decision to resect the tumor was made after presurgical evaluation due to the high risk for embolization given the size of the mass and the presence of a PFO. Left heart catheterization was unremarkable and the carotid Doppler study was significant for mild to moderate bilateral carotid disease. He underwent successful complete excision of mass without causing damage to the TV as well as successful closure of PFO. The post-surgical course was uncomplicated, and he was discharged three days postoperatively. Finally, Surgical pathology confirmed the presence of a fibroelastoma. On follow-up, the patient reported continued improvement in symptoms and was feeling better overall.PFE is a rare benign cardiac tumor that predominantly affects the cardiac valves particularly on the left side of the heart. Although its true prevalence is unknown due to its asymptomatic presentation and incidental discoveries, recent studies have reported a prevalence of about 10%, with tricuspid PFE being reported to be 6%-15% . Mean agCardiac masses could have a number of different etiologies ranging from infectious processes, thrombi, and primary or secondary malignancies. Therefore, it is important to have multiple imaging modalities that can help supplement clinical data in determining the possible causes of the mass. The portability, wide availability, lack of radiation, and relatively low cost make echocardiography easily accessible. Echocardiography of our patient was able to show a round, mobile, echo-dense mass leading to further investigation in our patient. PFEs generally range from 2mm to 40mm and are usually found to be pedunculated . They caCMRI can supplement TTE findings to further differentiate the makeup of the mass. Neoplastic cells have been found to be larger than normal cells thus containing more intracellular water, which leads to prolonged T1/T2 relaxation times thus allowing cMRI to pick up the inherent differences between tumors and normal tissue . The golUltimately, a definitive diagnosis is made on histopathology after surgical resection or biopsy. Histopathology of PFEs typically shows a central core of fibrin, mucopolysaccharide-rich connective tissue surrounded by a layer of the endothelium ,8. AlthoThe prognosis of PFE is good following the excision of the tumor especially because of its benign nature and rare recurrence after resection . RemovalRight-sided PFEs are rare and often found incidentally on cardiac imaging, especially echocardiography. Although surgical resection has been clearly shown to improve morbidity and mortality in left-sided tumors, management of right-sided tumors continues to be unclear. Further research is necessary to create better management guidelines for the management of right-sided tumors. This case presents an interesting report of right-sided PFE with surgical resection and pathological confirmation.


Langerhans cell histiocytosis is a rare hematological disorder. Skin rash is the typical early feature, and bony involvement is the second most common presentation.We present a case of a 5-month-old female infant with left hemifacial swelling, initially treated for infection with antibiotics. However, due to persistence of swelling and new onset fever, further evaluation with ultrasonography, CT scan, FDG PET/CT and eventually biopsy was performed.Imaging methods revealed mandibular osteolysis indicative of either osteomyelitis or histiocytosis X. Tissue biopsy was diagnostic for Langerhans cell histiocytosis.Langerhans cell histiocytosis may present in infancy with a variety of symptoms, included an isolated bony lesion. Langerhans cell histiocytosis, despite its rarity, should be included in the differentiated diagnosis, when bone osteolysis is found. Langerhans cell histiocytosis (LCH) is a systemic disease of young age, usually presented with s skin rash but it may involve several organs, such as gastrointestinal tract, liver, spleen, lungs. Tissue biopsy reveals propagation of abnormal Langerhans cells. Prognosis depends on the organs involved .A 5-month-old female infant with left hemifacial swelling for 4 weeks was brought to the outpatient pediatric hospital clinic for evaluation. Initially, even though no specific etiology was evident, the infant was treated with antibiotics for a possible unspecified infection, resulting in a slight decrease of tumefaction. Fifteen days later an urgent consultation was obtained by the Ear Nose and Throat department for persistence of the tumefaction and appearance of fever. Physical examination showed no change of color locally or spontaneous pain, but there was an indurated part in the pre-auricular region and the affected area was sensitive to palpation. Further evaluation with ultrasonography was performed that revealed an hypoechogenic solid mass, slightly vascularized, measured 33x13x17 mm emerging from the left inferior mandible. In addition, there were few hyperechogenic images adjacent to the bone contour which appeared destroyed.An evaluation with computed tomography (CT) scan was followed, revealing a large osteolytic lesion of the left mandible without categorical invasion of the neighboring soft tissue parts. In absence of an inflammatory clinical entity or of a typical osteomyelitis imaging, histiocytosis X was considered as a probable diagnosis . FluorodA transoral biopsy of a recently appeared granuloma-like lesion along the gingival sulcus was performed under general anesthesia. A biopsy of the normal bone-lesional tissue interface revealed Langerhans cell histiocytosis. The biopsy showed numerous Langerhans cells with oval, grooved, folded nuclei and moderately abundant cytoplasm admixed with eosinophils, neutrophils, small lymphocytes and sparse plasma cells .Since the disease involved only one site in the mandibular area, a combination therapy consisted of prednisone and vinblastine was administered weekly. At present a follow up after 8 weeks confirmed a clear decrease of the volume of left hemimandible mass.Langerhans cell histiocytosis represents a rare clinical entity with a characteristic pathology. In the neonatal population may range from an isolated bony lesion to a serious systemic disease. Its variety of clinical manifestations could make the diagnosis challenging requiring rigorous investigations for proper management .The mandibles may be affected in about 20%–30% of all cases making the diagnosis challenging ,5.Management of Langerhans cell histiocytosis varies depending on the location and the extend of the disease and may include surgery, radiotherapy and corticosteroids with or without chemotherapy. Chemotherapeutic agents include methotrexate, 6-mercaptopurine, hydroxyurea, vinblastine, cytarabine cladribine and others. In our case, since the lesion involved one site and the patient was an infant, a combination of prednisone with vinblastine was selected which had a favorable response. In many cases with single system involvement there is a cure, however, in cases with multisystem involvement recurrences are frequent and the prognosis may be guarded.A thorough investigation in an infant with a cheek swelling led to the rare diagnosis of Langerhans cell histiocytosis. Thorough investigation, included a biopsy, should be implemented in suspicious cases since the disease is potentially curable.


Hemangiomas of the tongue may reach a massive size resulting in functional compromise. Surgical resection, despite the risk of hemorrhage, may be the only option if conservative measures such embolization are futile in reducing its size. Hemangiomas of the tongue may reach a massive size resulting in functional compromise. Surgical resection, despite the risk of hemorrhage, may be the only option if conservative measures such embolization are futile in reducing its size. This paper reports the surgical management of a non‐involuting massive congenital hemangioma of the tongue in an infant presenting with difficulty breathing, feeding and inability to close the mouth.Hemangiomas are common congenital head and neck anomalies characterized by rapidly proliferating endothelial cells that line blood‐filled spaces, having an incidence of 3%–10% at 1 year of age but disappear completely by 5 years of age in about 50%–80% of cases.Hemangiomas of the tongue usually present as small lesions less than 3–5 cm in size. However, giant lingual hemangiomas involving up to a half or two‐thirds of the tongue have been reported previously, posing a significant risk to patient which may considerably deteriorate their quality of life.Hemangiomas usually do not require any treatment due to their benign nature and high rate of complete involution over time. However, in cases where the lesion fails to involute despite conservative measures or is extremely large and poses an imminent threat to life, surgical intervention may be the only treatment of choice despite the inherent risk of potentially fatal intraoperative hemorrhage.2We report a case of a 6‐month old girl referred to the Nairobi hospital with a massive, progressively growing congenital mass in the oral cavity. A thorough medical history revealed no co‐morbidities with all vital signs within normal limits. Upon physical examination, the patient was found to have difficulty in breathing, difficulty feeding and inability to close the mouth. Intra oral examination revealed a large swelling involving more than two‐thirds of the tongue, most of which lay outside the oral cavity Figure . The lesMagnetic Resonance Imaging (MRI) with gadolinium revealed a well‐circumscribed soft tissue mass measuring 3.9 cm × 4.1 cm × 5.8 cm. It involved most of the tongue except for the base and extended to both lateral borders, while inferiorly, involved the entire thickness of the tongue to its ventral surface. The mass was hypointense on T1‐weighting and was found to be hyperintense on T2‐ weighting. Multiple low signal foci were noted which were compatible with flow voids (Figure The lesion was monitored closely over a period of 1 year with the expectation of improvement by involution, however, the lesion showed no signs of reducing in size and in fact enlarged over this duration Figure . After aOne month post embolization, a color change from deep red/purple to a lighter shade was noted and the lesion seemed to have reduced slightly in size as shown by its surface having mucosal folds as opposed to appearing stretched and shiny prior to embolization Figure . Despiteth day after surgery. Histology of the tissue specimen revealed the diagnosis to be a capillary hemangioma (Figure Postoperative hospital stay was uneventful with no medical or surgical complications. The patient was fed via a nasogastric tube for a period of 5 days after which it was removed and oral feeding begun. The patient was discharged on the 8a Figure . At the a Figure .3Hemangiomas deserve special attention and consideration, not because of their rarity of occurrence but due to the potential of life‐threating complications such as airway obstruction and hemorrhage. Similar to hemangiomas in other anatomical sites, hemangiomas of the tongue occur at birth or shortly after and grow rapidly up to 6 months of age after which they spontaneously involute.The etiopathogenesis of congenital hemangiomas remains unknown. However, theories such as the placenta theory, metastatic theory, progenitor cell theory and the extrinsic factor theory attempt to describe the development of infantile hemangiomas.Management of hemangiomas depend upon factors such as age, size, location, involved vasculature and response to any previous treatment strategies. Numerous conservative approaches may be implemented prior to opting for surgical intervention including corticosteroid therapy, beta blocker therapy, sclerotherapy, cryotherapy and laser therapy.Large vascular malformations usually respond well to selective embolization, reducing the lesion's size significantly to avert life‐threatening complications.A modified key hole resection technique was specifically devised to achieve adequate reduction in both transverse and antero‐posterior dimensions of the tongue that would allow it to fit in the oral cavity but yet ensure that functionality remained intact.4Hemangiomas are a benign proliferation of endothelial cells that are common in the head and neck region. In the oral cavity, the lesion may involve the tongue and reach a size that can cause airway embarrassment and cause significant functional impairment. Surgical intervention, despite the inherent risk of fatal intraoperative hemorrhage, may be the only treatment of choice if other conservative measures do not produce the intended results or the rapid expansile nature of the lesion causes an imminent threat to life.No conflict of interest to declare.Krishan Sarna: Collected the data and wrote the findings of this manuscript. Dr. Martin Kamau: Provided supervision and guidance during preparation of the manuscript and approved the final version of the manuscript. Professor Symon Guthua: Performed the surgery in the case described, supervised the development of the manuscript and approved the final version.The manuscript was prepared according to standard publication ethical guidelines.The authors confirm that a signed consent was obtained from the patient prior to publication.


