Bladder cancer is the 17th most common cancer in the female population. Most bladder cancers are of urothelial origin. Sarcomas of the bladder are very uncommon.This case concerns a 57-year-old female from a rural town in the Eastern Cape who was diagnosed with myxoid/round cell liposarcoma of the bladder. After the initial transurethral resection of the bladder tumour, she underwent an anterior exenteration with Bricker’s diversion  and negative margins were achieved.Sarcomas of the bladder are known to have a poor prognosis. Our patient is alive with good stoma function more than 24 months since her radical surgery. We are of the opinion that her favourable overall survival is attributable to early radical surgical intervention with negative margins. Of all human cancers, bladder cancer ranks 17th among the commonest in the female population with an incidence of approximately 70,000 cases per year [In July 2018, a female patient  was referred to the outpatients department of the Port Elizabeth Provincial Hospital. The referring doctor was concerned about her persistent microhematuria and dysuria. On having her history taken, the patient mentioned a long period (± 30 years) of intermittent macrohematuria, along with occasionally passed clots. This was associated with pain over the bladder and with lower urinary tract symptoms (LUTS) in the form of urgency, urgency-incontinence, and dysuria. She reported no history of smoking, and she had been a housewife all her life with no obvious occupational exposure to carcinogens. She had well-controlled hypertension and no other known comorbidities. Her chronic medication included daily calcium-channel blockers, thiazide diuretics, and aspirin. Regarding gynaecological history, her menopause started in her mid-40 s, she has 6 children  and had a bilateral tubal ligation in 1991. She reported no other surgical history as well as no family history of cancer.On clinical examination, she appeared healthy with stable vitals and a soft abdomen with adiposity and no palpable masses or organomegaly. Her vaginal examination revealed no abnormalities with no notable masses, blood, or observed incontinence. Her urine dipstick demonstrated microhematuria. A urine specimen was subsequently sent to our laboratory for microscopy, culture, and sensitivity (MCS) testing. The laboratory findings included numerous leukocytes and erythrocytes, a bacterial urinary tract infection (UTI), namely Escherichia coli and Enterococcus faecalis, but no Schistosoma ova.A kidney-ureter-bladder (KUB) ultrasound revealed right hydroureteronephrosis and a large, irregularly shaped, heterogenous mass with internal vascularity at the base of the bladder Figs.  and 2.Fi2), most likely because of her chronic hypertension, and this complicated matters regarding further imaging. The ideal imaging of choice would have been a contrasted computed tomography (CT) of her abdomen and pelvis, but we did not want to risk further renal deterioration because of contrast nephropathy. At our resource-challenged facility, we had a very long waiting period for magnetic resonance imaging (MRI) and therefore proceeded with a noncontrasted computed tomography scan of the abdomen and pelvis in order to prevent further delays in definitive treatment. The results confirmed the presence of the right hydroureteronephrosis and a bladder mass of 6.3 cm by 6.9 cm  grade 2.With regard to immunohistochemistry, the tumour stained positive for Vimentin, S100 and CD10; and stained negative for 34BE12, CK7, CK20, p63, CK5/6, CD45, CD117, GATA3, Desmin, SMA, P504S, Melan A and HMB45. The Ki-67 proliferation index was ± 10–15%.A diagnosis of myxoid/round cell liposarcoma of the bladder was made. Subsequently, it was decided to perform an anterior exenteration and Bricker’s diversion . Although an anterior exenteration is regarded as having multiple complications (intra- and post-operatively), the surgery was successful with no adverse intra-operative events. The histology of the specimen confirmed the initial diagnosis of myxoid/round cell liposarcoma of the bladder. Infiltration into the muscularis propria with numerous lipoblasts in a myxoid stroma was also noted. The tumour did not extend through the muscularis propria, and there was no vascular or perineural invasion. The adnexa, distal ureters, pelvic lymph nodes, and uterus were all tumour-free. There was an incidental finding of a leiomyoma of the uterus.Post-operatively, the patient’s stay was unfortunately complicated by chronic diarrhoea with profound hypokalaemia. Her condition was further complicated by an acute kidney injury (AKI). She also developed venous thrombo-embolism (despite prophylaxis with subcutaneous unfractionated heparin), with bilateral pulmonary emboli and ileo-femoral thrombi. After a couple of weeks, the patient recovered from the diarrhoea and AKI. She received anti-coagulation therapy and had an inferior vena cava filter inserted by the hospital’s vascular surgeons. The patient was also diagnosed with HIV and an Absolute CD4 count of 333. She was thus referred to the Internal Medicine department of the same hospital for initiation of anti-retroviral therapy.During further long-term follow up consultations at our facility, the patient developed a single UTI for which she was treated. There have been no stoma-related complications, and, at present, the stoma is functioning well. No local or regional recurrence of the tumour has been noted with follow up CT scans. More than 24 months since radical surgery, it can be confirmed that the patient is alive and well.Sarcomas of the bladder are very rare, even more so a liposarcoma of the bladder. This is emphasised by a review of 1583 adult soft tissue sarcomas, of which only 10 had tumours that originated in the bladder . FurtherThe etiology of sarcomas is mostly idiopathic with ionizing radiation having been identified as the most common causative environmental factor. Some genetic syndromes associated with sarcomas include retinoblastoma, neurofibromatosis type 1, familial adenomatous polyposis syndrome, and Li-Fraumeni syndrome . BladderAccording to Levy et al., radiologically, myxoid liposarcoma contains an abundant myxoid matrix with a high water content. On CT imaging, it appears hypoattenuating. Magnetic resonance imaging (MRI) findings include hypointense lesions on T1-weighted images and hyperintense lesions on T2-weighted images. Enhancement of the myxoid matrix is an important feature that distinguishes this from a cystic mass and is better demonstrated with MRI rather than with a CT scan. Characteristically, thick enhancing septa, which correspond to fibrous bands running through the myxoid stroma, course through the mass. An enhancing patchy or nodular soft tissue component that demonstrates intermediate signal intensity on T1- and T2-weighted MRI images is also present. Areas of mature fat may be identified within the lesion . About 3The foundation for definitive treatment of operable bladder liposarcoma is surgical resection that ensures negative surgical margins. In the case of metastatic disease, however, soft tissue sarcomas are treated mostly with palliative cytotoxic chemotherapy. In such cases, the chemotherapy agent of choice for myxoid liposarcoma is doxorubicin-based chemotherapy .., the aggressiveness of myxoid liposarcomas is determined by the contribution of the round cell component, where more than 5% of such round cell component carries a poor prognosis [Three important prognostic factors for adult genito-urinary sarcomas have been identified: site; size; and grade of tumour . Accordirognosis . A typicrognosis .From the above discussion, it is clear that the prevalence of myxoid/round cell liposarcoma is sporadic and the prognosis is weak. Once accurately diagnosed, doctors may thus be confronted with difficult decisions in terms of management and treatment.In general, the prognosis of any bladder sarcoma is poor. Sufficient convincing data regarding bladder sarcoma response to chemotherapy and/or radiotherapy are scarce, given the paucity of cases. More specifically, effective management and treatment of myxoid/round cell liposarcoma, as a rare and uncommon tumour of the bladder, is unclear. Our case report suggests that early surgical control with negative surgical margins could be considered a potential intervention to improve patient overall survival.


