Nephroblastomatosis is an uncommon pathologic process characterized by the presence of persistent embryonic nephrogenic rests. Progression to Wilms tumour occurs in an estimated 35% of patients. Cure rates are based on histologic findings and disease stage and have improved from 10% in the 1920s to over 90% today.We report a case of a 9-month-old female presenting with a 2-month history of abdominal distension. Ultrasonographic and computed tomographic assessments demonstrated features consistent with bilateral, diffuse, hyperplastic perilobar nephroblastomatosis (DHPLNB) for which she underwent chemotherapy. Magnetic resonance imaging 6 weeks following commencement of chemotherapy revealed a mass concerning for unilateral Wilms tumor for which she underwent partial nephrectomy. Pathology confirmed DHPLNB with a unilateral Wilms tumor.3.5 year radiographic follow up demonstrates complete recovery. To our knowledge, there are no similar cases with imaging depiction recently published. With potential for malignant transformation into Wilms tumour and low survival rate for late diagnosed Wilms tumors, it is important to recognize nephroblastomatosis early, both clinically and radiographically to improve overall patient prognosis. Nephroblastomatosis is an uncommon pathology, with distinct but rare radiological findings. With potential for malignant transformation into Wilms tumour and low survival rate for late diagnosed Wilms tumors, it is important to recognize nephroblastomatosis early, both clinically and radiographically to improve overall patient prognosis. The following describes a case of Wilms tumour complicating bilateral nephroblastomatosis.A 9-month-old female presented with a 2-month history of abdominal distension and weight loss.The patient underwent an uncomplicated birth following an unremarkable pregnancy at 39-weeks gestational age via elective caesarean section. The toddler attained normal developmental milestones aside from the inability to roll prone from supine. Initially at the 70th percentile at birth for weight, she presented at the 30th percentile with stable height and head circumference (50th percentile). The patient did not exhibit any clinical features of Beckwith Wiedemann Syndrome including omphalocele, macroglossia and macrosomia.There is no consanguinity, no family history of recurrent malignancies, haematological or renal conditions.Initial limited ultrasonographic study of the abdomen demonstrated massively enlarged kidneys with loss of corticomedullary differentiation. The right kidney measured 13.1 cm and the left measured 15 cm  [Computed tomography (CT) of the abdomen and pelvis with IV and oral contrast demonstrated homogenous, diffuse, renal enlargement and loss of normal architecture with renal parenchyma replaced by homogenous low attenuating peripheral masses bilaterally Fig. . There wGadolinium enhanced magnetic resonance imaging (MRI) of the abdomen performed after 6 weeks of chemotherapy demonstrated hypointense peripheral masses on T1/T2 images with thick septations suggestive of bilateral DHPLNB. A small heterogeneously enhancing lesion with multiple small linear and round cysts in the medial left kidney was characterized on T1 imaging, suspicious for a superimposed Wilms tumor . No other tumour markers or genetic testing was performed.Bilateral DHPLNB complicated by a left renal mass (likely Wilms Tumour)Renal lymphoma (uncommon in infants and young children).The patient was treated with COG protocol AREN 0534 EE-4A chemotherapy, including vincristine and dactinomycin for 20 weeks. Due to the presence of the superimposed left renal mass, a left partial nephrectomy and perihilar lymph node dissection was performed. Following the surgery, she also received left flank radiotherapy to a dose of 1080 cGy in 6 fractions.Pathology was consistent with DHPLNB and resection of a Wilms tumor. Resection margins were positive. Nodal dissection was negative. Following surgical resection and chemotherapy, the patient was followed with MRI every 3 months for a year. Thereafter, the patient was followed with MRI every 6 months for 2 years. Currently, the patient is now on surveillance with ultrasounds every 3–4 months.Nephroblastomatosis is a premalignant condition typified by multiple residual embryonal cells known as nephrogenic rests . NephrogSonographic features of nephroblastomatosis are typified by enlarged diffusely hypoechoic kidneys. CT imaging demonstrates low-attenuating poorly enhancing peripheral nodularity with scattered patches of adjacent normally enhancing renal parenchyma. On MRI, this corresponds to peripheral nodules with low-signal intensity on T1 and T2-weighted sequences when compared to normal renal parenchyma .In contrast to the multiplicity of the subcapsular nephrogenic rests which define nephroblastomatosis and frequently serve as precursors to nephroblastomas (Wilms tumours); Wilms tumours typically manifests as solid intrarenal masses distorting the renal parenchyma and collecting system . They arTreatment of nephroblastomatosis complicated by Wilms tumor incorporates multimodal therapy including surgery, chemotherapy and radiation, with more aggressive initial