A 61-year-old male presented with chronic shoulder pain resistant to conservative treatment. Imaging identified a nodular lesion in the deltoid muscle, which histology after resection identified as a glomus tumor. After surgery, the patient became asymptomatic and at 4-year follow-up has not shown signs of recurrence.  Glomus tumors around the shoulder should be considered when investigating chronic shoulder pain, as they are more common than thought. Despite being elusive, when diagnosed, excellent outcomes may be expected, with surgery resection being curative. Glomus tumors are benign soft tissue neoplasms of vascular origin . These tIn this paperwork, we present a rare case of chronic shoulder pain caused by a glomus tumor in the deltoid muscle. As these lesions appear to be more prevalent than assumed, we also present a compilation of all the published cases and a short literature review.The patient was informed that data concerning the case would be submitted for publication, to which he consented.A 61-year-old right-handed male was referred to our department presenting shoulder pain for 12 months, without any known trigger event. The patient described a dull intermittent right shoulder pain, reporting acute exacerbations and tenderness. His complaints persisted despite medical treatment, including physiotherapy and long-standing medication with oral NSAIDs.During physical examination, there were no visible asymmetries between the shoulders and the patient exhibited no inflammatory signs. The neurologic and vascular examination of the right upper limb was normal. The shoulder range of motion and strength was preserved and painless. There were no evident or palpable masses, but the patient presented trigger-point tenderness at the posterolateral surface of the right shoulder.Plain radiographs were normal . UltrasoIt was decided to perform an excisional biopsy of the lesion. Under general anesthesia, the patient was placed in 30 degrees left-lateral position and a 5 cm incision was made directly over the point of tenderness which had been priory marked. Surgical exploration identified a well-limited nodular lesion between the posterior fibers of the deltoid muscle, which was completely excised Figures .The patient was discharged in the next day after surgery and presented pain-free by the time of stitch removal.Gross examination of the specimen revealed a brownish nodular mass with 10 × 10 × 6 mm in size, while microscopic examination showed a well-limited lesion formed by ovoid epithelial cells without signs of atypia. No mitoses were observed. The lesion displayed a rich vascular network. Marginal excision was confirmed. Immunohistochemical staining was positive for expression of actin . These findings were compatible with the diagnosis of glomus tumor Figures .The patient did not undergo any other treatment besides surgical excision of the lesion.After 4 years of follow-up, the patient is asymptomatic and showing no clinical or imagiological evidence of local or systemic recurrence.Glomus tumors are vascular benign neoplasms , 5, arisDespite being widely scattered, glomus bodies are far more frequent in the digits, palms, and soles of feet , with 70In general, glomus tumors represent 1-6% of all soft tissue tumors  and 1-5%Extradigital locations seem less rare than what was previously believed , 4. OtheGlomus tumors are generally small lesions (<1 cm in diameter)  but partth case of a glomus tumor located in the deltoid muscle [To our knowledge, this is the 4d muscle , 15, 16.Extradigital locations impose true diagnostic challenges  and may Glomus tumors are classic manifested by the triad of paroxysmal pain, cold hypersensitivity, and pinpoint tenderness, which when present can be considered diagnostic . 63-100%Moreover, there have been described several clinical tests which may help in the diagnosis of glomus tumors, such as the pin test of Love, the Posner test (pain induced by cold), the Hildreth ischemia test, and transillumination , 22. NonMRI can be helpful in the diagnosis of glomus tumors, which mostly exhibit low signal intensity on T1-weighted images, hyperintensity on T2-weighted images, and enhancement on T1-weighted images after gadolinium injection . In addiMRI has been described to have a sensitivity of 90% for the diagnosis of glomus tumours , being aα-SMA  and a variable expression of CD34 [The final diagnosis is confirmed by microscopic and immunohistochemistry examination of the lesion . Histolo of CD34 .Surgery is usually required, as conservative treatment is often unsatisfactory , 16, 23.Malignization is rare . Of thosSurgery outcome is excellent , with prThe atypical location, the usual small-size of the lesions, and deeper locations explain why these lesions are frequently overlooked on common imaging exams such as simple ultrasonography, accounting for the delay in diagnosis. In our case, despite being small in size, the lesion was fortunately identified on ultrasonography which prompted further investigation. The fact that full recovery is expected after adequate treatment further emphasis the needs to raise awareness for this pathology when considering causes of chronic shoulder pain.Glomus tumors around the shoulder seem less rare than what was previously believed and should therefore be included in the differential diagnosis of unexplained shoulder pain. MRI may help confirm diagnosis in a high suspicious clinical setting. Surgery with marginal excision is usually curative, and excellent clinical outcome is expected.


Hairy tongue is a furry appearance on the dorsum surface of the tongue with variable colors. This lesion is due to defective shedding of filiform papillae. Various treatments are proposed for this condition like keratolytic agents, surgical procedure with a scalpel, or laser application. In this case study, we reported successful treatment of hairy tongue with a diode laser. The diode laser with wavelength 810 nm, power of 4 watts, a pulse width of 20 milliseconds, and an interval of 20 milliseconds by a 400-micron optical fiber was selected for treatment of this lesion. There was no bleeding during this minimally invasive surgery, and the patient experienced a low level of pain. Loss of taste function was completely resolved. Based on the result of this study, a diode laser can be a good alternative for minimally invasive surgical treatment of hairy tongue. Hairy tongue (HT), known as furred tongue, is referred to as an acquired benign tongue pathology. From the clinical view, HT is diagnosed through “hair-like” projections, developed when elongated, and hypertrophied filiform papillae are present on anterior 2/3 of dorsum aspect of the tongue. This pathology is the consequence of decreasing in cell desquamation and accumulation of keratinized layers .HT may occur in various colors ranging from unpigmented, whitish, yellowish, bluish, tan, brown to black. Accordingly, what determines the color is an extrinsic factor that adheres to the lingual surface .It may be prevalent within a wide range of 8.3 to 57% percent. Although there is no age limit for HT, its incidence and prevalence are positively correlated with age. It is reported to be more prevalent among men, yet females are also prone to this pathology .HT is associated with the following:1—poor oral hygiene; 2—smoking; 3—drugs ; 4—low fiber diets; 5—Candida albicans; 6—anemia; 7—alcohol; 8—dentures; 9—therapeutic radiation of head and neck. Scarcely has HT been found in human immunodeficiency virus (HIV) infection, graft versus host disease (GVHD), and internal malignancies [It is asymptomatic and self-resolving in general, but patients may occasionally complain about halitosis, abnormal taste, gagging and tickling sensation, nausea, dysgeusia, and burning mouth sensation . As the 2, Erbium family, Nd: YAG, and diode lasers [Literature has also outlined the second-line therapy in different measures like topical retinoid, keratolytic agents (podophyllin), antimicrobial therapies, topical triamcinolone acetonide, gentian violet, salicylic acid, vitamin B complex, thymol, topical urea solution, and keratolytic agents like trichloroacetic acid. What merits attention is that the above-mentioned therapeutic measures are endowed with potential side effects from local irritation and possible systemic absorption. Surgical treatment of several oral lesions  can be addressed by laser application with different modalities like COe lasers , 8. Lesse lasers . Meserene lasers  first inA 22-year-old nonsmoker woman presented with whitish-yellowish discoloration on the dorsum of the tongue and was referred to a private dental office . The patMedical history was unremarkable. She did not take any medication. She mentioned that she had high-stress lifestyle due to her job. Intraoral examination showed good oral hygiene according to Silness and Löe  plaque iAfter obtaining informed consent, a surgery was performed under local anesthesia . A diode laser  was used by an expert surgeon. The laser enjoyed a wavelength of 810 nm, a power of 4 watts, a pulse width 20 milliseconds, and an interval of 20 milliseconds by a 400-micron optical fiber. The half of the lesion was removed by the laser in a slowly sweeping motion 4 mm above the surface and with a 30-degree angle relative to the tongue (to decrease penetration depth and reduce tissue damage) for 3-4 minutes, accompanied by high vacuum suction removal . SpecialIn the follow-up session after 1 and 2 weeks, partial healing was observed Figures . The othHT is a relatively common abnormal coating on the dorsal surface of the tongue. This condition stems from elongation and lack of desquamation of filiform papillae, so they grow longer rather than falling off .2 and Erbium family lasers are good options [Various techniques have been employed to treat this lesion. We started with the second-line treatment  as the patient's oral hygiene was good, and she did not have any risk factors (the requirements of the first-line treatment). Applying a knife to carry out the surgical excision of the papillae may be followed by some bleeding, where suturing is difficult and needs special skill. Also, it causes the removal of massive amount of tissue (with no control), which in turn increases the healing period and levels of pain . Laser t options , but the options . The dio options . Regardi options .Thanks to the hemostasis and coagulation effect of the laser, the operator had an outstanding field visibility during the procedure and there was no need for suturing. Patients undergoing a diode laser surgery in the oral cavity seem to have less pain. In addition, it was proven that the diode laser can eliminate bacteria and disinfect the wound –20; in tIn this study, the patient was satisfied with the result of the surgery and her ageusia was completely resolved. Consequently, her quality of life was significantly improved. To evaluate the lesion recurrence, she was examined 3 months later and there was no sign of recurrence. For further evaluation, long-term follow-ups were recommended.Within the context of this study, application of the diode laser in the treatment of hairy tongue can be beneficial for both patients and clinicians. This modality provides minimal postoperative complications and a simple surgical procedure with the least possible side effects.