Lipodystrophies are a group of diseases which are characterized by abnormal adipose tissue deposition and are frequently associated with metabolic changes. Congenital generalized lipodystrophy is an autosomal recessive syndrome, with a prevalence < 1:10 million. Acromegaly is a rare disease, secondary to the chronic hypersecretion of growth hormone and insulin-like growth factor-1, with characteristic metabolic and somatic effects. “Acromegaloidism” is a term used for patients who manifest clinical features of acromegaly, but do not present a demonstrable hormone growth hypersecretion. The extreme shortage of subcutaneous adipose tissues and muscle hypertrophy confer an acromegaloid-like appearance in these patients.We describe a case of a patient with the rare combination of Berardinelli–Seip congenital lipodystrophy and acromegaly; our patient is a 63-year-old white man, who was referred to an endocrinology consultation for suspected lipodystrophy. He had lipoatrophy of upper and lower limbs, trunk, and buttocks, with muscular prominence, acromegaloid facial appearance, large extremities, and soft tissue tumescence. In addition, he had dyslipidemia and prediabetes. His fat mass ratio (% trunk fat mass/% lower limbs fat mass) was 1.02 by densitometry and he also had hepatomegaly, with mild steatosis , and left ventricular hypertrophy (from an electrocardiogram). His first oral glucose tolerance test had growth hormone nadir of 0.92 ng/mL, and the second test, 10 months afterwards, registered growth hormone nadir of 0.64 ng/mL . Pituitary magnetic resonance imaging identified an area of hypocaptation of contrast product in relation to a pituitary adenoma and he was subsequently submitted to transsphenoidal surgical resection of the mass. A pathological evaluation showed pituitary adenoma with extensive expression of growth hormone and adrenocorticotropic hormone, as well as a rare expression of follicle-stimulating hormone and prolactin.AGPAT2 gene in homozygosity.A genetic study revealed an exon 3/exon 4 deletion of the Congenital generalized lipodystrophy is a rare disease which occurs with acromegaloid features. As far as we know, we have described the first case of genetic lipodystrophy associated with true acromegaly.Although this is a rare association, the presence of congenital generalized lipodystrophy should not exclude the possibility of simultaneous acromegaly. Lipodystrophies are a group of genetic or acquired diseases characterized by abnormal adipose tissue deposition, including peripheral fat loss (lipoatrophy) and central fat accumulation , which could be present separately or combined in the same individual –3. TheseFurthermore, the extremely low levels of leptin, resulting from subcutaneous adipose tissue loss, play an important role in the pathophysiology of the associated comorbilities .AGPAT2; BSCL type 1) and BSCL2 (BSCL type 2) are the most common variations [Congenital generalized lipodystrophy (CGL) or Berardinelli–Seip congenital lipodystrophy (BSCL) is a well-defined syndrome which is characterized by a generalized absence of adipose tissue from birth or shortly after, as well as severe insulin resistance , 8. The riations . Howeverriations , 8. TherThe AGPAT2 protein belongs to the family of AGPATs. These enzymes are fundamental for the biosynthesis of glycerol-3-phosphate triglycerides and phospholipids. The protein is highly expressed in adipose tissue, and its deficiency can cause lipodystrophy by inhibiting/reducing the synthesis of triacylglycerols and its storage in adipocytes. It is likely that a low activity of AGPAT2 could also increase the tissue levels of lysophosphatidic acid, which, in turn, could affect the functions of adipocytes , 11.Ever since Berardinelli described a very rare case of generalized congenital lipodystrophy in a 2-year-old boy from Brazil in 1954 , nearly With a prevalence of 38 to 80 cases per million, acromegaly is a rare disease. It is an insidious disease, which is secondary to the chronic hypersecretion of growth hormone (GH) and insulin-like growth factor-1 (IGF-1), with its characteristic metabolic and somatic effects. Most cases of acromegaly are caused by pituitary adenoma and the first-choice treatment of these cases is transsphenoidal surgery .The extreme shortage of subcutaneous adipose tissue and other adipose tissues, and muscle hypertrophy, confer an acromegaloid-like appearance in patients with CGL ; howeverA 63-year-old white man Fig. , who wasHe experienced uncomplicated prenatal and postnatal periods, with normal developmental milestones.His 59-year-old sister has a similar phenotype, and had generalized lipoatrophy since childhood, without diabetes or hypertension, and with normal IGF-1. A physical examination revealed that neither of the parents had lipodystrophic changes and neither had diabetes.2; waist circumference 91 cm; blood pressure 119/75 mmHg; and near-total absence of subcutaneous fat on his upper and lower limbs, trunk, and buttocks, which is suggestive of generalized lipoatrophy. His facial appearance demonstrated a notable acromegaloid appearance, with thick lips, widening of the wings of the nose, creased nasolabial grooves, dental diastema, and prominence of the supra-ciliary arches, with slight prognathism. He also had muscular prominence, large hands and feet, and soft tissue tumescence, without acanthosis nigricans.The results of our patient’s physical examination were: weight 95.4 kg; height 1.72 m; body mass index (BMI) of 32.24 kg/mBiochemical tests Table  revealedRenal function, hepatic function, gonadal function, prolactin (PRL), morning adrenocorticotropic hormone (ACTH), and cortisol were all normal.The fat mass ratio (FMR) by bone densitometry (% trunk fat mass/% lower limbs fat mass) was 1.02 (FMR in men ≥ 1.961 discriminates lipodystrophy ), withouTotal colonoscopy and upper digestive endoscopy were normal. Hepatosplenomegaly (17 cm) with mild fatty liver was observed in an abdominal ultrasound. In an electrocardiogram, left ventricular hypertrophy was observed; mild concentric left ventricular hypertrophy with mean ventricular ejection fraction of 67% was observed in an echocardiogram.In the pituitary magnetic resonance imaging  and Sanger sequencing revealed absence of amplification of exons 3 and 4. In order to clarify this fact, deletions/duplications located in the AGPAT2 gene  demonstrated no amplification of the intron 3/exon 3 region for the AGPAT2 gene, which pointed to an exon 3/exon 4 deletion of the AGPAT2 gene in homozygosity, previously described [The genetics analyses of escribed , 18.Our patient was prescribed a diet, exercise, lifestyle modification, metformin 700 mg twice a day, atorvastatin 20 mg, and levothyroxine 75 μg for diabetes, dyslipidemia, and CH, respectively. Three months after pituitary surgery, his IGF-1 level of 172 ng/mL  with true acromegaly is the first to be reported in the literature. The diagnosis of acromegaly was challenging due to the GH/IGF-1 discrepancy, which was surpassed by the use of a cut-off which was adjusted to the assay. Although this is a rare association, the presence of CGL should not exclude the possibility of simultaneous acromegaly.This case of CGL type 1 (


Cystic lymphangiomas are a rare entity in adults. It is commonly congenital due to obstruction in the lymphatic drainage. We report the case of a 45-year-old female who was admitted with complaints of a lateral neck swelling associated with multiple palpable cervical lymph nodes. Patient was evaluated and relevant investigations were carried out. Her radiological investigations reported conflicting results of lymphatic cyst, branchial cyst and infected cyst or abscess. However, after an excisional biopsy when the sample tissue was sent for histopathology, the results featured a lymphangioma, which is a rare finding in adults of this age. Lymphangiomas are deformities of the lymphatic system characterized by lesions that are thin-walled cysts. The lymphatic system is a connection responsible for reallocating extra fluid from tissues and lymph nodes to the venous channels, which detect any microorganism or trigger agents. Just as haemangiomas occur in blood vessels, lymphangiomas occur in lymphatics. It was first described by Redenbacher in 1828 [They could also be capillary or cavernous or cystic. Capillary lymphangiomas are present in the form of skin vesicles at the junction of the body to the extremities like axilla, groin and buttock, whereas cavernous lymphangiomas are classically present as lobulated, transilluminant swellings . CapillaA 45-year-old female presented with complaints of swelling over the right lateral aspect of neck for the last 10 years associated with mild pain over the swelling. Occasionally, the patient also complained of difficulty in breathing and restricted opening of her mouth. She did not complain of any difficulty in swallowing. There was no history of any previous infection like fever, tooth ache or trauma or any other medical condition. Initially, the swelling was small in size and gradually progressed to a size of 10 × 12 cm, extending 4 cm below the lower border of mandible to upper border of clavicle, medial border being 4 cm lateral to midline and lateral border extending up to posterior triangle. On examination, the patient was pale and the swelling was measured to be of size 10 × 12 cm over the right lateral aspect of neck, which did not move on deglutition, was soft and cystic in nature, mobile, non-tender, no local rise in temperature, lateral border of the swelling was extending up to the posterior triangle, and medial border and superior border could not be made out, whereas the inferior border extended up to the clavicle. There was no discharge, redness, sinus from swelling and the overlying skin appeared normal. On intraoral examination, no abnormal findings were noted. Multiple palpable lymph nodes in the cervical region were noted. The nerve supply seemed to be in order.The baseline investigations for complete blood count, routine urine test and renal function test were reported within normal limits. Ultrasonography of the neck revealed a large cystic swelling of size 13 × 9 × 8 cm (419 cc volume) on the right cervical region extending from the level of hyoid bone to clavicle, with external echoes, no vascularity and no calcification. Carotid vessels were pushed medially in inferior aspect and posteriorly in superior aspect. Multiple enlarged nodes in the cervical region were present with the largest measuring 1.2 cm. The impression was reported as an infected cyst/abscess in right cervical region. A magnetic resonance imaging (MRI) of neck was also performed, which showed a well-defined cystic lesion 13 × 8 × 8 cm extending from level of mandible to right supraclavicular fossa, displacing larynx and trachea to left, carotid vessel displaced medially. The impression was reported as a second branchial cyst or lymphatic cyst.The plan for this patient was excision biopsy as there was uncertainty in the diagnosis as well as prognosis with a horizontal incision 3 cm below the mandible (Risdon’s incision). This involved complete removal of vesicles in order to prevent any further recurrences, although the risk of complications like scar and disfigurement significantly rises . The excPost-operative period was uneventful. There was no neurological impairment. Patient was on antibiotics, analgesics and proton pump inhibitors. Biopsy report confirmed the diagnosis as lymphangioma. It had clusters of lymphoid tissue, large underdeveloped lymphatic channels with unorganized smooth muscle cells in its walls surrounded by connective tissue. It also contained vascular spaces lined by epithelial cells, which were thickened in some places .Lymphangiomas are the lymphatic malformation commonly seen on the neck; however, uncommon in adults and the exact aetiology of which remains unclear in adults. It is often confused with branchial cyst, thymic cyst, pericardial cyst, cystic teratoma and bronchogenic cyst. Generally, all the lymphangiomas remain asymptomatic with no sex predilection. They are soft and progressively growing masses, which may cause compressive symptoms when encroaching surrounding structures of the neck.Our case was an atypical one as the patient presented with a single large swelling on the side of the neck with no previous history of trauma or infection. The patient underwent an excisional biopsy to reach the final diagnosis.The patient was followed up for 4 months and patient was doing better. As both the radiological and clinical results were conflicting and amongst the radiological investigations, none came to a single diagnosis, the case was diagnosed clinically as lymphatic cyst with the cystic nature of the swelling and the upper borders, which remained non-palpable. Later the swelling was excised under general anaesthesia and the specimen was sent to histopathology for confirming the diagnosis as cystic lymphangioma. Hence, histopathology proved to be useful in reaching a final diagnosis.Although clinical evaluations of such swellings may lead to a diagnosis, but it may need further investigations to distinguish it from other types of neck swellings . RadioloThe patient was regularly followed up and looked out for any signs of recurrence, which in any case could be treated by marginal or segmental resection.In conclusion, lymphangiomas are common in paediatric age group and a rare entity in older age group. Owing to the vague clinic-radiographic characteristics, it is frequently misdiagnosed. However, surgical excision and histopathology study of the specimen is the only option for confirming the diagnosis. In this case, the radiological report ruled out the malignancy and gave diagnosis of lymphatic cyst. However, the histopathology reports revealed the swelling to be lymphangioma. Patient was followed up for 4 months continuously and was stable. Also, this highlights the radiological reports that led to the misdiagnosis. Radiographic reports although more frequently relied on by surgeons proved to be deceiving. The gold standard, hence, for diagnosis of lymphangiomas remains histopathology and cytology study along with long-term follow-up for recurrences.The case report also highlights the difference in the diagnosis when comparing the histopathological and radiological reports. This makes the diagnosis of cystic lymphangioma in adults all the more challenging.A written and informed consent was taken from the patient for the publication of her case as a report.None declared.