therapeutic protocols in the presence of poor prognostic factors. Poor prognostic factors include anaplastic tumour histology, tumour staging, unfavourable molecular and genetic markers and patient age greater than 2 years old . FortunaGadolinium-enhanced T1 and T2-weighted MR imaging is often used to monitor regression or progression of nephrogenic tissue. Tumor regression is evident when there is a reduction in size of the nephrogenic tissue as well as decreased enhancement of the original lesion reflecting increased fibrosis and devascularization of the nephrogenic rests . IncreasNephroblastomatosis is an uncommon pathology characterized by the presence of persistent embryonic nephrogenic restsWilms tumour complicates approximately 35% of casesDiagnostic imaging can be useful for the diagnosis of diffuse hyperplastic perilobar nephroblastomatosis, and screening for complications such as Wilms tumour.


In this case, we report an epithelioid haemangioma (EH) of the fibula with ill-defined multifocal lesions and a resultant pathologic fracture. Based on radiographic appearance, these lesions were initially thought to represent a malignant process, such as primary malignant bone tumour, metastases or multiple myeloma. Osseous EHs are rare. Although they can present as multifocal lesions, the majority of bony EHs are solitary and arise in the diaphysis or metaphysis of long tubular bones, with a predilection for the lower extremity. Non-specific radiological findings, debatable cytological appearance and unpredictable clinical growth patterns commonly cause misdiagnosis of malignancy. To the best of our knowledge, a case of EH with multiple growing lesions of the fibula has not yet been reported in the literature. A 63-year-old female with no significant prior trauma and relevant past medical history presented with a 2-month history of severe, persistent, gradually worsening, sharp and non-radiating pain over her left distal fibula. She reported that the pain interfered with sleep and did not subside with non-steroidal anti-inflammatory drugs. The pain was accompanied by no constitutional symptoms. Physical examination revealed extreme tenderness to palpation over the lateral distal fibula. There was soft tissue prominence over the mid to lower third of the fibula.Left lower leg radiographs depicted two ill-defined osteolytic destructive lesions measuring 29 and 37 mm in length in the fibular diaphysis. A pathologic fracture was present through the distal lesion . Bone sc1 is an uncommon, slow-growing vascular tumour that generally presents on the skin and subcutaneous soft tissues of the head.2 Osseous EHs are rare. Although they can present as multifocal lesions, the majority of bony EHs are solitary and arise in the diaphysis or metaphysis of long tubular bones, with a predilection for the lower extremity. On radiographic examination, these lesions demonstrate a well-defined osteolytic process with sclerotic margins or mixed lytic and sclerotic lesions. These lesions are often located eccentrically and may demonstrate cortical disruption or an intact cortex.3 In terms of histological features, intraosseous EHs have been classified as benign neoplasms in the World Health Organization taxonomy of bone tumours.4 However, some researchers believe that EH of bone should be considered a variant of haemangioendothelioma, a tumour with malignant potential.5 Non-specific radiological findings, debatable cytological appearance and unpredictable clinical growth patterns commonly cause misdiagnosis of malignancy.EH, also known as angiolymphoid hyperplasia with eosinophilia and histiocytoid haemangioma,The patient was initially managed conservatively and placed in a walker boot. Although some pain relief was achieved, the patient’s pain persisted. At the 2-month follow-up, an additional third smaller lesion was detected along with an interval increase in size of the two previously seen lesions . Additio3Accurate diagnosis of EH requires an understanding of the clinical, radiological and pathological features of this entity. Patients can present with a wide variety of symptoms and signs, and the radiographic appearance of these lesions can mimic malignant neoplasms. The histological aspect of this group of tumours varies from lesions easily identified as being vasoformative to those that may imitate other mesenchymal neoplasms or even metastatic carcinoma.6 Females and males seem to be affected equally by EH, unlike other types of intraosseous haemangiomas that predominantly occur in females. Two cases of EH in females were reported after pregnancy, which is not the case in our patient.7Although most osseous EHs are identified as incidental findings on radiographs, pain has been the most common presenting complaint of patients with these lesions, as in our case report. A case report of EH with secondary lymph node enlargement has been described, which demonstrates its behaviour as a malignant tumour.8 EHs do not commonly cause destruction of the bony cortex but when the cortex is involved, focal cortical destruction is commonly seen with thick reactive periosteal new bone formation.3 Our initial radiographs demonstrated two destructive, lytic