MEN1 variant c.781C>T (LRG_509t1), which was previously reported only once in a family with isolated hyperparathyroidism. A heterozygous missense variant in exon 4 of the gene was identified in the sequence of the MEN1 gene, i.e., c.781C>T, leading to the amino acid change p.Leu261Phe in a three-generation family. In the screened family, 5/6 affected members had already developed hyperparathyroidism. In the index patient and two other family members, an aggressive course of pancreatic neuroendocrine tumor (insulinoma and non-functioning neuroendocrine tumors) with dissemination was diagnosed. In the index patient, late diagnosis and slow progression of the disseminated neuroendocrine tumor have been observed (24 years of follow-up). The very rare variant of MEN1, LRG_509t1 c.781C>T /p.Leu261Phe (LRG_509p1), diagnosed within a three-generation family has a heterogenic clinical presentation. Further follow-up of the family members should be carried out to confirm the spectrum and exact time of clinical presentation.Multiple neuroendocrine neoplasia type 1 (MEN1) is a rare genetic disorder with an autosomal dominant inheritance, predisposing carriers to benign and malignant tumors. The phenotype of MEN1 syndrome varies between patients in terms of tumor localization, age of onset, and clinical aggressiveness, even between affected members within the same family. We describe a heterogenic phenotype of the  MEN1 alterations have been noted [MEN1 variant, or (iii) cascade genetic screening in an asymptomatic carrier [Multiple neuroendocrine neoplasia type 1 (MEN1) is a rare, heterogeneous genetic disorder with an autosomal dominant inheritance, predisposing the carrier to benign and malignant tumors. The prevalence of MEN1 is estimated to be approximately 1:30,000 in individuals, with no sex predominance ,5,6,7,8.en noted ,11. The  carrier . Genetic carrier ,14. MEN1 gene, located on chromosome 11q13, encodes the menin protein [CKI) genes CDKN1b (encoding p27) and CDKN2C (encoding p18). To date, over 1500 variants of MEN1 have been described, including frameshift variants, nonsense variants, missense variants, splice site variants, and large deletions. Additionally, patients with these variants should be carefully monitored due to their uncertain clinical significance and heterogeneous presentation. [MEN1 variant c.781C>T, previously reported only once in a family with isolated hyperparathyroidism [The  protein ,16. Menintation. ,5. In thyroidism .® Blood kit . All coding exons of MEN1 (LRG_509t1) were amplified using tiTaq DNA polymerase  and 100 ng DNA in a 25-µL reaction in an Eppendorf MasterCycler thermocycler. Following electrophoresis (Biometra Horizon 20.25 with a Consort EV243 power pack) on a 1% agarose gel stained with ethidium bromide, the obtained PCR products were visualized by a gel documentation system with a UV transilluminator (UVP GelDoc IT310). After purification of the PCR products through the use of a commercially available kit , the fragments were sequenced using 25 ng of the purified PCR product and the BigDye® Terminator v3.1 Cycle Sequencing Kit , purified by ethanol precipitation. These fragments were then read on an ABI3500 sequencer . The obtained sequences were aligned to the appropriate reference using SeqScape software . Further analyses were performed using the UCSC Genome Browser  and associated links on the human GRCh38/hg38 genome assembly. Multiplex ligation-dependent probe amplification (MLPA) analysis (MRC Holland probemix P017) was performed according to the manufacturer’s recommendations in order to rule out large deleterious events in the gene. PROVEAN (v1.1.3) and SIFT (v4.0.3) tools (provean.jcvi.org) and the Bayes classifier-based MutationTaster prediction tool (www.mutationtaster.org)  were used for prediction of the identified variant’s impact on the protein’s biological function. Extraction of DNA from whole blood of the patients, which was collected on EDTA, was performed using the NucleoSpinMEN1 variant as confirmed by genetic testing. The index patient, II.3 of the pedigree  scan of the abdomen revealed a pancreatic tumor. The patient underwent surgical pancreatoduodenectomy (Traverso-Longmire procedure). An insulin-secreting neuroendocrine tumor was confirmed by histopathology. Additionally, in 1996, the patient underwent cholecystectomy due to cholecystolithiasis. During that time, hypercalcemia was not found in the patient’s medical records; however, ultrasound examination of the kidneys suggested nephrolithiasis. After surgery, the patient developed diabetes, which was poorly controlled with intensive insulin therapy. In 1999, the patient underwent a total hysterectomy with bilateral salpingo-oophorectomy due to a myoma of the uterus. 99mTc result was negative. Repeat surgery was performed, with resection of masses in the mesentery as well as those located near the splenic vessels. Histopathological analysis revealed metastases of the neuroendocrine tumor infiltrating adipose tissue, Ki67 < 1%; 90% of the cells had positive staining for somatostatin receptor (SSTR) type 2, type 4, and type 5, and 50% of the tumor cells showed positive staining for SSTR type 3. Post-operative SRI detected no pathological accumulation of the tracer. The diagnosis of primary hyperparathyroidism (PHPT) was confirmed in 2007. At this point, the patient had already suffered from nephrolithiasis for several years. Ultrasound examination of the neck revealed a nodular goiter and a single hypoechogenic mass in the topography of the left inferior parathyroid gland. The patient underwent a partial strumectomy and resection of the enlarged parathyroid. Parathyroid adenoma was identified via histopathology. The patient was diagnosed with MEN1 syndrome based on the clinical diagnostic criteria. Pituitary magnetic resonance imaging showed a normal pituitary gland (size 12 × 6 × 14 mm) with no sign of focal lesions. In 2008, during the radiological follow-up, abdominal CT scan revealed focal lesions—possible metastases—located ventrally to the connection of the splenic and the inferior mesenteric vein, in the remaining part of the duodenum, and in the mesenteric lymph nodes. The somatostatin receptor imaging (SRI) with 99mTc) Tc-MIBI. In 2011, repeat SPECT/CT showed a pathological accumulation of 99mTc-MIBI in the topography of the left lower parathyroid. In 2011, the patient underwent a total parathyroidectomy, resulting in subsequent iatrogenic long-lasting hypoparathyroidism. During a follow-up visit in 2008, biochemical relapse of primary hyperparathyroidism was detected (parathormone (PTH) 163.8 pg/mL, total calcium concentration 2.81 mmol/l) without any visible lesions in ultrasonography or single-photon emission computed tomography/computed tomography (SPECT/CT) with technetium-99 m methoxyisobutylisonitrile (68Ga) Ga-DOTATATE positron emission tomography/computed tomography (PET/CT) revealed the pathological expression of somatostatin receptors in an epigastric lymph node and in the second segment of the liver. In 2018, abdominal CT detected hypodense focal lesions in the liver and enlarged mesenteric lymph nodes  Ga-DOTATATE PET/CT was performed. In the liver, no pathological accumulation of the tracer was found; however, lymph nodes along the lesser curvature of the stomach showed pathological expression of SSTR. At this point, the patient did not consent to any further invasive diagnostic procedures. Physical examination revealed the presence of cutaneous collagenomas and hyperpigmented skin lesions (Fluorine-18 fluorodeoxyglucose PET/CT (FDG-PET) revealed a metabolically active region in the liver and in the mesogastric region, either in the small intestine or in a lymph node. Subsequently, ( lesions d. The patient’s mother is 84 years old (patient I.2). Calcium and PTH concentrations in serum are within the normal range. She suffers from chronic gastritis but is in good general condition. The patient’s mother did not consent to genetic testing. The patient’s father suffered from prostatic hyperplasia and died at the age of 84 due to a pulmonary infection. Genetic testing was not performed. The index patient has two siblings: a brother with MEN1 syndrome (patient II.4 in the pedigree) and a sister (patient II.1) who is in good general condition. Her total calcium and PTH concentrations in serum are within the normal range. Genetic testing has not yet been performed.68Ga) Ga-DOTATATE PET/CT, the lesion showed intense accumulation of the tracer (SUVmax 52). Additionally, pathological expression of the somatostatin receptors (SUVmax 3.32) in the marrow cavity of the proximal 1/3 of the femur was discovered. Subsequent CT confirmed the presence of a focal lesion in this location (32 mm), which was suspected to be metastatic. Additionally, suspicious lesions with pathological expression of the somatostatin receptors were discovered in the femur and tibia. The patient did not consent to any further diagnostic procedures.The index patient’s brother was born in 1955 and is the father of five children, two of whom are unaffected. He was first admitted to the Endocrinology Department in 2015 and MEN1 syndrome was confirmed via familial genetic screening. During the patient’s first endocrinological evaluation, hypercalcemia (2.7 mmol/L) and elevated PTH (230 pg/mL) were detected. A nodular goiter and an enlarged right upper parathyroid gland were seen in the thyroid ultrasound examination. The right lobe and the isthmus as well as the parathyroid gland were resected in 2016. Histopathological examination revealed parathyroid adenoma; however, malignancy of the thyroid gland was not detected. Since the surgery, calcium concentration and PTH levels have been within the normal range and the patient has no signs or symptoms of nephrolithiasis or osteoporosis. Multiple MRI examinations performed during follow-up visits (most recently in 2017) did not detect any focal lesions of the pituitary gland or excessive hormone secretion. An abdominal CT from 2016 revealed a 25-mm tumor of the uncinate process of the pancreas. Subsequent SRI confirmed an abnormal accumulation of the tracer in this location. In 2017, the patient did not consent to endoscopic ultrasound nor to surgical resection of the pancreatic lesion. The patient did not attend any follow-up visits until October 2019, when another abdominal CT was performed. Imaging revealed that the pancreatic lesion remained stable; however, in , with no excessive hormonal function. Additionally, CT confirmed the presence of a pancreatic cyst, which was left in place for radiological follow-up. In 2015, MRI revealed a pituitary microadenoma, clinically and biochemically silent. During a follow-up visit in 2016, abdominal CT revealed a round 8-mm tumor with calcifications in the corpus of the pancreas a. 68Ga) Ga-DOTATATE PET/CT was performed. It revealed multiple focal accumulations of the tracer in the pancreas, with the most prominent one observed within the pancreatic corpus (SUVmax 22.8), located in the topography of a previously described calcified tumor, and a probable metastasis in the left lung (SUVmax 6.54). In January 2018, due to dissemination of the neuroendocrine tumor, treatment with Lanreotide autogel at 120 mg s.c./4 weeks was initiated with good patient tolerance. In December 2018, an endoscopic ultrasound was performed. Histopathological examination of the samples revealed the presence of a probable neuroendocrine tumor . In January 2020, follow-up CT revealed a partial regression of the pancreatic lesions, while the pancreatic neuroendocrine tumors and metastatic lung lesion were stable with no further progression . Subsequently, endoscopic ultrasound-guided fine-needle aspiration biopsy of the pancreas was performed. Histopathological examination revealed the presence of a neuroendocrine tumor . Radiological follow-up has been scheduled. In addition, the patient has multiple cutaneous collagenomas. MEN1 gene (LRG_509), i.e., c.781C>T, leading to the amino acid change p.Leu261Phe at the protein level. This variant has been registered in the NCBI dbSNP with the identifier rs878855198. Based on criteria provided by a single submission to NCBI ClinVar, this variant (ID 241826) is classified as having uncertain clinical significance. In the LOVD database (ID MEN1_000346), this alteration has no assigned clinical classification. In the UMD-MEN1 database, there are three entries for this variant , two of which relate to samples derived from members of one family. In all three cases, the variation has been classified as “causal”. The variant is located in a phylogenetically conserved region, and leucine at position 261 of the protein is, itself, highly conserved . In only one affected family member, pituitary microadenoma was detected at the time of diagnosis (at age 34) along with other manifestations of the syndrome . To date, the MEN1 LRG_509 c.781C>T (p.Leu261Phe) variant has been described in one family presenting with a mild phenotype, with isolated hyperparathyroidism reported as a gene variant with uncertain significance [MEN1, genotype–phenotype correlation has not been estimated due to the heterogeneity of the disease. Previous studies of unrelated patients with the same variant demonstrated the variable clinical expression of MEN1 characteristic tumors. Even case reports of monozygotic twins have reported different phenotypes of MEN1 syndrome [Our study describes the diverse outcomes (aggressive and benign) of a very rare syndrome ,19. It isyndrome . Screenisyndrome ,21. MEN1 variants are observed in 1–18% of patients [The earliest and most common MEN1-related feature is primary hyperparathyroidism due to parathyroid hyperplasia or parathyroid adenoma. Up to the age of 50 years, hyperparathyroidism shows almost 100% penetrance. Conversely, among all patients diagnosed with PHPT, patients . Hyperpapatients . In addipatients . Four afpatients . The mospatients . Insulinpatients ,27,28. Tpatients . In the Pituitary tumors associated with MEN1 occur mostly in the fourth decade of life ,21. ProlA limitation of our study is the fact that family members of the index patient were not followed-up prospectively but were diagnosed at a certain point in time, long after the index patient was diagnosed. Therefore, the exact onset of the MEN1 manifestations could not be established. The delay in the cascade genetic screening resulted from a delayed transfer of information about the genetic background of the disease between the family members. The probable psychological barriers which hinder the sharing of information about genetic diseases need further investigation and support . MEN1, LRG_509t1 c.781C>T /p.Leu261Phe (LRG_509p1), diagnosed within a three-generation family, has a heterogenic clinical presentation. This included the presence of previously unreported neuroendocrine tumors in selected family members. Further follow-up of the family members should be performed to confirm the spectrum and exact time of clinical presentation of this alteration. The very rare variant of