A 58-year-old female patient presented with a single-digit clubbing on the second finger of her right hand two years previously. After investigation with imaging and incisional biopsy, superficial acral fibromyxoma was diagnosed. A brief review on single-digit clubbing and its causes is presented, focusing on superficial acral fibromyxoma. A 58-year-old hypertensive woman reported a progressive increase in volume in the distal phalanx of the second right finger for two years, asymptomatically. On examination, she presented hypertrophy of the distal phalanx associated with increased nail bed convexity, suggesting single-digit clubbing  and confWhat is your diagnosis?A.EnchondromaB.Osteoid osteomaC.Myxoid cystD.Superficial acral fibromyxomaHistopathology assessment showed dermal proliferation of spindle and stellate cells without atypia, within a myxoid stroma with moderate proliferation of small vessels, suggestive of neurofibroma or superficial acral fibromyxoma (SAFM). Immunohistochemistry assessment was requested to distinguish between the two tumors; cells positive for vimentin, CD34, and KI-67 (1%), and negative for S-100  were obsDigital clubbing is characterized by a focal increase in the terminal segments of the fingers due to the proliferation of connective tissue between the nail matrix and the distal phalanx. For confirmation, it is recommended to calculate the profile angle, which should be greater than 180°, and the ratio of the depth of the distal phalanx to the interphalangeal joint, which should be greater than 1.2Single-digit clubbing is a rare condition, usually caused by an expansive process in the distal phalanx. Enchondromas, osteoid osteoma, myxoid cyst, and myxochondromas have been described as the cause of this condition.SAFM is a rare and benign soft tissue tumor, usually with slow and painless growth. It usually affects the periungual and subungual regions of the fingers and toes. Its radiographic findings are nonspecific.5Histologically, there is dermal or subcutaneous proliferation of fusiform and stellate cells within a myxoid, mixoid-collagenous, or collagenous matrix, with prominent microvasculature and mast cell infiltrate. Mild nuclear atypias and mitoses may be observed. Immunohistochemistry assessments are positive for CD34, CD99, vimentin, and CD10, and negative for S-100.8Differential diagnoses include myxoid tumors and those that affect the distal part of the limbs, such as dermatofibrosarcoma protuberans, myxoid neurofibroma, fibrous histiocytoma, acquired digital fibrokeratoma, acral myxoinflammatory fibroblastic sarcoma, sclerosing perineurioma, superficial angiomyxoma, and low-grade fibromyxoid sarcoma, in addition to the previously mentioned causes of single-digit clubbing.9In acquired digital fibrokeratoma, CD34 may be positive, but it differs histopathologically, as it presents pronounced hyperkeratosis and acanthosis, low cellularity, and thick collagen bundles parallel to the long axis of the lesion. Low-grade fibromyxoid sarcoma is negative for CD34, unlike SAFM, which is positive.Treatment consists of complete excision of the lesion.None declared.Larissa Crestani: Approval of the final version of the manuscript; elaboration and writing of the manuscript; intellectual participation in propaedeutic and/or therapeutic conduct of studied cases; critical review of the literature; critical review of the manuscript.Isaura Azevedo Fasciani: Critical review of the literature; critical review of the manuscript.Priscila Kakizaki: Approval of the final version of the manuscript; intellectual participation in propaedeutic and/or therapeutic conduct of studied cases; critical review of the literature; critical review of the manuscript.Neusa Yuriko Sakai Valente: Approval of the final version of the manuscript; intellectual participation in propaedeutic and/or therapeutic conduct of studied cases; critical review of the literature; critical review of the manuscript.None declared.


Oncocytic adrenal neoplasms are rare and mostly benign lesions. Available literature supports indication for a surgical resection, but criteria to predict aggressive behavior are unreliable, thus making decision of surgical approach (laparotomy versus laparoscopy), and extent of resection, difficult to define.This is the case of a 46-year-old male, with an incidental finding of a 10 cm asymptomatic tumor in the left adrenal gland identified by MRI, performed in the setting of the initial assessment of liver steatosis. Adrenal hormone levels were in the normal range, thus, a CT-guided needle biopsy was performed and showed an adrenocortical oncocytic neoplasm. A laparoscopic left adrenalectomy was performed sparing the adjacent left kidney. Histological examination of the resected tumor showed a 10 cm oncocytic adrenocortical neoplasm of uncertain malignant potential with negative resection margins. A follow-up MRI was scheduled at six months after surgery, and no recurrence was found.Although rare, oncocytic neoplasms should be included in the differential diagnosis of adrenal “incidentalomas”. Determination of their malignant potential is difficult in the preoperatory setting. Final diagnosis is based on histological analysis of the whole surgical specimen. Laparoscopic complete excision with negative resection margins is feasible and safe. HeterotThe preoperative evaluation of possible malignancy, the optimal surgical approach and the extent of resection  remain challenging .The work has been reported in line with the SCARE criteria .22) had no oncologic past medical history. He had no relevant family history of endocrine diseases and was on antidepressant medication . The initial assessment of the hepatopathy with abdominal MRI incidentally revealed a heterogeneous lesion of the left adrenal gland, measuring 62 × 69 mm, with a spontaneous hyperintense T1 signal  with a necrotic center and calcifications, suggesting an adrenal carcinoma. Multiple left-sided retroperitoneal lymph nodes, up to 13 mm in size, were also found. The clinical differential diagnosis included adrenal neoplasm and retroperitoneal sarcoma. The patient underwent re found , Fig. 3.In order to plan the surgical approach and to determine the need for an en-bloc retroperitoneal resection including the left kidney, a preoperative biopsy of the lesion was performed that concluded to AON.After a multidisciplinary discussion, a laparoscopic left adrenalectomy (two 12 mm and one 5 mm trocars), with extraction of the tumor through a subcostal mini-laparotomy protected by a plastic bag (operative time: 141 min) was performed. Asymptomatic low plasmatic cortisol level was observed at post-operative day one, and a substitution was prescribed for 3 days. The patient was discharged home at day 4 after surgery. At two-weeks follow-up, he reported a slight dyspnoea. A dedicated CT-scan revealed distal pulmonar embolisms, despite post-operative antithrombotic prophylaxis. He was successfully treated with anticoagulation.Pathology examination showed a well delimitated and encapsulated 10 cm sized neoplasm weighting 325 g. On section, it was red-brown with a central haemorrhagic area A. Histol3The challenge is to determine the malignant nature or not of AONs to plan the type of surgical resection. These tumors are rare, usually benign and large tumors. They mainly occur in middle-aged women .AONs are functional in about 17% of cases, and, as for all cases of adrenal tumors management, hormonal status must be assessed .18F-FDG-PET-CT findings can guide, but not confirm, the malignancy status [Radiological findings are unspecific. The available literature suggests that a density of less than 10 HU (lipid-rich) on CT scan, without any necrosis or cyst, is more likely associated with benign lesions but the diagnosis and malignancy cannot be determined based on these characteristics ,14. Simiy status .Available literature on adrenal “incidentalomas” only recommend preoperative fine-needle aspiration in selected cases, as it may be associated with some complications risk 0–12%) and can have false negatives. In fact, biopsies should be performed in cases where surgical attitude could be affected by these results , or in presence of other known malignant lesions 2% and ca,17. ThesThe histological hallmark of AON is the presence of oncocytic cells, which were described in the adrenal glands by Kakimoto et al. in 1986 . The proper se a criterion of malignancy. For this reason, a revision of this classification was performed by Bisceglia et al. to produce an ad hoc scheme especially adapted for oncocytic adrenocortical tumors (Lin-Weiss-Bisceglia criteria) [The first classification scheme of adrenocortical neoplasms was proposed by Weiss . Since triteria) ,20. Majoriteria) ,6,12. Thriteria) .In the present case, the malignancy potential could not be determined after multiple imaging and percutaneous biopsy. We decided to perform a laparoscopic resection after multidisciplinary discussion, and final histology concluded to an AON of uncertain malignant potential. Follow-up did not reveal any recurrence.Concerning adrenal tumors, international guidelines recommend laparoscopic resection for benign lesions and pheochromocytomas. Many authors have shown interest in analysing superiority of laparoscopy over open approach for adrenocortical carcinoma, but literature is still conflicting. At this day, gold standard remains open resection, but laparoscopic resection should be proposed for selected cases in expert centers, depending principally on the tumor size (cut-off 10 cm without any adjacent organ invasion) ,22. TherThere is no consensus regarding the follow-up after resection and the necessity for an adjuvant treatment . Oncocyt4AONs are very uncommon tumors with a challenging diagnosis. Laparoscopic resection with clear margins is safe and feasible even in large tumors. There is no consensus in the literature about follow-up after resection and each case should be discussed in a multidisciplinary tumor board.The authors report no declarations of interest.There is no extra-founding for this article, for any author.In Switzerland a formal protocol for the EC is not mandatory when less than 5 patients are concerned.The patient was informed about the present article and submission and gave his written consent.Penelope St-Amour, Reza Djafarrian and Tobias Zingg wrote the manuscript and contributed together.Stefano La Rosa reviewed the pathology, produced the pictures of the pathology slides and wrote the corresponding part of the manuscript.Maurice Matter initiated the article participate to the writing and reviewed the article with Nicolas Demartines.1.http://www.researchregistry.com.Name of the registry: 2.Unique identifying number or registration ID: researchregistry5960.3.https://www.researchregistry.com/register-now#home/registrationdetails/5f4904752b51c00018055a1d/.Hyperlink to your specific registration (must be publicly accessible and will be checked): Matter M., MD.Not commissioned, externally peer-reviewed.