and expansile lesions with wide zones of transition associated with mild adjacent periosteal reaction. Given the patient’s clinical presentation, age and aggressive radiographic appearance, our primary differential diagnosis included metastatic disease, lymphoma, multiple myeloma and, less likely, primary bone lesions.Intraosseous EHs lack any characteristic features and radiographs generally demonstrate a well-defined lytic lesion involving the bony metaphysis or diaphysis with associated osseous expansion and sclerosis. EHs may also show a mixed lytic and sclerotic pattern of bone destruction as described previously in the literature.9 It is more difficult to differentiate between EH and EHE as a result of significant overlap at the cytological level, with epithelioid cells showing well-defined cell borders and abundantly dense eosinophilic cytoplasm. Both entities may have some level of cytological atypia. The presence of a specific myxochondroid or densely sclerotic stroma in EHE and the focal presence of mature vessels with open lumen formation in EH are the only consistent distinctive histological features between these two entities.10Histological features remain the cornerstone of the diagnosis of epithelioid vascular tumours. The differential diagnosis of EH includes epithelioid haemangioendothelioma (EHE) and epithelioid angiosarcoma. Characteristics that distinguish EH from epithelioid angiosarcoma include the absence of significant cytological atypia, brisk mitotic activity, as well as necrosis and presence of well-formed vessels.EH is an uncommon, slow-growing vascular tumour that generally presents on the skin and the subcutaneous soft tissues of the head, with osseous EHs being rare.Although osseous EHs can present as multifocal lesions, the majority of bony EHs are solitary and arise in the diaphysis or metaphysis of long tubular bones, with a predilection for the lower extremity.Radiographically, EH may present as well-defined lytic lesions with sclerotic margins or mixed lytic and sclerotic lesions. These lesions are often located eccentrically and may demonstrate a disrupted or intact cortex.Written informed consent for the case to be published  was obtained from the patient for publication of this case report.


The aneurysmal bone cyst is a nonneoplastic, osteolytic and locally destructive lesion that mainly affects the metaphyseal area of long bones and only 2% of it is diagnosed in the maxillofacial skeleton. Although surgical treatment is the most common option, it is associated to high morbidity rates. The case of an aneurysmal bone cyst of a considerable size in a 27-year-old male patient illustrating a conservative surgical approach with preservation of the dental structures in the mandible to limit aesthetic and functional side effects is presented. Two-year clinical follow-up was performed with no evidence of recurrence. Key words:Aneurysmal bone cyst; curettage, conservative treatment, mandibular osteotomy. The aneurysmal bone cyst (ABC) is a benign intraosseous lesion that develops in patients under 30 years, its location is usually the metaphysis of long bones and only 2% of cases are diagnosed in the maxillofacial skeleton; it represents 1,5% of nonodontogenic maxillary cysts, and has a 3:1 mandible/maxilla rate  , vertebral column 20%), and around 2% manifests in the bones of the mandible 0%, and a. AccordiABC is a rare, expansive, locally destructive lesion and it constitutes a diagnostic and therapeutic challenge in the daily clinical practice . TherapeAlthough surgical treatment is the most common alternative, it is associated to high morbidity rates including postoperative aesthetic and functional alterations, usually as a consequence of extensive resections, in expansive lesions. This article presents the case of a mandibular ABC of significant size successfully managed through a conservative surgical approach aimed at limiting the aesthetic and functional side effects in a young patient.27-year-old male patient with no underlying systemic diseases who sought medical attention because of a three-month medical case of facial asymmetry from increased volume of the left chin area Fig. a, with nAdequate mouth opening and mandibular mobility were found in the clinical examination; no reactive lymphadenopathies were found. Permanent dentition, stable occlusion and displacement and rotation of tooth #33 were observed in the intraoral examination; swelling of the soft tissues and effacement of the bottom of the sulcus between teeth #34 and #42 were found. The oral mucosa was found to be healthy, smooth, firm and with no pain during palpation.Panoramic radiography revealed a multilocular radiolucent lesion with no defined borders, extending from the mesial root of tooth #36 to tooth #44, that affected the interradicular bone, with root resorption of teeth #31, #41 and #42   have bee