Diverticulitis is a common cause of an acute surgical abdomen and computed tomography has become an essential part of work up particularly to identify complications that commonly include intraperitoneal perforation, abscess and fistula formation. We report the case of an 81-year-old male who presented to the emergency department with acute lower abdominal pain and was found to have sigmoid diverticulitis with the rare complications of a diverticular abscess that had formed a sinus tract and perforated into the retroperitoneum and secondary acute appendicitis. Initial management was with intravenous antibiotics, a Hartmann's procedure and appendicectomy. Subsequently the retroperitoneal collection was drained percutaneously. The case was further complicated by the patient's multiple co-morbidities and unfortunately the patient died 6 weeks after admission from sepsis. This case highlights the role of computed tomography in the pre- and post-operative period to identify complications which are often clinically occult and require early surgical and interventional radiology management to optimize outcomes. Diverticulitis and acute appendicitis are among the most common causes of an acute surgical abdomen, with estimated incidences of approximately 110 and 190 per 100,000, respectively An 81-year-old male presented to the emergency department with 4 days of abdominal pain and anorexia followed by a fall and 14 h long lie on the floor. He denied any nausea, vomiting or change in bowel habit. The patient had multiple co-morbidities including stage IV chronic kidney disease secondary to polycystic kidney disease, ischemic heart disease, atrial fibrillation, type 2 diabetes, hypothyroidism, erosive gastritis, obesity and diverticular disease. On examination the patient was peripherally shutdown, had poor bibasal air entry and a peritonitic abdomen with left flank fullness. The patient was tachycardic (125 bpm), hypotensive (100/55 mm Hg), tachypnoeic (36 bpm) and afebrile (37°C), with initial blood results supporting a diagnosis of severe sepsis on a background of chronic kidney disease .Table 1APortal-venous phase contrast-enhanced computed tomography (CT) of the abdomen and pelvis demonstrated an approximately 20 cm long segment of circumferentially thickened sigmoid colon containing numerous diverticula and severe pericolic inflammatory stranding A. A 5 cmThe pre-operative P-POSSUM score was 95.8% mortality and 99.9% morbidity. A second consultant surgical opinion was sought given his high-risk profile and, taking into account the patient's strong wishes, a joint decision was made to proceed with a laparotomy.Intraoperative findings were an inflammatory mass attached to the left lateral sidewall containing a loop of perforated sigmoid colon and adherent appendix, cecum and distal small bowel. A sigmoid colectomy with rectal stump and end colostomy formation (Hartmann's procedure) and appendicectomy were performed. Minimal pus was found in the left lateral sidewall at time of operation and the retroperitoneum was not explored. Intra-abdominal drains were placed in the left paracolic gutter and pelvis. Histology confirmed perforated sigmoid diverticulitis with acute transmural inflammation, pericolic tissue abscess formation and acute suppurative appendicitis.The patient was extubated on day 1 and was managed in intensive care for 8 days requiring high levels of oxygen for a co-existing pneumonia. Following stepdown to the surgical ward the patient developed worsening sepsis and CT imaging, performed on post-operative day 21, demonstrated an enlarged, complex retroperitoneal collection in the left flank. In addition to intravenous antibiotics, 2 percutaneous drains were inserted into the retroperitoneal collection but unfortunately the patient deteriorated with worsening sepsis and died on post-operative day 40.We present the case of an 81-year-old man with perforated sigmoid diverticulitis complicated by acute appendicitis and retroperitoneal abscess formation. Perforation is a common complication of acute diverticulitis. The vast majority are contained perforations but in 1%–2% there is free intra-abdominal gas The retroperitoneum is not routinely entered during laparotomy and retroperitoneal collections often have non-specific symptoms. Mortality of patients with retroperitoneal abscesses was historically as high as 22%-46% Additionally, in this case there was co-existing acute suppurative appendicitis which we hypothesize occurred secondary to transmural bacterial spread from the inflamed sigmoid colon. Part of the sigmoid colon was located in the right iliac fossa and the appendix was fibrosed to the inflamed proximal sigmoid in the midline. Serosal appendicitis can occur with an extra-appendiceal source of inflammation but in this case histology demonstrated full thickness appendiceal ulceration, presumably a result of the delayed presentation facilitating transmural spread This case demonstrates the importance of cross-sectional imaging to diagnose the complications of sigmoid diverticulitis, both pre- and post-operatively, to facilitate appropriate and prompt multi-disciplinary management. Antibiotics, surgical resection, image-guided percutaneous drainage and high-quality supportive care are all important to optimize outcomes. In complicated cases early post-operative imaging is also essential to identify potential complications, particularly when there is a suspicion of retroperitoneal involvement that may not be visualized intra-operatively.