Hemorrhagic cerebrovascular disease in the pediatric population can have devastating and long-term effects. Progress in the fields of genetics, neuroimaging, pharmacology, and surgical techniques has led to improved diagnosis and management of pediatric cerebrovascular diseases. In this review we discuss the current etiologies and medical and surgical treatments of hemorrhagic cerebrovascular pathology affecting infants and children. A special emphasis is placed on neuroendovascular treatment options. Increased knowledge about this unique pathology and the medical and therapeutic options will empower practitioners to more quickly and accurately identify and accurately treat hemorrhagic diseases in the pediatric population. The incidences of non-traumatic subarachnoid hemorrhage (SAH) and intracerebral hemorrhage (ICH) in pediatric patients are 0.4/100,000/year and 0.8/100,000/year , 3]. In The presence of arteriovenous (AV) shunting through a nidus of coiled tortuous vascular connections that lack of capillaries and connect feeding arteries to draining veins are the hallmark of arteriovenous malformations (AVMs) . AVM incAVMs are usually diagnosed after ruptured with identification of a hematoma on a CT. MRI and MR/CT angiography are the imaging modalities used to characterize the precise location and the therapeutic planning. DSA remains the gold standard to define the AVM architecture and assessing high risk features such as aneurysm and or venous stenosis or ectasias . It is iAVMs have an annual hemorrhage rate that is thought to be between 2 and 4% with a mortality up to 25% per each event . This riArteriovenous fistulas (AVFs) are direct arterial to venous connections deprived of intervening capillaries. Unlike AVMs, AVFs do not contain nidus. Two groups of AVFs exist based on the location of the arterial-venous connection: pial and dural. AVFs can be acquired or congenital. The specific genetic patterns of these lesions are still not well-understood; however, numerous molecules have been implicated in the pathogenesis including vascular endothelial growth factor, sonic hedgehog, upstream-transcription factor II, notch chicken ovalbumin, and the ephrin family. Congenital pial AVFs have been linked to various congenital diseases, the commonest of which is hemorrhagic hereditary telangiectasia which co-presents with AVFs in one-quarter of patients . Recent AVFs can also be acquired. Sinus hypertension and sinus thrombosis have been implicated in the development of dual AVFs . Dural AThe estimated prevalence of pial AVFs ranges between 0.1/100,0000 and 1/100,000 –49 accouPrenatal diagnosis of AVFs usually occurs using fetal ultrasonography , 53. AVFMRI has limitations in the diagnosis of AVFs but can be used to evaluate for cerebral or spinal cord edema, dilated veins related to venous reflux, or ischemic infarct or atrophy. Catheter angiography is the gold standard diagnostic test where the presence of an AV shunting is demonstrated –58. SuspTreatment should be targeted at high flow symptomatic lesions , 53, 59.Endovascular techniques have become the first line method for treatment of pediatric AVFs , 59, 65.Microsurgical resection is usually reserved for cases when the risk of embolization of eloquent brain cortex is high , 42, 71.VOGM is a subtype of dural fistula that results from an abnormal regression of midline venous structures the medial prosencephalic vein of Markowski, which is the precursor of the vein of Galen , 73. ArtUltrasonography is used to assess VOGM flow in an ill neonate although a large procencephalic vein can be seen during the third trimesters of gestation by ultrasound. MRI of the brain can evaluate the brain for cerebral atrophy or ischemia that may be secondary to a CBF steal phenomenon. Cardiac function is assessed using echocardiography. Echocardiogram may show a dilated superior vena cava, dilated innominate vein, and right ventricular dilatation suggesting evidence of high cardiac preload. Furthermore, there can be diastolic runoff from the patent ductus arteriosus toward the brachiocephalic arteries by color Doppler.Endovascular treatment is the mainstay treatment for VGOM. Urgent endovascular treatment in the neonatal period is needed when medical management is not successful . The goaIn situations when hydrocephalus is not reversed post-embolization, it is treated with CSF diversion either via shunting or by endoscopic third ventriculostomy. In 2006 Lasjaunias et al. in a series of 233 patients treated with embolization reported the largest VGOM study . MortaliChildren compromise 0.5–4.6% of all unruptured, asymptomatic aneurysms. Ruptured aneurysms are more rare with 0.6% of all aneurysmal SAH comprising pediatric patients <19 years of age –81. ChilAneurysms appearance can be saccular  or fusiform . Mycotic aneurysms are a different type of aneurysm usually caused by infection (rarely tumor) and often appear similar to dissecting aneurysms on angiogram. Younger children (<5 years old) often present with dissecting fusiform aneurysm whereas older children have saccular aneurysms , 82, 85.Most aneurysms are asymptomatic and never found. Headache (80%) is the most common symptom. This is subsequently followed by loss of consciousness (25%), seizures (20%), focal neurological deficits (20%), and visual changes (10%) , 85, 87.The decision to treat an aneurysm is usually done with collaboration between open surgeons and neurointerventionalists. Endovascular therapy has transformed the treatment of pediatric aneurysms given its non-invasive nature when compared to open surgery. It is critical when possible to have cases reviewed by neurointerventionalists and open surgeons to come up with a balanced treatment strategy. In children lesions >3 mm in size usually warrant treatment. Mycotic aneurysms are initially treated with antibiotics and only intervened upon if they are persistent in follow up . Morbidide novo and be sporadic, though there is higher association of multiple lesions with familial CMs. CMs have preponderance in the Hispanic population. Specifically, in pediatric population, they have been associated with history of radiosurgery for CNS neoplasms. Approximately one-fourth of patients in multiple series were children  are a type of cerebral vascular malformation . They archildren . Seizurechildren . Initialchildren . Radiolochildren ]. CMs archildren , 128. Suchildren . Radiogrchildren , 131. Sechildren , 132. Rachildren . Almost The most common vascular malformations of the brain seen on routine imaging are developmental venous anomalies (DVAs). Fortunately, these lesions are often asymptomatic and incidentally found. DVAs are connected with parenchymal abnormalities and specific conditions like cerebral cavernous malformations. DVA angiographic architecture consists of a caput medusa and a medullary stem serving as a collecting vein. DVAs form the primary or only venous drainage for the area of the brain within their drainage territory. The caput medusa venous radicles are histologically defined by thin walled venous channels divided by normal brain parenchyma. The DVA collector is usually compromised of a thicker wall lacking a smooth muscle layer or elastica lamina , 128. OfDVAs are typically asymptomatic. In a prospective cohort of 80 patients, McLaughlin et al. found a 0.68%/year risk of hemorrhage in DVAs . This riDVAs are usually found on CT and MRI imaging. They are usually not seen on non-contrast CT. However, in large DVAs a tubular focus is visible. This tubular focus can be confused with intracranial hemorrhage given its attenuation is similar to cerebral blood vessels. On contrast-enhanced CT and MRI, the transparenchymal vein is the most distinct feature. In smaller DVAs, an ill-defined contrast blush can be seen related to the venous radicles. On MR imaging, the collecting vein may show up as a flow void on T2 imaging. There is variability in the T2 signal related to the venous radicles. This is dependent on venous flow and acquisition angle. Venous radicles can demonstrate a hyperintense signal on fluid-attenuated inversion recovery (FLAIR) imaging. Susceptibility weighted imaging (SWI) is a sensitive method of detecting DVAs. This is because of the deoxygenated venous blood within the DVA, On SWI, the venous radicles and larger collecting vein are usually hypointense/dark , 147.Horsch et al.  recentlyThe aim of this review is to provide insight into pediatric hemorrhagic cerebrovascular disease. This article provides a thorough review of the diagnosis and management of pediatric cerebrovascular hemorrhagic pathology. A special emphasis is placed on neuroendovascular treatment options and several clinical vignettes are presented. This is topic is not frequently addressed and discussed among clinicians. Thus, this review will increase knowledge about this pathology and will empower practitioners to care for this unique patient population.All authors listed have made a substantial, direct and intellectual contribution to the work, and approved it for publication.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Scrotal lymphedema is a rare condition, with significant psychological and functional disability. To date, association with giant congenital melanocytic nevus has not been reported.We report a case of a 15-year-old male with a giant congenital nevus associated with giant scrotal lymphedema. Surgical debulking with penoscrotoplasty achieved satisfactory functional and esthetic results.Early diagnosis and surgical intervention should be advocated for congenital causes of large scrotal swelling. Lymphedema is a chronic disease characterized by progressive deposition of protein-rich fluid in the subcutaneous tissue due to a variety of causes leading to impairment of the lymphatic drainage system. The causes vary between congenital and acquired conditions. It may affect any part of the body.A 15-year-old non-circumcised male patient presented with a history of dark skin discoloration and associated thickening since birth, affecting various parts of body but mainly the scrotum, perineum, both groins and buttocks. The parents reported progressive scrotal swelling when he was three years old. Over the years, the patient suffered from recurrent skin infections and ulcerations affecting the scrotal skin. There were no problems related to micturition. No history of other diseases or operations were documented. On initial presentation, the enlarged scrotum approximated the size of a basketball , impedinPhysical examination revealed multiple black hyperpigmented lesions all over the body extensively involving the external genitalia; groin, upper thigh, perineum and buttocks . The cirThe penile skin was lymphedematous and empty . ImmunocOur previously reported smile-like incisionHistopathology revealed a compound melanocytic nevus with deep tissue involvement, no cytological atypia and the cells were S-100 and HMB 45 positive.Genital pigmented lesions arise mainly on the vulva , although they may occur less often on the perineum, pubic region, and male genitalia (penis and scrotum).In a review of the literature, no similar scrotal lesions were reported. This case describes the occurrence of a congenital melanocytic nevus as a large scrotal mass. Biopsy remains the mainstay of diagnosis in cases of scrotal lesions along with the aid of immunohistochemistry.Debulking surgery with local excision and reconstruction of the external genitalia is the preferred treatment. These lesions have a low risk of local recurrence and no further adverse outcome as they have a low malignant potential. A major challenge is to reconstruct the covering of the penile shaft, for which a large number of surgical techniques and procedures have been reported.Giant penoscrotal lymphedema is a rare entity. Surgery is the most appropriate therapeutic option whatever the cause. Presentation of giant melanocytic nevus in association with giant scrotal swelling is very rare. Debulking of the diseased skin and subcutaneous tissue followed by penoscrotoplasty with coverage of distal penile shaft using prepucial skin gave an adequate and satisfactory cosmetic and functional outcome.None declared.