Enhancement patterns of visceral venous collaterals are well documented in cases of superior vena cava obstruction. Only recently has intraosseous venous collateral enhancement been described. We describe an unusual case of vertebral marrow enhancement in the lower thoracic spine related to venous collateral circulation caused by an incidental hemiazygos thrombus. Misinterpretation of this finding can lead to the erroneous interpretation of sclerotic bone metastases. A 66-year-old female with a history of breast and lung cancer, both treated and in remission, presented with new onset of generalized abdominal pain. Physical examination and laboratory work-up were unrevealing as to a potential cause.A contrast-enhanced CT (CECT) of the abdomen and pelvis was performed with series obtained in both arterial and delayed phases that failed to reveal an abdominal or pelvic source of the pain. Comparison with a CECT performed 2 years prior, however, revealed new areas of high attenuation in the T11 and T12 vertebral bodies and posterior elements . This waGiven the potential of tumour recurrence, a positron emission tomography CT scan was performed without contrast to evaluate the lesion and to assess for other sites of disease. The study revealed normal-appearing thoracic vertebrae without evidence of 18-fludeoxyglucose-avid metastatic disease. On review of the prior CECT in question, it was noted that the majority of the high density areas were centred in the region of the basivertebral veins. In retrospect, this finding was present on arterial phase images and absent on the delayed phase images that prompted a search for the potential cause of this atypical enhancement pattern. Careful review near the source of marrow enhancement revealed incidental thrombus in the hemiazygos vein . It was The leading diagnosis for a new finding of high attenuation centred in the vertebral body in a patient with known cancer is osteoblastic metastatic disease. Only after careful review was intraosseous venous collateral enhancement considered. Other potential causes of sclerotic bone lesions, including entities such as enostosis, osteoid osteoma or chronic osteomyelitis, were felt less likely given the interval appearance and imaging characteristics.Following the detection of venous thrombosis as explanation for the abnormal bone findings, the patient was treated with anticoagulation for 6 weeks without complications.To our knowledge, incidental thrombus in the hemiazygos vein is an unreported finding with scarce literature as to its imaging appearance and appropriate management. It was felt the clot was an unlikely cause of the patient’s abdominal pain and was instead an incidental finding. Clinical work-up for a potential source for the clot was unrevealing. The patient was anticoagulated for 6 weeks, with repeat CECT showing resolution of both the hemiazygos thrombus and unusual vertebral body enhancement.Although unusual, enhancement of venous collateral pathways has been reported, primarily in cases of superior vena cava (SVC) obstruction.Predictably, the pattern of venous collateral enhancement will depend on the site of venous obstruction as well as variations in venous anatomy and study technique. Atypical hepatic and pericardial enhancement related to collateral reflux has been described in cases of symptomatic obstruction of the SVC.Little is known about thrombus in the hemiazygos vein with respect to its imaging appearance. In our case, the initial finding was only identified after a careful search for the cause of atypical marrow enhancement. It was unclear from our study whether the hemiazygos clot was actually fully or near fully occlusive—only that it had elevated venous pressure enough to cause retrograde opacification through the vertebral venous plexus, which was absent on delayed phase. In retrospect, change in the vertebral body appearance on different phase images highlights the need to review potential pathology on all provided phases.With respect to managing hemiazygos vein thrombosis, literature review reveals scarce information. Work-up for a potential source of the thrombus included a negative sonography and coagulation profile. Given the small clot burden and lack of a potential source, our patient had a CECT performed following 6 weeks of anticoagulation that showed resolution of the thrombus and absence of the previously demonstrated vertebral body enhancement. No additional treatment was performed as it was felt the clot was an incidental finding and an unlikely source of the patient’s abdominal pain.The case presented illustrates a benign entity related to contrast filling the vertebral venous plexus in a retrograde manner owing to thrombus in the hemiazygos vein.Venous collateral enhancement depends on the site of obstruction with previous reports illustrating collateral enhancement in cases of SVC obstruction, typically by an obstructing mass. These patients may present with findings of SVC syndrome, including facial oedema, upper extremity venous congestion, dyspnoea and headache.Little is known about hemiazygos vein thrombosis with regards to its imaging appearance and management. Our patient presented with abdominal pain that was felt to be unrelated to the clot and was anticoagulated for 6 weeks without complication.Whenever there is new or unusual attenuation within a vertebral body, findings on all phases provided should be reviewed and contrast reflux through intraosseous venous collaterals considered as a potential pitfall.