Utero-cutaneous fistula is a rare pathology. It mostly occurs consecutively to surgical intervention such as Cesarean section. Blood discharge from the cesarean scar during menstruation is a quasi-pathognomonic feature. Imaging modalities, particularly with the injection of contrast material through the cutaneous fistulous opening, confirm the diagnosis. The management is mainly surgical.We report the case of a utero-cutaneous fistula in a 27-year-old lady, with systemic lupus erythematosus. She presented seven months after her third cesarean section with pain and blood discharge from the cutaneous scar during menstruation for four months. A pelvic CT scan with the injection of the contrast material through the cutaneous fistulous opening confirmed the diagnosis of utero-cutaneous fistula. Surgical management was successful. Utero-cutaneous fistula is a rare condition that manifests with cyclic pain and blood discharge of the abdominal wall.The confirmation of the diagnosis is based on imaging with opacification of the fistulous tract.The primary treatment is surgical excision of the fistulous tract.Utero-cutaneous fistula is an extremely rare condition and its pathophysiology is not fully understood. It mostly occurs consecutively to surgical intervention such as Cesarean section. Blood discharge from the cutaneous fistulous opening during the menstrual bleeding is almost pathognomonic We report the case of a 27-year-old woman, with systemic lupus erythematosus. The diagnosis of the utero-cutaneous fistula was confirmed by imaging. The surgical management was successful and consisted of the total excision of the fistulous tract, without hysterectomy.A 27-year-old woman, with systemic lupus erythematosus since 2014, under long-term corticosteroid therapy and Hydroxychloroquine. She presented pain and cyclic blood discharge for 4 months from her third Cesarean section that was performed 7 months ago.Examination of the abdominal wall revealed a cutaneous retraction and a fistulous opening at the right end of the scar, without a palpable nodule .Fig. 1A A pelvic transabdominal ultrasound was performed, showing a fistulous tract extending from the anterior body of the uterus, that shows a focal myometrial thinning related to the cesarean section scar, to the right end of the cutaneous scar. It was approximately 2 mm in diameter, 31 mm in length .Fig. 2TrA pelvic computed tomography (CT) was performed with injection of diluted iodinated contrast medium through the cutaneous fistulous opening, showing the opacification of the fistulous tract described on ultrasound, as well as the uterine cavity, confirming the diagnostic diagnosis .Fig. 3PePelvic magnetic resonance imaging (MRI) was performed. The fistulous tract showed low signal intensity on T1-weighted sequences, low signal intensity on T2-weighted sequences, high signal intensity on T2-weighted sequences with fat saturation, and enhanced after intravenous Gadolinium contrast medium administration .The surgical management was successful. It consists of total excision of the fistulous tract with uterus conservation. It leads to the disappearance of cyclic blood discharge and pain.The histopathological examination was compatible with parietal endometriosis.Utero-cutaneous fistula is an extremely rare condition, with few cases being reported in the literature. It is an abnormal communication between the cutaneous surface and the uterine cavity. The main manifestation is blood discharge from the cutaneous fistulous opening during the menstrual period, which is almost pathognomonic The pathophysiology is not well understood. Several possible causes may predispose to this condition. Most of the reported cases were consecutive to Cesarean sections. Others were causes reported such as multiple surgeries in the abdomen, use of drains, incomplete closure of incisions, septic abortion, pelvic abscesses, incomplete placenta removal, uterovaginal malformation, pelvic actinomycosis due to intrauterine devices, curettage, difficult delivery, or use of forceps Multiple imaging techniques confirming the diagnosis had been reported. Fistulography with the injection of the contrast material through the cutaneous fistulous opening confirms the connection to the uterus. Hysterosalpingography with a methylene blue injection through the cervix is used when the cutaneous opening is too small. CT scan with intravenous injection of contrast medium and MRI can also identify the fistula. Hysteroscopy allows direct visualization of the internal fistulous opening The primary treatment of utero-cutaneous fistula is surgical excision of the fistulous tract without or with a subtotal or total hysterectomy Gonadotropin-releasing hormone agonists would induce amenorrhea secondary to endometrial atrophy, resulting in contracture and healing of the fistulous tract. It can be used on its own for a fistulous tract of small size In our case, the diagnosis was confirmed by CT scan with an injection of contrast medium through the cutaneous fistulous opening. The treatment consisted of the total excision of the fistulous tract, without hysterectomy, with a good clinical outcome.Written and informed consent for publication of the case was obtained from the patient.


Surgical resection of the tumor was accomplished, as well as skin flap reconstruction, obtaining radical removal and satisfying wound healing. PEMs could suggest an infiltrative and aggressive behavior, which has never found a histopathological correlation with a malignancy . Whole-body 68Ga-DOTATOC PET/CT should be considered in the patient’s global assessment. Surgical removal is a resolutive treatment, and the examination of frozen sections can confirm the benignity of the lesion, reducing the extension of the removal of healthy tissue surrounding the tumor.Meningiomas represent the most common benign histological tumor of the central nervous system. Usually, meningiomas are intracranial, showing a typical dural tail sign on brain MRI with Gadolinium, but occasionally they can infiltrate the skull or be sited extracranially. We present a systematic review of the literature on extracranial meningiomas of the head and neck, along with an emblematic case of primary extracranial meningioma (PEM), which provides further insights into PEM management. A literature search according to the PRISMA statement was conducted from 1979 to June 2021 using PubMed, Web of Science, Google Scholar, and Scopus databases, searching for relevant Mesh terms  AND (head OR neck). Data for all patients were recorded when available, including age, sex, localization, histological grading, treatment, possible recurrence, and outcome. A total of 83 published studies were identified through PubMed, Google Scholar, and Scopus databases, together with additional references list searches from 1979 to date. A total of 49 papers were excluded, and 34 manuscripts were considered for this systematic review, including 213 patients. We also reported a case of a 45-year-old male with an extracranial neck psammomatous meningioma with sizes of 4 cm × 3 cm × 2 cm. Furthermore, whole-body  Meningiomas represent the most common benign histological tumor of the central nervous system; they originate from cellular elements of the meninges, arachnoid cap cells, arachnoid granulations, subarachnoid blood vessels, fibroblasts, and pia mater . UsuallyA literature search was conducted from 1979 to June 2021 (last published studies in 2020) using PubMed, Web of Science, Google Scholar, and Scopus databases, searching for the following Mesh terms:  AND (head OR neck). Papers not written in English were excluded from this study. The PRISMA flow diagram for our search is outlined in One reviewer extracted data from the included articles, then confirmed independently by two additional reviewers. Missing data were either not reported in the original paper or could not be differentiated from other data. All included studies were meticulously reviewed and scrutinized for their study design, methodology, patient characteristics, and extraneural location. Data for all patients were recorded when available, including age, sex, localization, histological grading, treatment, possible recurrence, and outcome. The risk of bias was independently assessed by two reviewers using the Joanna Briggs Institute checklists for case reports and case series .A total of 83 published studies were identified through PubMed, Web of Science, Google Scholar, and Scopus databases and additional reference list searches from 1979 to date. After a detailed examination of these studies, 49 papers were excluded from our review because they reported localizations other than head and neck or cases of metastatic tumors, or they had no data available. Patients’ demographics are reported in Surgical resection was performed in 99.1% of patients (229/231). In the remaining four cases, two were partial resections  and one excisional biopsy , while the localization and the type of resection were not reported in one patient with neck disease.The distribution by type of response versus tumor localization is reported in A 45-year-old male was referred to our department complaining of a cutaneous posterior neck lesion, present since birth, characterized by a callous appearance and superficial protuberance, with a diameter of 4 cm × 3 cm and a thickness of 2 cm . Since t68Ga-DOTATOC PET/CT was performed, excluding a tumor spread in other body areas. After a multidisciplinary meeting, we decided to perform tumor removal and scalp reconstruction. The first stage of the procedure was conducted by neurosurgeons, performing a hockey stick skin incision exposing the occipital bone and the fistulous tract exposure  .Based on their histological and immunohistochemical characteristics, PEMs can be classified as common intracranial meningiomas . Epithel68Ga-DOTATOC PET/CT should be considered in the global assessment of the pathology. Surgical removal is the treatment of choice, and the examination of frozen sections can confirm the benignity of the lesion, reducing the extension of the removal of healthy tissue surrounding the tumor.PEMs could suggest an infiltrative and aggressive behavior, which has never found a histopathological correlation with malignancy . Considering the rarity of these tumors, clinical examination and neuroimaging are not pathognomonic, so the final diagnosis requires histological investigation. Whole-body