Dieulafoy's disease is a rare vascular lesion characterized by presence of large aberrant arteries within the submucosa of gastrointestinal tract or respiratory tract with a potential to cause life-threatening hemorrhage. Treatment includes bronchoscopy ablation, angiographic embolization or surgery. We report management of 7-year old girl with Dieulafoy's disease in the airway who presented with recurrent hemoptysis. Bronchial angiography revealed multiple feeding vessels to the lesion. Considering the potential risk of recurrence with embolization, sleeve resection of bronchus offered complete resolution. This case demonstrates the usefulness of bronchial angiography as part of multi-faceted approach before surgery in the management of Dieulafoy's disease. Dieulafoy's disease (DD) is a distinct pathologic condition characterized by presence of large aberrant arteries within the submucosa of the gastrointestinal tract . Most coOur patient was a 7-year old girl who presented with frequent episodes of unprovoked massive hemoptysis for 5 months. She was referred to us after a bronchoscopic finding of a polypoid lesion in her bronchus intermedius (RBI). On admission to our hospital, she appeared well but continued to have small-volume hemoptysis. Physical examination was essentially normal, especially with no cutaneous telangiectasia.Computer tomography angiography (CTA) and a repeat bronchoscopy revealed an abnormal vascular lesion in her RBI . To furtHistopathology revealed increased number of thick-walled vessels with prominent intimal thickening within submucosa and peri-bronchial connective tissue of the resected specimen. Places of erosion of the respiratory epithelium close to these thick-walled vessels could be found. Patchy chronic inflammation in the submucosa and patchy hypertrophy of the sub-epithelial connective tissue were also present . CurrentDieulafoy's disease of the bronchus was first described in 1995 by Sweerts et al.  and to dIts pathogenesis is hypothesized to be a hypertrophic response to chronic inflammation in a background of a possible congenital malformation. Patients in reported series are mostly middle-aged individuals with a male preponderance and with a history of smoking . PediatrRecurrent massive hemoptysis was the most common cause of hospitalization of those children described in the literature. Bronchoscopy in all these patients showed polypoid and non-pulsatile lesions in their airways. A white cap was described in one patient. The vascular lesions were able to be identified by CTA in three cases. It was non-diagnostic in the fourth patient, in whom an endobronchial ultrasound revealed the underlying lesion.n = 2) underwent bronchial artery embolization (BAE) with initial success in one of them (50%). Lobectomy was performed in one patient after failure of BAE (50%). Recurrence after initial treatment was reported in two patients  of which one underwent lobectomy and the other, continued with conservative management. Histological examination of all those published cases showed dilated tortuous branches of bronchial artery in the submucosa of the respiratory tract in association with mucosal erosion.One patient underwent upfront segmentectomy due to lack of expertise in interventional radiology in their facility. Rest of the patients  can be more appropriate in selected scenarios where definitive treatment with lower risk of recurrence is preferred.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation, to any qualified researcher.Written informed consent was obtained for the publication of this case report from the parents of the child treated for such condition while in hospital and they are fully aware of the images as well as the report sent across to the journal.Y-TY and MR prepared the initial draft. SC, DM, and NMc worked on editing the pictures. JS, J-NR, SS, and SC contributed to interventional radiology component. NMu edited the final version and approved for submission.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Hepatocellular carcinoma with biliary ductal invasion is rare and associated with a significantly lower survival rate.We present an unusual case of a patient with hepatocellular carcinoma and biliary invasion, who had his diagnosis confirmed by histological analysis from tissue extracted by endoscopic retrograde cholangiopancreatography. An 87-year-old male presented with a 1-day history of right upper quadrant pain and jaundice. His past medical history included recurrent gallstone cholangitis and a previous cholecystectomy. An abdominal CT demonstrated a dilated intrahepatic biliary tree with left proximal intrahepatic hyperdensities, as well as a 3 cm hepatocellular carcinoma. He was initially suspected to have concurrent gallstone cholangitis and a newly diagnosed hepatocellular carcinoma. Endoscopic retrograde cholangiopancreatography and balloon trawling of the intraductal lesions extracted necrotic tumour-like tissue which was histologically consistent with hepatocellular carcinoma. The extraction of the intra-biliary portion of HCC resulted in complete resolution of his jaundice, enabling further treatment with nivolumab, which would not have been possible if the obstruction was not cleared. The patient is currently well and has completed his 6th cycle of nivolumab.Obstructive jaundice is an uncommon presentation for patients with HCC. it is key for clinicians to be aware of the possibility of intrabiliary invasion in order obtain an early diagnosis and to reduce any delay in treatment. Hepatocellular carcinoma (HCC) is the most common primary malignant hepatic neoplasm. However, it only rarely shows biliary ductal invasion, with a reported incidence of 1–9% . We discAn 87-year-old Chinese male presented with right upper quadrant pain and obstructive jaundice for one day. He had no fever, nausea, or vomiting. He had no history of alcohol use. His medical history was significant for hyperlipidemia, diabetes mellitus, recurrent gallstone cholangitis, and a previous cholecystectomy 20 years ago. Of note, he had presented with gallstone cholangitis 4 months prior, during which an ERCP had been performed with successful balloon trawling of a 14 mm gallstone from the distal common bile duct. Physical examination revealed scleral icterus and mild right hypochondrial tenderness. His laboratory workup was notable for raised total bilirubin of 81 μmol/L, a low serum albumin of 33 g/L, thrombocytopenia, and an elevated serum alphafetoprotein level of 611μg/L. He had no leukocytosis, and his INR was normal.A computed tomography multiphasic scan of his abdomen showed a dilated intra- and extra-hepatic biliary tree with thickening and enhancement of the ductal wall as well as hyperdensities within the proximal left hepatic duct and common hepatic duct suggestive of choledocholithiasis. It also showed a 3 cm segment 3 lesion with faint arterial enhancement and subsequent washout on the delayed phase suggestive of HCC Fig. . Given hAn ERCP was performed which demonstrated a dilated left intrahepatic duct with amorphous filling defects obstructing its distal end Fig. . BalloonAfter discussion at a multidisciplinary tumour board meeting, the options of surgical resection and systemic therapy were both offered to the patient. However, the patient opted for systemic therapy in view of his age and personal preferences, understanding that this was a palliative option. The extraction of the intra-biliary portion of HCC resulted in complete resolution of his jaundice, allowing initiation of systemic therapy. Nivolumab was eventually started after discussion with the patient and his family. A repeat computed tomography scan after completing six cycles of nivolumab showed partial response, and the patient remains well and free of jaundice.HCC only rarely leads to bile duct invasion, with a reported incidence of 1% .Despite advancements in hepatobiliary imaging, the diagnosis of an intrabiliary HCC remains challenging, and is often confused with other causes of intrabiliary filling defects, such as choledocholithiasis, biliary cystadenomas and cholangiocarcinomas . IntrabiObstructive jaundice is typically a late clinical manifestation of intrabiliary HCC. Prognosis is generally poor, and is closely related to the stage of disease, location of tumour and degree of tumour extension. The mean survival rate ranges from 2 to 4.5 months amongst several studies , 6, 7. CWe initially suspected choledocholithiasis as the cause of the obstructive jaundice in this case, given his significant history of recurrent gallstone cholangitis with a recent episode prior to presentation. However, the diagnosis of HCC was subsequently clinched when the tumour tissue was trawled out during an ERCP. To our knowledge, there has only been one other case report in the literature where HCC was also diagnosed from tissue removed during an ERCP . In caseIn conclusion, the diagnosis of intrabiliary invasion of HCC is rare, but needs to be considered when intraductal filling defects are noted on imaging, especially in the context of underlying liver disease and cirrhosis.


Any cystic lesion occurring in the mesentery which may or may not extend into the retroperitoneum is referred to as a mesenteric cyst; they have an infrequent incidence rate in the pediatric age group. Definitive etiology of the cystic lymphatic malformations is still not known but there are multiple hypotheses. A young male child presented with acute onset abdominal pain and palpable intra-abdominal mass and ultrasonography revealed presence of two lesions, one of them as an encysted turbid fluid collection in the right lumbar region and the other as a dilated, tortuous, intercalated structure. On CT, the first one was identified definitively as a mesenteric cyst while the other as a possible neoplastic mass in close proximity to the first one. Histopathology confirmed the diagnosis as a cystic lymphatic malformation of the mesenteric cyst. The limited awareness of its existence along with its usually asymptomatic nature, are the likely reasons that it still remains an elusive diagnosis. Based on our case we discuss, the use of a multi-modality approach towards diagnosing cystic malformation disorders and how the use of MRI is under-utilised when it could prove decisive. Any cystic lesion occurring in the mesentery which may or may not extend into the retoperitoneum is referred to as a mesenteric cyst. Reported cases in the literature of mesenteric cysts are far and few with a few reports stating their incidence between 1/10000 to 1/25000 in the pediatric age group and around 1/105000-250000 hospitalized adult surgical patients . DefinitWe report such a case where the likelihood of mesenteric cyst was kept foremost but the appearance was mimicking in places that of a malignancy. The limited awareness of its existence is one of the reasons that it still remains an elusive diagnosis with most cases being diagnosed incidentally. Herein, we have utilised this case to describe how the usage of a multi-modality approach involving ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI) is highly effective in suspecting and confirming the diagnosis, and why MRI, if possible, must be carried out in all such cases .An eight-year-old male child presented to the paediatric department complaining of diffuse abdominal pain and abdominal distension since 15 days. On examination, there was a soft, palpable lump of size 5 x 4cm in the right lumbar and right iliac fossa region. He did not have any complaints associated with the passage of stools or episodes of vomiting. The child had an uncomplicated birth history and was developing normally for age; there was no family history of any such previous presentations. The referring doctor advised a US of abdomen and pelvis to establish the aetiology of the mass.An ultrasonographic evaluation revealed the presence of an encysted turbid fluid collection of size 71x81x56mm (161 cubic centimetres in volume)  x 80mm (anteroposterior) x 53mm (transverse) in the right lumbar and iliac region, between medial wall of the caecum and ascending colon laterally, and terminal ileum medially just beneath the anterior abdominal wall  of 1.01.The patient was planned for an exploratory laparotomy with the option for resection and anastomosis if needed. During laparotomy, the patient was found to have multiple, thin-walled, yellow coloured, translucent, encysted collections, adjacent to each other. Few enlarged paracolic lymph nodes were seen. Since these could not be removed, resection and anastomosis of the adjacent intestine were carried out Figures -6 AppropThe patient became asymptomatic post the operation and had stable vitals. Both the one-month and six-month follow-up by clinical examination revealed a healthy, asymptomatic child.First diagnosed by the anatomist Benevieni in 1507 during an autopsy, mesenteric cysts have been reported sporadically in literature. Since those times, a variety of mesenteric cysts have been documented, all of whom have similar pathogenesis but are varied based on their histo-pathological origin and structure. Frequently, they turn out to be an ectopic lymphatic tissue- lymphatic, chylous cysts.One very commonly cited clinical classification groups mesenteric cysts under six broad categories, which include cysts of mesothelial origin, lymphatic origin, enteric origin, urogenital origin, mature cystic teratomas and non-pancreatic cysts . The cysThe cystic spaces have a lining of endothelium and the presence of lymphoid aggregated in the cyst wall. CLM usually contains serous, serosanguineous, or chylous fluid. The milky appearance of chylous fluid is due to abundant content of fat; variation in the appearance of the content of fluid is probably due to difference in degrees of lymph stasis and a variation in the number of communicating channels within the lymphatic system . The sizTogether US and CT prove very effective in making the diagnosis . In the While both US and CT were performed in the case, an MRI evaluation of abdomen could not be performed due to cost prohibitions; if indeed it had been, it would have been a very helpful adjunct. Gadolinium-enhanced MRI is useful in differentiating micro-cystic from macrocystic malformations prior to surgery, as lesions of small size and infiltrative nature are much better appreciated on contrast administration. MRI could have helped identify the contents as chylous or blood products. With chylous products, the MRI would have revealed the signal intensity drops out on fat-saturated images and chemical shift artefacts on opposed phase images -11.Once diagnosis is certain then the management should preferably be surgical to avoid various future complications. Most commonly resected by surgical enucleation which also happens to be the preferred method of choice ,9,10, buTo conclude, each modality adds on a layer of information towards our final diagnosis of mixed cystic lymphatic malformation; while the US allows for closer and dynamic visualisation of the cyst wall, CT allows us a better delineation of its extent and MRI as a final step allows us to comment upon the cyst contents, thus making ‘radiological biopsy’ possible that is the radiological description and diagnosis approaches such high levels of accuracy that it anticipates in advance the histopathological findings including a comment on the content of such lesions. It is thus advisable to systematically perform these modalities and give the treating surgeon an accurate diagnosis and description of what to expect and not leave them in the blind.