Splenic hematoma is a known complication of blunt force abdominal trauma. Traditional management of splenic hematomas has been primarily surgical. However, more recently, spleen-sparing management has been favored over surgical management for cases that meet certain criteria, with surgery now reserved for patients with complications. In this report, we present a case of a massive splenic hematoma that was managed conservatively and analyze the challenges faced in clinical decision making. The spleen and liver are commonly injured in cases of blunt force abdominal trauma such as motor vehicle accidents -2. SplenIn this report, we discuss a case of a 66-year-old patient who initially presented with abdominal pain, weakness, and anemia. This case presented a significant dilemma to us due to inaccurate history and poor recollection of events on the part of the patient, ultimately leading to his massive splenomegaly.A 66-year-old male with a past medical history significant for hypertension, diabetes, coronary artery disease, hepatitis C, benign prostatic hypertrophy, polysubstance abuse, and post-traumatic stress disorder with suicidal ideation presented to the Veterans Affairs Hospital's Emergency Department with abdominal pain, weakness, and anemia. He denied any history of falls or abdominal trauma.oF. Physical examination revealed a full, soft abdomen with no masses and normal bowel sounds. Laboratory studies showed mild leukocytosis, microcytic anemia with a hemoglobin of 6.7 g/dL, hematocrit levels of 25.1%, and thrombocytosis with a platelet count of 860 x 109/L. The chemistry panel was normal. Prothrombin time of 16.8 and activated partial thromboplastin time of 35.1 were noted. Chest X-ray showed a left lower lobe opacity suggestive of consolidation or pleural effusion. He was admitted for pneumonia and anemia.On initial evaluation, his vital signs were as follows: a blood pressure (BP) of 148/78 mmHg, heart rate (HR) of 122 beats per minute, respiratory rate (RR) of 16 breaths per minute, and temperature (T) of 97 A subsequent CT scan of the abdomen showed a very large (16 x 14 x 13 cm), complex cystic mass in the left upper quadrant (LUQ), epicentered in the region of the spleen, with peripheral enhancing nodularity. This was suggestive of a large necrotic mass, presumably within the spleen. No normal splenic tissue was seen. There was also a moderate-sized left pleural effusion with left lower lobe compressive atelectasis. The patient received intravenous fluids and blood transfusion and was started on a pain-management regimen. An abdominal ultrasound revealed a large (16 x 12 x 15 cm) solid mass in the LUQ.Surgery, Gastroenterology, and Hematology/Oncology services were consulted. The patient again denied any history of falls, blunt force trauma, or abdominal injury. He did report a poor appetite and weight loss. He also informed that he had prior hospitalizations in other area hospitals for similar complaints and had undergone a colonoscopy at one hospital, which had revealed internal hemorrhoids but no bleeding. CT angiography at the previous hospital had revealed no bleeding but had shown splenic, hepatic, and renal cysts. The case was discussed in the tumor board, where it was determined that the mass was not consistent with a hemorrhage. Working diagnoses included a pseudocyst, a complex splenic cystic, a hemorrhagic splenic cyst, a cystic splenic neoplasm, or a parasitic infection. The plan was to perform thoracentesis and send the pleural fluid for cytology and analysis for infection. The surgical recommendation was that he would likely need a splenectomy, and immunization was recommended.Helicobacter pylori. The EUS showed a large, 14-cm LUQ mass, with some hyper-echoic areas within the mass, which raised concerns about intra-tumoral bleeding or necrosis.Thoracentesis was performed with the analysis of the pleural fluid revealing an exudative effusion with 2+ polymorphonuclear neutrophils with no organisms seen. Cytology was negative for malignancy. An esophagogastroduodenoscopy with endoscopic ultrasound (EUS) showed a gastric polyp, which was biopsied with pathology and revealed minimally inflamed mucosa; there was no dysplasia or malignancy and it was negative for In light of the above-mentioned findings, a continuation of the conservative approach was pursued, and the patient's condition gradually began to improve. He was able to tolerate a diet; the pain became less severe and his counts were stable.We reviewed all of the patient’s imaging radiology, and on a closer look at the scans, the initially reported findings of “splenic cysts” were determined to be more suggestive of a contained splenic bleed. On further direct and focused questioning of the patient, it was revealed that he had indeed sustained a fall on his left side a few weeks prior to the development of his symptoms.A repeat CT scan showed a decrease in the size of the cystic lesion from 14 x 13 x 16 cm to approximately 8.6 x 8.1 x 10.5 cm. A follow-up CT scan three weeks later showed that the mass had now decreased to a size of 4.8 x 3.0 x 4.8 cm. The report on this last scan also noted that “the spleen overall is somewhat irregular in shape and in density suggesting this may represent a resolving traumatic injury to the spleen.” Figure As previously noted, there has been a shift in the approach to the management of splenic injury following abdominal blunt force trauma in recent years ,6. With A retrospective study conducted at the University of Texas at Houston, a level 1 trauma center, has provided some insight into the delayed complications of nonoperative management of blunt adult splenic trauma . The stuThis case highlights the aspects of conservative management of late complications of splenic injury from blunt force abdominal trauma. As noted above, patients who have severe injuries with either overt or occult bleed can become symptomatic shortly after the injury or within the first week to 10 days. Subsequently, many patients do reasonably well with serial observation, monitoring for late complications, and nonsurgical management as described in this case report.


Apical hypertrophic cardiomyopathy is a variant of hypertrophic cardiomyopathy that predominantly affects the apex of the left ventricle and rarely involves the right ventricular apex or both apexes. Heart transplantation is the traditional treatment for apical hypertrophic cardiomyopathy. Although surgical myectomy approaching the apex has been available for decades, its safety and accuracy greatly depend on the surgeon’s skills and experience.The first case involved a 63-year-old man with apical hypertrophic cardiomyopathy, wherein preoperative contrast computed tomography findings revealed apical hypertrophy and complete apical cavity obliteration. The patient underwent extended myectomy, which revealed the apex cavity filled with abnormal muscles. Using the transaortic approach, the location of the bilateral papillary muscle was confirmed, thereby providing the required orientation. The abnormal muscle mass was successfully resected, and the postoperative end-diastolic volume was extremely increased. The second case involved a 43-year-old man with an apical left ventricular aneurysm and mid-hypertrophic cardiomyopathy obstruction. The thin-walled apical aneurysm contained a large apical-basal band. Upon detecting the bilateral papillary muscle, mid-ventricular myectomy was performed from the apex. During postoperative catheterization, there was no pressure gradient between the left ventricle and aorta.We reviewed two cases of apical hypertrophic cardiomyopathy, efficiently treated using extended apical myectomy. Although it is an uncommon procedure, the cases presented show how it can be used to successfully manage cases of apical hypertrophic cardiomyopathy. However, it is important to secure the postoperative left ventricular end-diastolic volume. Apical hypertrophic cardiomyopathy (aHCM) is a variant of HCM first described in 1976. It predominantly affects the left ventricular apex and rarely involves the right ventricular apex or both apexes. Furthermore, aHCM presents unique echocardiographic findings of asymmetrical apical hypertrophy in an ace-of-spades configuration . ApproxiA 63-year-old man with an 8-year history of aHCM had remained healthy for 7 years until he was diagnosed with aHCM, during which he began to experience exertional dyspnea. He was referred to our hospital for surgical aHCM treatment. Preoperative contrast computed tomography (CT) revealed apical hypertrophy and complete apical cavity obliteration. Preoperative transesophageal echocardiography (TEE) revealed no systolic anterior motion (SAM) and an abnormal apical-basal band.The patient underwent extended myectomy, which revealed the apex cavity filled with abnormal muscles Fig. . TherefoPostoperative contrast CT findings showed a small LV aneurysm, which was surgically resected 3 weeks after the initial myectomy. On postoperative day 3, the patient developed septic shock because of urinary tract infection and mediastinitis. After antibiotic administration and omentopexy, he was transferred to his previous hospital where he underwent 90 days of rehabilitation. Postoperative TEE demonstrated an LV end-diastolic volume (LVEDV)/end-systolic volume of 105.3/56.5 mL . Postoperative left ventriculogram and angiography findings are presented in Fig. A 43-year-old man with an apical LV aneurysm and mid-HCM obstruction was referred to our hospital for further evaluation. Eight years earlier, he was fitted with an implantable cardioverter-defibrillator (ICD) for non-sustained ventricular tachycardia. Mid-HCM was detected using CT and transthoracic echocardiography (TTE), and he was followed up as an outpatient for 5 years. One month before presenting at our hospital, he developed syncope and palpitations.Preoperative contrast CT findings revealed an apical aneurysm and mid-ventricular obstruction. Preoperative TEE revealed mid-acceleration but no SAM. He underwent extended myectomy to alleviate palpitations and arrhythmia. A large apical-basal band was located inside the thin-walled apical aneurysm. Mid-ventricular myectomy was performed from the apex after detecting the bilateral papillary muscle. The resected myocardium weighed 9 g. The aneurysm was removed, and the incision was closed. Cardiopulmonary bypass lasted for 85 min, and the aorta was clamped for 50 min. Postoperative TEE findings revealed no SAM or ventricular septal perforation. During postoperative catheterization, there was no pressure gradient between the left ventricle and aorta.On postoperative day 8, the patient exhibited atrial flutter, and defibrillation was performed via an ICD, which restored sinus rhythm. He was discharged on postoperative day 10.LV septal myectomy is the gold standard surgical treatment for LV outflow tract obstruction (LVOTO) in patients with HCM . HoweverThe patient from Case 1 had extensive hypertrophy, especially in the apex; therefore, we could not perform myectomy using only the transaortic approach. However, using both apex and transaortic approaches, we were able to determine the positions of the bilateral papillary muscles, chordates, and mitral valve. This was our first case of treating aHCM using a bidirectional approach. In Case 2, extended myectomy was preferred because we considered that the removal of both the thin apex aneurysm and part of the septum was necessary, and we could secure the patient’s postoperative LVEDV. A comparison of pre- and post-CT scans in both cases, showed increased LVEDV, especially in the first one.Moreover, poor visibility caused considerable difficulty in the first case Table . TherefoAfter a discussion with our team of cardiovascular surgeons and cardiologists, we reached a consensus to perform these procedures rather than provide medical treatment for the patients of the two cases. Consequently, we hope that the positive outcomes from these two cases will lead to improvements in extended myectomy.Apical myectomies are rarely performed; however, there is a need to standardize this method. In Japan, heart transplantation for aHCM is not feasible because there are very few transplantation facilities. Heart transplantation is a longer procedure than extended myectomy. The latter can be challenging, but a detailed surgical procedure has been recently reported , 11–13.