Sclerosing mesenteritis is a rare and often benign condition characterized as a fibrotic disease consisting of non-suppurative inflammation of adipose tissue. Through mass effect, sclerosing mesenteritis can compromise the gastrointestinal lumen as well as mesenteric vessel integrity. There is a poor understanding of this disorder and its pathogenesis, which presents with various symptomatology and often without identification of inciting factors. Patients with sclerosing mesenteritis exhibit gastrointestinal and systemic manifestations including weight loss, fever, nausea, vomiting, diarrhea, and abdominal pain. This case presents a patient with a seven-month history of chronic, epigastric abdominal pain following laparoscopic surgery for acute uncomplicated appendicitis. The patient underwent work-up with computed tomography and magnetic resonance enterography that confirmed the presence of a mesenteric mass of unknown etiology located in the mid-epigastrium. Due to the inability to safely sample the mass, the patient underwent diagnostic laparoscopy, which was subsequently converted to an open procedure where excision of the mesenteric lesion was performed. Surgical pathology revealed fat necrosis with fibrosis, granulomatous inflammation, and dystrophic calcifications consistent with sclerosing mesenteritis. The patient was seen in follow-up with the resolution of her epigastric abdominal pain. This case report demonstrates a unique presentation of a symptomatic patient with a mesenteric mass not amenable to non-invasive biopsy. Complete excision of this lesser sac mass revealed sclerosis mesenteritis as the pathological cause. Sclerosing mesenteritis is a rare and often benign condition characterized as a fibrotic disease consisting of non-suppurative inflammation of adipose tissue ,2. ThrouThis disease process correlates to a range of idiopathic chronic inflammatory diseases that occur within the intestinal mesentery causing significant fibrotic reactions . A varieThis case was diagnosed as a result of clinical examination and imaging studies and then managed by the department of general surgery at a community-based surgical residency program. The case is unique as the imaging characteristics were more consistent with a mass of unknown origin and there was an inability to sample the lesion without operative intervention. Given the non-specific presentation of this condition, it is crucial to rule out other pathologies that can range from granulomatous and infectious diseases to benign or malignant masses . This caA 55-year-old Caucasian female presented to the surgical office with a chief complaint of chronic epigastric abdominal pain for seven months duration. The patient was referred to the office after an emergency department visit for her chronic abdominal pain. The workup showed normal laboratory values with a nonspecific, lobulated soft tissue lesion located within the mesentery of the upper abdomen measuring up to 3.2 cm on computed tomography with intravenous and oral contrast. The patient noted her pain had developed and had been persistent since she underwent laparoscopic appendectomy for acute appendicitis roughly one year prior. She described her pain as sharp in nature with intermittent flares. The patient reported that eating exacerbated her symptoms. She endorsed anorexia as well as weight loss. Physical examination at that time was considered unremarkable. The patient’s medical history included gastroesophageal reflux disease, gastritis, depression, and a seizure disorder. The patient’s surgical history included a cholecystectomy for cholelithiasis, hemorrhoidectomy, tonsillectomy, and an appendectomy. Family and social history were non-contributory.Based on the initial radiographic results and the location of the mesenteric lesion, the patient was referred for endoscopic evaluation and possible biopsy. They were then started on a trial of proton pump inhibitors. The patient had multiple visits to her primary care provider and was eventually transitioned to sucralfate as her symptoms were unresolved. The patient underwent esophagogastroduodenoscopy that revealed LA Grade B gastroesophageal reflux disease, a 2 cm hiatal hernia, and diffuse gastritis. Repeat computed tomography of the abdomen and pelvis with intravenous and oral contrast was performed revealing a lobulated lesion at the level of the kidneys within the anterior mesenteric fat, concerning for an enlarged lymph node or a neoplastic mass as demonstrated by the arrow in Figure The patient returned to the clinic for a follow-up examination. After an informed conversation regarding the possible etiologies of the mass and medical and surgical options, the decision was made to further characterize the soft tissue mass in preparation for surgery. Magnetic resonance enterography was performed which redemonstrated the previously reported mid-abdominal mesenteric mass located posterior-inferior from the gastric antrum, with etiology remaining unclear. In addition, there was a continued concern for an enlarged lymph node versus an underlying neoplastic process as seen in Figures The patient was consented for and underwent diagnostic laparoscopic exploration. Upon entering the abdomen, the omentum, mesentery, and peritoneum were without signs of gross disease. Takedown of the gastrocolic ligament and omentum was performed and upon entering the lesser sac, there was a firm, mobile mass located inferior to the greater curvature of the stomach. Careful dissection was attempted via laparoscopic approach, but due to the dense nature of the mesentery and inability to properly characterize the location of the mass about the surrounding structures, the decision was made to convert to an open procedure. The mass was found to lay within the mesentery and was free of attachments to the underlying vasculature and pancreas. Careful sharp excision was performed to circumferentially inscribe the mass as demonstrated in Figure The postoperative period was uneventful and the patient remained stable throughout its entirety. The patient was discharged without complication on postoperative day two. The patient was seen in the office for follow-up at one week and at one-month post-operatively in which she endorsed the slow resolution of her previous symptoms. The authors can not say with certainty that her symptoms improved secondary to surgical intervention. Histological evaluation of the surgical specimens revealed adipose tissue with a nodular collection of inflammatory cells composed predominantly of histiocytes with interspersed lymphocytes. The histiocytes contained foamy cytoplasm and surrounding areas of confluent fat necrosis with associated cholesterol clefts. Granular basophilic particles representing dystrophic calcification were dispersed throughout the areas of fat necrosis. Examination by polarized light microscopy did not show foreign material. Special stains for infectious organisms were negative. Histological examination of the surgical specimen is seen in Figures Sclerosing mesenteritis was first described in 1924 as a retractile mesenteritis . Since tThe etiology of this disease process is unknown in many cases. There have been several theories proposed, that span from ischemia, trauma, or secondary to a neoplastic process, most commonly non-Hodgkin's lymphoma. In addition, it often presents in three different forms: type I presenting with diffuse mesenteric thickening (42%), type II presenting with a single discrete mass (32%), and type III with multiple discrete masses (26%) .The treatment for sclerosing mesenteritis is often observed as the disease is most commonly self-limiting. If the disease is severe or has a protracted course, first-line therapy is a combination of prednisone and tamoxifen. Other multifactorial treatments have been proposed, however, most have limited power. Current prospective trials testing the efficacy of thalidomide are underway. Other case reports present success with anti-inflammatory medications .Most patients with sclerosing mesenteritis present with non-specific abdominal pain. Oftentimes, a diagnosis of sclerosing mesenteritis can be achieved through imaging alone as it has relatively specific findings of well-demarcated mesenteric mass-like lesions with hazy surrounding soft-tissue attenuation on computed tomography. When imaging characteristics are not diagnostic and the clinical picture is discordant, sampling of the tissue is recommended . In a reThe diagnostic approach for the case presented was utilized as a step-up fashion to surgery. In exploring the peritoneum via laparoscopy and subsequent laparoscopic dissection into the lesser sac, concern for metastatic involvement was reduced. Complete resection was performed once the mass was noted to be free from surrounding structures in an attempt to remove pathological tissue and increase surgical yield. An alternative approach to consider could have been laparoscopic core-needle biopsy. Excisional biopsy was favored, however, given the extent of mesenteric involvement, relation to surrounding vasculature, and proximity to vital organs. Sclerosing mesenteritis is a benign and rare condition characterized by non-suppurative inflammation of adipose tissue. As a spectrum of disease processes, its presentation, and the pathological impact can vary. This case presents a patient with a mesenteric mass in the setting of a seven-month history of chronic epigastric abdominal pain following laparoscopic surgery for acute uncomplicated appendicitis. After a non-diagnostic work-up and inability to obtain a tissue sample safely, the patient underwent diagnostic laparoscopy with conversion to open and subsequent complete excision. Surgical pathology demonstrated fat necrosis with fibrosis, granulomatous inflammation, and dystrophic calcifications without evidence of atypia or malignancy. This case demonstrates a unique approach to an unknown mesenteric mass found to be consistent with sclerosing mesenteritis.