Aggressive angiomyxoma of the vulva is a benign, slow-growing tumor originating from myxoid cells of connective tissue. The tumor is known for multiple local recurrences with a low tendency to metastasize. Only around 350 cases have been documented in the scientific literature so far.We report a case of a 40-year-old North Indian, unmarried woman with a swelling on the left labium majora for 7 years, along with surface ulceration over the mass. Local examination showed a well-circumscribed, 8 × 8 cm pedunculated  mass arising from the left labium majora with an overlying ulcer measuring 6 cm × 4 cm. After taking informed written consent, wide local excision of the mass and surrounding margins was done under anesthesia. Histopathology was suggestive of aggressive angiomyxoma. Immunohistochemistry was done, which was positive for estrogen and progesterone receptors. Her postoperative recovery was uneventful. The patient was given three doses of gonadotropin-releasing hormone agonist  once a month. No recurrence has been reported so far on follow-up visits for 1 year.Aggressive angiomyxoma is one of the differential diagnoses for vulvovaginal growth in a female. As the tumor is well known for local recurrences, correct diagnosis and appropriate management using a multidisciplinary approach are crucial to managing such patients. Aggressive angiomyxoma (AA) of the vulva is a benign, slow-growing, and locally invasive mesenchymal tumor originating from myxoid cells of connective tissue. It was first described in a case series of nine patients by Steeper and Rosai in 1983 . It is aA 40-year-old North Indian, unmarried, nulliparous woman came to gynecology Out Patient Department (OPD) with a swelling on the left labium majora for 7 years, slowly growing in size. There was ulceration over the mass associated with serosanguinous discharge. Past medical and family history was unremarkable. On general examination, the patient was moderately built and afebrile. There was no evidence of jaundice, anemia, cyanosis, lymphadenopathy, clubbing, weight loss, or any bowel and bladder function alterations. Her menstrual cycles were regular with normal flow. She was a known case of epilepsy and was on tablet levetiracetam 500 mg once daily. She had a history of hemithyroidectomy 6 years back and was on thyroxine tablet 50 μg once daily. Her abdomen was soft and nontender. On local examination, a well-circumscribed, 8 × 8 cm pedunculated mass arose from left labium majora lateral to introitus at five o’clock position. On palpation, the mass was nontender, nonreducible, and soft in consistency. There was an overlying ulcer measuring 6 cm × 4 cm, and the floor was covered with unhealthy pale granulation tissue. There was mild serosanguinous discharge from the ulcer Fig. . No inguAngiomyxomas are recognized as superficial (cutaneous myxoma) or aggressive. Superficial angiomyxomas occur more commonly in middle-aged patients presenting as a single nodule or a polypoidal mass in the head and neck region, trunk, and lower extremities. They are easily confused with skin tags, cysts, or neurofibroma and are commonly associated with Carney’s complex . AA is reported almost exclusively in women of reproductive age, with occasional cases seen in perimenopausal females and children. This may be attributed to the hormone-responsive nature of the tumor, as its growth is stimulated by estrogen and progesterone, backed by a case report of rapid enlargement during pregnancy . The tumHMGI-C) gene located in the region 12q13-15 of chromosome 12 seems to play a role in the pathogenesis of this tumor. Abnormal expression of this gene, HMGI-C, detected using the immunoperoxidase technique, may be a possible marker of microscopic residual disease [The exact pathogenesis of AA is still unclear. However, since the tumor cells express desmin and smooth muscle actin, it is believed to originate either from specialized mesenchymal cells or from the multipotent perivascular progenitor cells . High-mo disease .The patient is usually asymptomatic and presents with a slow-growing pedunculated mass in the vulva, gluteal region, or suprapubic region. Nonspecific symptoms such as dull aching pain, the feeling of local pressure, dysuria, urinary retention, or dyspareunia may be present. The size may range from a few centimeters to > 60 cm. The true extent of AA is frequently underrated on gross inspection as the clinically visible tumor exhibits only a fragment of the more widespread involvement of the deeper tissues of the pelvis and retroperitoneum.Clinically, the tumor needs to be differentiated from angiomyofibroblastoma, Bartholin gland cyst, sarcoma botryoides, superficial angiomyxoma, vulvar hypertrophy with lymphedema, lipoma, and sarcoma. The histological differential diagnosis includes myxoma, myxoid liposarcoma, myxofibrosarcoma, and nerve sheath myxoma. Marked vascularity in aggressive angiomyxoma helps to differentiate it from most of the above neoplasms. The absence of lipoblasts differentiates it from liposarcoma. AA may be commonly misdiagnosed as angiomyofibroblastoma. However, the latter is well circumscribed, more cellular, and more vascular.et al. conducted a study to evaluate the role of CT and MRI imaging techniques. They elucidated that both CT and MRI precisely predict the extent of the tumor. However, MRI is more specific and is superior to CT when ascertaining the tumor’s relation to the surrounding structures [Preoperative imaging is extremely important to see the extent of the tumor and plan surgical excision accordingly. On USG, it is seen as a hypoechoic or cystic mass. The mass shows a distinct feature of swirled and layered tissue on computed tomography (CT) and magnetic resonance imaging (MRI). Wu ructures . Thus, Met al., recurrent AA of the vulva was treated solely by 3 months of GnRH agonist without needing any other medical therapy or surgery [et al. described nine AA cases with local recurrence, all due to incomplete excision [et al. suggested multimodality treatment for AA: excision of the tumor without mutilation, adjuvant therapy such as arterial embolization or hormonal therapy, and radiotherapy in symptomatic patients resistant to embolization/hormonal therapy.Many treatment modalities have been tried with varying success. Radical surgical excision with negative margins is the conventional treatment of choice. However, it is not always possible to achieve negative resection margins as the tumor is locally infiltrative, leading to high operative morbidity. Therefore, less radical surgery is recommended nowadays. Adjuvant therapy in raloxifene, tamoxifen, or GnRH agonists like leuprolide acetate and goserelin have proven beneficial where the tumor is estrogen and progesterone receptor sensitive. In a case report by Fine  surgery . However surgery . Begin eexcision . Han-Gueet al. reported a death due to multiorgan metastasis invading the peritoneum, lungs, and lymph nodes [The tumor was conventionally considered to be nonmetastasizing. However, metastasis has been reported as an exceedingly rare event in literature. Siassi ph nodes . In anotph nodes . No evidAggressive angiomyxoma is a rare, locally aggressive neoplasm. It should be borne in mind as a differential diagnosis whenever a patient presents with growth in the vulvovaginal region, perineum, or pelvis. As the tumor is well known for local recurrences, timely diagnosis and management with surgical excision and adjuvant therapy are beneficial for such patients.


Nevus lipomatosus cutaneous superficialis (NLCS) is a rare cutaneous hamartoma characterized by mature adipocytes in the dermis. Here, we present a unique case of NLCS in a 57-year-old female that strikingly resembled an acrochordon, demonstrating features of the classical and solitary form of NLCS. This presentation of NLCS has not been widely reported and emphasizes that the diagnosis of NLCS should be considered when evaluating suspected acrochordons. Nevus lipomatosus cutaneous superficialis (NLCS) is a rare cutaneous hamartoma characterized by mature adipocytes in the papillary or reticular dermis ,2. CliniNLCS is almost always asymptomatic and has not been associated with malignant changes; however, there are reports of NLCS causing ulnar nerve entrapment as well as ulcerating after external trauma. NLCS has also been associated with multiple cutaneous disorders such as follicular papules, hypertrophic pilosebaceous units, angiokeratoma of Fordyce, café-au-lait macules, scattered leukoderma, and hemangioma .The histopathology of NLCS is characterized by the presence of ectopic fat in the reticular and papillary dermis, making up 10-50% of the lesion . This usA 57-year-old female presented with a non-painful mass on her lower back which she stated was present for some time. Physical examination revealed a solitary skin-colored, pedunculated nodule with a smooth surface on the sacral area Figure . The nodWe report a case of a solitary NLCS that strikingly resembled an acrochordon, including the fibrolipomatous variant . A diagnThe pathogenesis of NLCS is still unclear. Hoffman and Zurhelle postulated that the deposition of adipocytes in the dermis is secondary to degenerative alterations in connective tissue . Other tWe believe this case is unique as it highlights features of both the classical and solitary form of NLCS, placing it within the wider differential diagnosis for skin-colored pedunculated lesions Table  2,3]. A,3. A2,3]NLCS is managed with surgical excision. However, if left untreated, NLCS can increase in size, causing apprehension. Unlike acrochordons, NLCS is not associated with insulin resistance or metabolic syndrome. Patients with NLCS should be reassured that this condition is benign and not a known marker for other underlying conditions. In addition, further research is needed to properly classify NLCS subtypes and recognize that certain lesions may not fit well into existing classifications. Dermatologists and other practitioners should be aware of the possibility of an NLCS diagnosis when evaluating a patient with an isolated, pedunculated skin-colored nodule which may have the appearance of an acrochordon. Atypical acrochordae should be sent for histological review when a diagnosis of NLCS is possible.


Neuroblastoma represents the most common solid extracranial tumor in children under 5, accounting for 8% to 10% of all childhood cancers. Primary central nervous system (CNS) neuroblastomas are a very rare location and only few cases are available in the literature. It was first described in 1973 by Hart and Earl as supratentorial primitive neuroectodermal tumors. Clinical presentation is highly variable and depends on the initial location of the tumor. Regarding imaging, primary brain neuroblastoma shows no pathognomonic appearance on brain computed tomography (CT) whether or not enhanced or magnetic resonance imaging (MRI). There were no standard guidelines available for the adjuvant treatment in case of primary CNS neuroblastoma. Surgery remains the main and the first tool toward these lesions. Radiotherapy associated or not to chemotherapy is offered based on patient´s age. Here, the authors report a new pediatric case of primitive central nervous system neuroblastoma revealed by an intracranial hypertension syndrome and confirmed by both histopathological and immunohistochemistry study after a gross total surgical excision. The postoperative course was uneventful and the child had good recovery. Neuroblastoma represents the most common solid extracranial tumor in children under 5, accounting for 8% to 10% of all childhood cancers [Patient information: a 6-year-old boy with medical history of mild persistent asthma since the age of one year undergoing long-term control medications made of inhaled corticosteroids and who is followed in the department of child psychiatry for attention deficit hyperactivity disorder under psychological counseling was admitted in our department of neurosurgery for raised intracranial pressure of 6-month duration made of intractable holocranial throbbing headache with several episodes of vomiting and bilateral blurred vision outside any context of head trauma of associating fever.Clinical findings: upon neurological examination, he was perfectly conscious, a little agitated without any motor or sensory deficit or other localization signs. Examination of the fundus revealed a bilateral grade I papilledema. The rest of general physical examination of the patient did not show any abnormalities.Diagnostic assessment: a non-enhanced brain CT scan ( CT scan  was perf CT scan  confirme99mTc-MDP) uptake in the whole skeleton  scan  was perfskeleton . We did Therapeutic intervention: the patient underwent, through a frontotemporal craniotomy in supine position with his head rotated about 30° contralaterally and placed in a standard Mayfield skull clamp, a gross total excision of his tumor having, preoperatively, a firm and vascular appearance without definite plane with normal brain parenchyma. Hemostasis was laborious, the dura was tightly closed and the bone flap was returned and secured with four transosseous stitches.Follow-up and outcomes: the postoperative awakening was uneventful when general sedation was gradually stopped. Postoperative CT scan at day 2  neuroblastomas are a very rare location and few cases are available in the literature. It was first described in 1973 by Hart and Earl as supratentorial primitive neuroectodermal tumors. They are extremely variegated in histology. Primary CNS localization has often been classified as part of primary neuroectodermal tumors, although this is controversial. The diagnosis is confirmed by a surgical pathologist. On microscopy, tumor cells are usually described as small, rounded, bluish cells with rosette patterns (Homer-Wright pseudorosettes). A variety of immunohistochemical staining is used for histologic distinction of neuroblastomas from other similar pathologies such as rhabdomyosarcoma, Ewing's sarcoma, lymphoma and Wilms tumor. All these features were also suggested by Horten and Rubinstein . On elecClinical presentation is highly variable and depends on the initial location of the tumor, the size, as well as the local and distant extension . HoweverRegarding imaging, primary brain neuroblastoma shows no pathognomonic appearance on brain computed tomography (CT) whether or not enhanced or magnetic resonance imaging (MRI). Cystic or calcium formations can be observed in some patients accompanying the fleshy component which remains predominant. This feature agrees with our patient´s imaging with no particular radiological findings. However, exact data for the same is not available in the literature due to the rarity of the disease . Investiet al. [et al. [There were no standard guidelines available for the adjuvant treatment in case of primary CNS neuroblastoma. Surgery remains the main and the first tool toward these lesions. Radiotherapy associated or not to chemotherapy is offered based on patient´s age. In their study, Bennett et al.  suggeste [et al.  stated tWith current treatments, patients with intermediate and low risk of the disease tend to have a very good prognosis, with cure rates higher than 90% in low-risk patients and 70%-90% in intermediate-risk patients . In contConcerning prevention and screening, several attempts have been made to screen neuroblastomas by methods for detecting catecholamine metabolites in urine samples; screening programs since the 1980s have included 3-week-old newborns in Japan, 3-week-olds and 6-month-olds in Canada and 1-year-olds in Germany ,14. JapaPrimary CNS neuroblastoma remains a rare pathology with high risks of local or distant recurrence. It carries a severe prognosis due to its aggressive nature. The preoperative diagnosis is based on imaging although it is quite difficult. It should be considered as one of the differential diagnoses of tumors in the pediatric population. Radical surgery followed by adjuvant chemotherapy and/or radiotherapy appears to be the best treatment at present.