Giant cerebral tuberculoma is an uncommon but serious form of tuberculosis. We report two patients who had a single, large lesion on magnetic resonance imaging (MRI) of the brain. Both patients underwent neurosurgery for the excision of the mass lesion as neuroimaging findings were suggestive of a brain tumor. Tuberculoma was later diagnosed on histopathological examination. We want to highlight that cerebral tuberculomas can mimic malignant brain tumors, as the clinical, laboratory, and radiologic features of cerebral tuberculomas are nonspecific.  Tuberculosis of the central nervous system is less common compared with other organ systems . CentralCase 13 in the right temporal lobe, with significant perilesional vasogenic edema  score was 15. There were no signs of meningism or focal neurological deficits. In laboratory tests, the results of the erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and other routine biochemical tests were normal. The chest X-ray was unremarkable. Brain MRI revealed a peripheral enhancing solitary space-occupying lesion measuring 4 x 3.9 x 3.7 cmMR spectroscopy showed an increase in choline peak with a decrease in the N-acetylaspartate peak. The choline/N-acetylaspartate and choline/creatine ratio were high, suggestive of high-grade glioma. Considering her age, the initial radiological differential diagnoses included cystic glioblastoma and intracranial metastasis. The work-up for metastasis, including contrast-enhanced computed tomography scans of the chest and abdomen, was non-contributory. Because of the clinical presentation, the mass effect caused by the lesion, and uncertain clinical diagnosis, the patient underwent a right frontotemporoparietal craniotomy for the excision of the mass on the third day of admission. The mass was firm, grayish, avascular, and partly adherent to the dura with well-demarcated tissue planes. After complete excision, the specimen was sent for histopathological and microbiological examinations. The histopathological examination found a necrotizing granulomatous inflammatory lesion, consistent with the diagnosis of tuberculoma. Anti-tuberculosis treatment (ATT) with isoniazid, rifampicin, pyrazinamide, and ethambutol was started. ATT was continued for 18 months, leading to complete clinical recovery.Case 23 in the left cerebellar hemisphere with significant perilesional edema (Figure A 26-year-old man presented to the hospital with a one-month history of progressive intermittent headache, which was associated with dizziness and vomiting. He also had swaying of the body to the left while walking. There was no history of fever or seizure. The patient was taking ibuprofen but was not on other drugs. On examination, the GCS score was 15. There were cerebellar ataxia and right-sided dysdiadochokinesia. There were no signs of meningism. The patient’s ESR, CRP, and other biochemical tests were normal. A plain chest X-ray was unremarkable. MRI of the brain revealed a peripheral-enhancing solitary space-occupying lesion measuring 3.5 x 3.1 x 2.7 cmThe provisional diagnosis of a posterior fossa tumor was made. Considering the progressive nature of his symptoms with the presence of cerebellar signs, a suboccipital craniotomy and excision of the mass was done. The excised mass was pale with solid consistency. The histopathologic examination found a granulomatous inflammatory lesion consistent with the diagnosis of a tuberculoma. ATT was started and continued for 18 months with complete clinical and radiologic recovery.Cerebral tuberculoma is a dreaded presentation of tuberculosis that results from hematogenous spread of a distant tuberculous focus. Cerebral tuberculomas may be solitary or multiple and are most commonly seen in the basal parts of the brain . The typThe neuroimaging picture for cerebral tuberculoma is different according to the type of granulomatous lesion. If the tuberculoma comprises granulomas that are non-caseating, the lesion is usually hypointense or isointense to gray matter on T1-weighted images, and hyperintense on T2-weighted MR-images. Caseating granulomas typically have a solid center that is hypointense or isointense on T1-weighted images and isointense on T2-weighted imaging. A central region of T2-hypointensity can be seen because of gliosis and monocyte infiltration. This is a useful finding, as we do not find it in many other intracranial lesions. In post-contrast imaging of tuberculoma, we typically see a peripheral ring-enhancement because of vasogenic edema. Sometimes, central liquefactive necrosis can occur in tuberculoma, which can make differentiation from a cerebral abscess difficult. Some reports have described the ‘target sign,’ which is a ring-enhancing lesion with an additional central area of enhancement or calcification, as characteristic of cerebral tuberculomas . HoweverCerebral tuberculomas should always be considered in the differential diagnosis of solitary intracranial mass lesions. However, the diagnosis is difficult because the neuro-imaging presentation is varied and can be non-specific. In many cases, a definitive diagnosis can only be established with a biopsy of the CNS lesion for histopathology and acid-fast bacilli stain and culture. Antitubercular drugs are usually effective in treating cerebral tuberculomas. A long course of chemotherapy for nine to 18 months is typically required, but total cure rates are high . SurgicaIn regions where tuberculosis is endemic or in patients with a history of prior tuberculosis exposure or infection, cerebral tuberculomas should always be considered in the differential diagnosis of single and multiple space-occupying brain lesions. However, because of its rarity, a giant cerebral tuberculoma can easily be misdiagnosed as an intracranial tumor such as a high-grade glioma. A timely and accurate diagnosis allows the early administration of anti-tubercular drugs, decreases patient morbidity, and can potentially prevent neurosurgical excisions that are not required in all patients.


Although preoperative bradycardia has been reported in several pheochromocytoma cases, postoperative bradycardia has not. This is the first case report of complete atrioventricular dissociation and sinus arrest occurring after pheochromocytoma resection.A 38‐year‐old woman was referred for a left adrenal incidentaloma. Twenty‐four hour urinary collection showed elevated noradrenaline. Iodine‐123‐meta‐iodobenzylguanidine scintigraphy showed high tracer uptake in the left adrenal region. Open left adrenalectomy was performed, and histopathological examination confirmed the diagnosis of pheochromocytoma. Thirty minutes following surgery, complete atrioventricular dissociation and sinus arrest developed. Vagal reflex attenuation due to decreased noradrenaline after tumor removal and perioperative pain and fear were believed to be the causes. A temporary pacemaker was implanted to prevent sudden death due to vagal overstimulation.Vagal reflex attenuation after pheochromocytoma resection can result in complete atrioventricular dissociation and sinus arrest. Adequate preoperative preparation and close monitoring during and after surgery are imperative. We report the case of a 38‐year‐old woman with complete atrioventricular dissociation and sinus arrest occurring after pheochromocytoma resection. Vagal reflex attenuation due to decreased noradrenaline after tumor removal and perioperative pain and fear were believed to be the causes. Adequate preoperative preparation and close monitoring during and after surgery are imperative.Pheochromocytomas are catecholamine‐producing neuroendocrine tumors arising from chromaffin cells of the adrenal medulla or extra‐adrenal paraganglia.A 38‐year‐old woman was referred to our department for a large left adrenal incidentaloma. She had a history of intractable headache and recent left abdominal pain and swelling. The family history revealed that her aunt was diagnosed with gastric cancer. Physical examination showed blood pressure 147/98 mmHg, height 166 cm, weight 57 kg, and body mass index 20.6. Electrocardiography and chest X‐ray findings were normal. Routine laboratory findings were within the physiologic range.High‐performance liquid chromatography on 24‐h urinary collections showed elevated noradrenaline , normetanephrine , and vanillylmandelic acid . An abdominal magnetic resonance imaging revealed a large 11 × 8.5‐cm left adrenal tumor ;Although vagally mediated death is rare, some deaths can be attributed to vagal overstimulation.Some limitations of this case report are worth noting. First, we were unable to validate the effectiveness of atropine and ephedrine, which have been reported to be effective for vagally mediated bradycardia and sinus arrest.Nevertheless, adequate preoperative preparation with alpha‐adrenergic blockers and increased salt and fluid intake to prevent postoperative reduction in cardiac venous return and consequently vagal activation, and close monitoring during and after surgery, are imperative.To the best of our knowledge, this is the first case report of complete atrioventricular dissociation and sinus arrest occurring after pheochromocytoma resection. Vagal reflex attenuation due to decreased noradrenaline after tumor removal and perioperative pain and fear were believed to be the causes. Adequate preoperative preparation to prevent postoperative reduction in cardiac venous return and close monitoring during and after surgery are imperative.The authors declare no conflict of interest.


Various congenital anomalies of the female tract such as agenesis, vertical or lateral fusion failure, and canalization failure occur when the normal development of the Müllerian duct disrupts in any stage of developmental milestones. A cavitated non-communicating rudimentary horn is reported in about 20%-25% of women with unicornuate uterus. A 36-year-old patient, gravida 2 para 2, was admitted to the hospital with a complaint of worsening lower abdominal pain occurring on each menses for 8 months. A 6-cm accessory cavitated left uterine mass suggestive of hematometra was shown on ultrasound examination. It was decided to perform hemi-hysterectomy to remove the left uterine horn by the laparoscopic route. Here we aimed to demonstrate the laparoscopic management of a rudimentary horn case and emphasize the crucial steps that surgeons should safely perform during the operation. Various congenital anomalies of the female tract such as agenesis, vertical or lateral fusion failure, and canalization failure occur when the normal development of the Müllerian duct disrupts any stage of the developmental milestones. The unicornuate uterus is caused by the normal maturation of only one Müllerian duct. In some cases, the contralateral Müllerian duct is absent or partially develops, called a rudimentary horn, which may or may not communicate with the normally developed one Müllerian duct, called a unicornuate uterus.,2. The symptoms differ with the functionality of the endometrial cavity, and the patients’ symptoms depend on the presence of an obstructive anomaly causing pain regarding hematometra, hematosalpinx, or endometriosis due to retrograde menstruation. There have been studies reporting reproductive outcome improvement by removing the rudimentary horn, but information on such approach is still lacking.A cavitated non-communicating rudimentary horn is reported in about 20%-25% of women with a unicornuate uterusIn the present case, we aimed to discuss the laparoscopic management of a multiparous patient with a rudimentary horn presenting with dysmenorrhea and dyspareunia symptoms in her late 30s. by the laparoscopic route. Evaluation of the abdominal cavity revealed a left non-communicating rudimentary horn tightly residing on the lateral abdominal wall and two grossly normal ovaries and tubes (A 36-year-old patient, gravida 2 para 2, was admitted to the hospital with a complaint of worsening lower abdominal pain occurring on each menses for 8 months. Her external and internal genitalia, including the cervix, were normal except for the 6 cm accessory cavitated left uterine mass suggestive of hematometra that is compressing the urinary bladder without any other genitourinary system pathologies shown on ultrasound examination . Diagnosnd tubes . A proba. After controlling the bleeding and irrigating and suctioning the abdominal cavity, no other hemostasis sutures were required, and the operation was completed successfully. The patient was discharged on the first postoperative day, and normal regular menstrual cycles without any pain and complaints during the 6 and 12 months after the surgery were noted.First, to remove the fallopian tubes from the left uterine horn, they were coagulated and divided by careful tissue transection. Second, the vesicouterine peritoneum was divided to create the bladder flap from the cervix and the left uterine horn. Third, dissection of the retroperitoneal space beneath the round ligament to identify the ureter and the left hypogastric artery branches was performed. The broad ligament was fenestrated to lateralize the left ureter and facilitate transection of the utero-ovarian pedicle. The retroperitoneum was dissected, and the ureter tract was followed. Posterior peritoneum was also opened to create distance from the ureter and provide a place for the division of the horn by a monopolar hook. After the dissection and coagulation of the left uterine artery at the origin of the left hypogastric artery to minimize the bleeding during excision of the uterine horn by an advanced bipolar energy device , the resThe patient signed an informed consent that allowed us to use her data., laparoscopy has become the standard treatment with proven advantages, including short operative time and hospital admission duration and less blood loss and postoperative pain. Although laparoscopic excision of the rudimentary uterine horn seems to be an effective and feasible surgical approach in experienced hands, it should always be remembered that anatomical landmarks and retroperitoneal space must be defined as the cleavage planes of the uterine horn and that the unicornuate uterus is not well defined all the time.Since the first documentation of laparoscopic removal of the rudimentary horn in 1990 by Canis et al.. Here, the rudimentary horn was attached firmly to the right unicornuate uterus and the left sidewall. When the borders are firm and not easy to distinguish, it can be difficult to remove the horn. To minimize the risk of penetration into the cavity of the hemiuterus while laparoscopic dissection, a hysteroscopic transillumination technique was performed in three cases by Nezhat et al.. In a recent study by Jan et al., this technique was detailed in a technical video demonstrating the hysteroscopic transillumination of the plane of the dissection between the rudimentary horn and the uterus. At the beginning of the operation, we first performed a hysteroscopic evaluation of the uterine cavity to visualize the relation.Two anatomical variations in the attachment of the rudimentary horn to the unicornuate uterus were reported, and one can be attached by either a band of tissue or firmly to the latter. When no fusion occurs with the contralateral duct, a fibrous or fibromuscular band connects the two horns. Anomaly observation of these coexisting defects should be performed, and the right treatment should be planned after defining the anatomy of pathology as possible with proper imaging techniques such as an ultrasound scan and magnetic resonance imaging. Regarding the definition of the anatomy of the pathology, type of attachment, and communication between the rudimentary horn and the hemiuterus considering other probable pathology exclusions like myomas or an obstructed hemivagina, the right treatment option should be discussed and performed.Besides, the most important point about these anomalies is the preoperative evaluation of the patients because other probable concomitant female reproductive tract anomalies and renal and skeletal system abnormalities may co-occur with Müllerian anomaliesAfter defining the anatomy of pathology, type of attachment, and communication between the rudimentary horn and the hemiuterus, the right treatment option should be discussed and performed. Here, preoperative examinations including transvaginal ultrasonography and an intravenous pyelogram showing the normal kidneys and ureters were performed.. Therefore, the ligation of the major blood supply from the uterine artery at the isthmus level may be impossible to achieve, so the dissection of the retroperitoneal space to develop a plane for the ipsilateral ureter lying adjacent to the vascular supply of the uterine horn is of great importance to prevent injury of the uterine artery during coagulation and ligation at the level of the hypogastric origin. A monopolar hook may not be sufficient while resecting the rudimentary horn; thus, advanced bipolar energy devices are needed to control the bleeding as the rudimentary horn may receive blood from the myometrial arcuate arteries of the contralateral uterine artery. In cases where a firmly attached horn is present, the laparoscopic surgeon should handle large myometrial defect with sutures for reconstruction after removal of the horn. Moreover, if the patient has a desire for a future pregnancy, the myometrial defect should also be sutured to avoid probable uterine rupture. The removal of the ipsilateral fallopian tube should always be performed to prevent a tubal pregnancy and cancer development.Another step of the operation depends on the blood supply of the rudimentary horn as it may not always be from the ipsilateral uterine artery but also from the contralateral uterine arteryIn conclusion, a careful preoperative examination should be performed to detect the anatomical subtype, attachment type, and other coexisting genital malformations. Although the laparoscopic approach in these abnormalities seems to be effective and feasible, it should always be remembered that the anatomical landmarks and retroperitoneal space must be defined, and careful hemostasis must be performed in every step of the operation.


Benign epithelial tumors of the lung are uncommon and can represent a diagnostic challenge. Herein, we describe one such emblematic case. A 59-year-old former smoker male was admitted to the hospital complaining of cough for a long time. A radiological examination showed a centrally excavated mass strictly connected to the visceral pleura. The patient underwent tumorectomy. At gross examination, the tumor was composed of solid and cystic areas containing clear liquid. Histological examination highlighted a sub-pleural encapsulated tumor, with foci of capsular invasion, characterized by a single layer of columnar and cuboidal epithelial cells lining moderately cellular fibro-vascular cores. A wide spectrum of immunohistochemical markers was performed. The final diagnosis was suggestive of a peripheral pulmonary papillary tumor of undetermined malignant potential. At the last follow-up, six years after surgery, no recurrence or metastases were described. Reporting this case, we would like to point out the existence of these rare entities that should be taken into account in the diagnostic process, thus avoiding potential misdiagnosis. Moreover, the presence of capsular invasion should be better investigated in order to reconsider the exact terminology of the tumor and the classification of its malignant potential.  Pulmonary papillary adenoma is a rare neoplasm, predominantly occurring in peripheral lung areas, usually asymptomatic, that consists of cuboidal to columnar cells without nuclear pleomorphism and absent or low proliferative index. It mainly affects the male population, with an age range from 2 months to 70 years . Pulmona


Isolated duodenal perforation following blunt abdominal trauma is a rare injury in children. Bicycle accidents  are a frequent cause of blunt abdominal trauma in children and may occasionally be associated with isolated duodenal perforation (IDP). Prompt diagnosis and surgical treatment are vital to prevent increased morbidity and mortality.We report the rare case of an 11-year-old boy admitted for blunt abdominal trauma and treated for an asynchronous double IDP. The first perforation, located on the 2nd/3rd portion of the duodenum, was promptly diagnosed by contrast-enhanced abdominal CT scan after a negative US scan, five hours after injury, and the lesion repaired with a single stitch suture. The second duodenal perforation appeared in the duodenal bulb as a worsening biliary leakage, 48 h after the primary suture of the initial lesion. The perforation was initially seen by digestive endoscopy and sutured in the same way as the first lesion. A third laparotomy was needed 4 days later due to an intestinal obstruction, after which the patient was recovered completely and was discharged home.IDP is a rare consequence of blunt abdominal trauma, and is normally associated with a lesion of other organs, such as the pancreas or bile duct. A delayed diagnosis strongly increases the incidence of morbidity and mortality, and different kinds of surgical management have been proposed, depending on the type of lesion. To our knowledge, this is the first case described in literature of a double isolated asynchronous duodenal perforation following blunt abdominal trauma in children. On arrival, the patient showed right upper quadrant and epigastric abdominal pain with cutaneous swelling, and was alert and afebrile, with a pulse rate of 110/min. Laboratory tests showed an increased WBC count (20.35 × 10 e3/uL - N-91 % with a reference range of 4.0–10.0 e3/uL). Abdominal US was negative, but due to his worsening overall condition, the patient underwent a contrast-enhanced CT scan that showed a suspected duodenal perforation in the II portion of the duodenum, with free air in the retroperitoneum and free fluid in the peritoneal space, wall thickening, and duodenal haematoma a and b. 3Injuries to the duodenum are uncommon due to its retroperitoneal position, and the cause is rarely “blunt” trauma . The duoThere are a number of points we would like to focus on. Despite the negative US, the CT scan permitted an immediate diagnosis, and the laparotomy was performed less than 7 h after the trauma. The careful positioning of the drain allowed safe management and monitoring of the post-operative period, and allowed us to detect the bile leakage from the second asynchronous perforation. To our knowledge, this is the first case reported in literature of an asynchronous double isolated duodenal perforation due to blunt abdominal trauma.4Isolated duodenal perforation due to blunt abdominal trauma is a rare event. In the event of blunt abdominal trauma with a negative ultrasound, the persistence or worsening of upper abdominal pain and the appearance of severe vomiting , a contrast-enhanced CT scan is essential. Management of IDP following blunt abdominal trauma can be extremely challenging, but a prompt diagnosis is fundamental to avoid affecting the outcome. Although different surgical approaches have been described depending on the grade of lesion, it is our opinion that a single primary suture, possibly with an omental patch, should be the first choice of treatment, a position supported by the literature. Correct positioning of the drain is fundamental for post-operative monitoring. We suggest a scrupulous evaluation of the perfusion, of the anatomical distribution of the lesion and of the peripheral extension of the suffusion, when possible, to predict or suspect a subsequent perforation. To our knowledge, this is the first case reported in literature of an asynchronous double isolated duodenal perforation due to blunt abdominal trauma.NO funding or sponsor have been received.NO ethical approval is needed for this CASE REPORT"Written informed consent was obtained from the parents of the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request”.Vito Briganti: Project design, final revision, surgeryStefano Tursini: Project design, final revision, surgery, figure selection, editingStefania Ianniello: Radiologic counseling, figure selection, revisionAndrea Cortese: Radiologic counseling, figure selection, revisionRoberto Faggiani: endoscopic counselingNO registration is due for this CASE REPORTGuarantor: Dr. Stefano TURSINI M.D.Not commissioned, externally peer-reviewedNO financial and personal relationships with other people or organisations that could inappropriately influence the work. NO conflict of interestThe author declare