The report presents a case of a 70-year-old male with a known mesenteric neuroendocrine tumour and metastases to the liver diagnosed with acute cholecystitis. During surgery, the patient developed a carcinoid crisis with mixed distributive and cardiogenic shock involving systemic vasodilation and arrhythmia. During surgical procedures, carcinoid crisis can be precipitated by tumours that secrete a pathological shower of vasoactive mediators. Somatostatin analogues are utilized to control carcinoid syndrome and are routinely used peri-operatively. However, no standard infusion regimen exists. The case raises the suggestion that metastatic liver neuroendocrine disease may confound the diagnosis of cholecystitis, complicates the management of acute surgical presentations and highlights the need for agreement on octreotide therapy for surgical patients with carcinoid tumours. Neuroendocrine neoplasms (NENs) are derived from enterochromaffin cells mainly present in the gastrointestinal tract. NENs are subdivided into the poorly differentiated neuroendocrine carcinomas (NECs) and well-differentiated neuroendocrine tumours (NETs) and approximately 20% of patients with NENs experience carcinoid syndrome . CarcinoSomatostatin analogues act as inhibitory peptides on gastrointestinal proliferative and secretory processes and are the mainstay of management to prevent and treat carcinoid syndrome and crises. However, pre-operative octreotide acetate alone is not sufficient in preventing intraoperative carcinoid crisis even in patients undergoing liver resection , 9. Pre-Somatostatin analogue use is associated with impairment of gallbladder function, increasing the formation of bile crystals in the bile, inhibiting sphincter of Oddi relaxation and impairing gallbladder emptying. If not treated with somatostatin analogues, very few NEN patients (7%) suffer from symptomatic gallstones .9/L, C-reactive protein 212 mg/L and liver function tests within normal limits. Vital signs were reassuring, and an abdominal ultrasound described a thickened gallbladder wall, mobile sludge and an 8.5-mm common bile duct suggestive of cholecystitis  titrated over 7 hours and noradrenaline at 5 mL/h (5ug/min) titrated over 6 h. Later, cardiac angiography showed minor coronary disease and within 24 h, he was extubated, his pain resolved, and inflammatory markers had normalised within 48 h.During surgical procedures, NENs are prone to pathological neuroendocrine secretion of serotonin, tachykinins, histamine, kallikreins, prostaglandin and catecholamines. Pre- and intra-operative infusion with somatostatin can mitigate the risk of on-table carcinoid crisis by reducing the release of these vasoactive mediators. The American neuroendocrine tumour society (NANETS) claims that routine administration of octreotide does not prevent a carcinoid crisis but does advise an intraoperative 100–500 μg/h infusion . The EurHypotension is the most common problem during anaesthesia, and there is a theoretical risk of worsening hypotension by triggering further release of vasoactive peptides . HoweverTo date, there are no cases reported of carcinoid crisis at the time of laparoscopic cholecystectomy for cholecystitis. Performing the surgery required manipulation of the liver, which likely released vasoactive mediators precipitating the crisis. The crisis could, however, be explained by anaesthesia and although the CT demonstrated necrotic metastasis, the patient was previously asymptomatic making necrosis alone a less likely explanation.Treatment with somatostatin analogues is known to cause biliary stasis, which can sometimes become symptomatic and require surgical management. The patient in this case carried an increased risk of developing gallstones and subsequently suffering cholecystitis due to somatostatin analogue use. This case supports the practice of prophylactic cholecystectomy  in this


Indocyanine green (ICG)-guided near-infrared fluorescence has been recently adopted in pediatric surgery, although its use in the treatment of congenital hyperinsulinism has not been reported. We present a case of focal congenital hyperinsulinism in which ICG-navigation with ICG was used during surgical treatment. A 3-month-old infant was referred to our institution from a peripheral hospital for episodes of persistent hypoglycemia since birth, with no response to intravenous treatment with diazoxide, octreotide, or hydrochlorothiazide. An abdominal positron emission tomography-computed tomography scan showed a hypermetabolic nodule in the proximal portion of the body of the pancreas, compatible with focal congenital hyperinsulinism. A heterozygous mutation in the ABCC gene (Ala1516Glyfs*19) frameshift type inherited from the father was identified, which supported this diagnosis. Laparoscopy-assisted surgery was performed with ICG-guided near-infrared fluorescence, with intravenous injection of 16 mg ICG (2 mg/mg), which allowed localization of the focal lesion in the body of the pancreas. The lesion was resected with bipolar electrocautery and intraoperative histological study confirmed complete resection. Plasma glucose values normalized 6 hours after surgery and the patient was discharged 5 days later. In conclusion, the use of ICG in the treatment of congenital hyperinsulinism helps to identify hypermetabolic pancreatic nodules, decreasing the likelihood of incomplete resection. In the last few years, the recent advent of fluorescence-guided surgery has changed the intraoperative decision process, essentially through the use of indocyanine green (ICG).Although most of the current applications of ICG are mainly reported for adult surgery, there has recently been an exponential increase in the number of publications in pediatric surgery.A 3-month-old infant was referred to our institution from a peripheral hospital for episodes of persistent hypoglycemia since birth. Pregnancy underwent an appropriate prenatal care and an unremarkable prenatal course, with an emergency cesarean delivery at 40 + 2 weeks due to fetal tachycardia. Since birth he presented persistent hypoglycemia in the first hours of life, confirmed by several blood glucose determinations, with need for continuous enteral nutrition by nasogastric tube from the second day of life, with no response to intravenous treatment with diazoxide, octreotide, or hydrochlorothiazide.On admission, the patient weighed 8.2 kg, and required continuous glucose supplementation via nasogastric tube (17 mL/h) to avoid hypoglycemia. Physical examination revealed a macrosomic appearance with mild axial hypotonia. The abdomen was soft and depressible, with no palpable masses and no other findings of interest. An abdominal 18F-fluoro-L-dihydroxyphenylalanine (18F-DOPA)-positron emission tomography/computed tomography scan showed a hypermetabolic nodule in the distal portion of the body of the pancreas, compatible with focal congenital hyperinsulinism . A geneLaparoscopy-assisted surgery was performed with ICG-guided near-infrared fluorescence, with intraoperative intravenous injection of 16 mg ICG (2 mg/kg) once the pancreas was identified, without need of injecting it prior to surgery, which allowed localization of the focal lesion in the body of the pancreas . A lapaCongenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in neonates and can lead to irreversible brain damage.The main difficulty in the treatment of focal CHI is the intraoperative location of the lesion. Focal lesions often have subtle differences in appearance, ranging from a slightly reddish color to a marble-like appearance, and are often slightly firmer compared with normal tissue. Intraoperative high-resolution ultrasound can sometimes help in localizing a focal lesion particularly if the lesion has a pseudocapsule, but most focal lesions have similar echogenicity to normal pancreas.Fluorescence-guided surgery with ICG allows precise localization of the focal CHI lesion, helping to identify it intraoperatively and to determine the margin of resection to perform a limited resection, avoiding repeated biopsies. ICG is a water-soluble tricarbocyanine dye that produces fluorescence when excited using near-infrared light with a specific wavelength light (∼820 nm), and it is visualized using specific cameras to be transformed and displayed as visible light.To the best of our knowledge, we present the first case of focal CHI in which ICG-navigation was used during surgical treatment. In our experience, ICG technology can facilitate the identification of hypermetabolic nodules in focal CHI, decreasing the likelihood of incomplete resection. It is very useful for safe dissection of hyperfunctioning pancreatic masses because fluorescence allows a more precise identification of the resection margins as well as the pancreatic vascular anatomy and its vascular relationships with other main vessels. To perform this procedure, it is essential to have equipment with compatible technology, which is the main limitation of this technique.