Orbital lesions are traditionally managed through external approaches when laterally located, and through a transnasal approach or other external approaches when medially located. However, when the lesion is superomedially located, it may determine a technical challenge.In this study, we present the case of a patient with a superomedial intraconal venous malformation of the left eye. We addressed the mass through a combined approach, using the transnasal route as the main approach, and the superior eyelid approach to push down the lesion to facilitate the excision. We have called this approach “push–pull technique.”We achieved a complete resection of the lesion and did not observe any intraoperative or postoperative complications. The last follow-up at 6 months postoperatively showed no recurrence, and the patient was satisfied and completely recovered.According to our experience, the “push–pull” technique seems to be a safe procedure and might be considered a valid alternative to address selected superomedial intraconal lesions. Orbital disease management, although technically demanding and challenging, benefits nowadays several surgical approaches. According to the location, biological behavior, and size of the lesion, the surgeon should design and choose his/her surgical procedure to offer the best angle of attack the lesion itself, thus minimizing the risks to damage noble neurovascular and muscular intraorbital structures.Laterally located lesions are traditionally managed through lateral orbitotomy or superior eyelid (SE) approach,A 45-year-old woman was referred to the Division of Otorhinolaryngology at the University of Insubria-Varese, Italy, for the incidental finding of a left asymptomatic intraorbital mass, anteriorly located in the superomedial intraconal space, between the superior rectus (SR) and superior oblique (SO) muscles. The imaging suggested a diagnosis of venous malformation. The patient was submitted to a wait-and-scan strategy, and the magnetic resonance imaging (MRI) performed over the years showed a progressive significant increase in the size of the lesion . In FebWe performed resection of the middle turbinate, complete sphenoethmoidectomy, and opening of the frontal recess. We identified, cauterized, and incised the anterior ethmoidal artery to free the periorbit and mobilize the anterior orbital content. We removed the lamina papyracea and opened the periorbit just in close relationship to the lesion. Once the lesion was visualized, it was dissected free with the aid of cottonoids. Hence, we performed an SE approach. We identified, infiltrated, and incised an adequate palpebral crease. A careful dissection below the orbicularis oculi muscle was performed, and the superolateral border of the orbital rim was reached and skeletonized. After this, a careful subperiosteal dissection of the orbital roof was performed until reaching the medial periorbital region close to the lesion. Having done that, while the lesion was pulled transnasally, from the outside we pushed the orbital content, taking care not to damage the periorbita, in a lateromedial direction to complete the resection of the lesion. In this way, we completed the dissection and resection of the lesion. The periorbit should be spared during the SE approach to make the external push more effective. We have called this approach “push–pull technique” .The surgical procedure was performed by a multidisciplinary skull base team with sound experience in extended endoscopic-assisted skull base surgery. The surgical time was approximately 90 minutes. We obtained Institutional Review Board approval for reporting the data.We achieved complete resection of the lesion, as confirmed on the postoperative MRI . We didOrbital surgery requires great care to safeguard structural integrity of the vital intraorbital content and associated functions, as well as to preserve the aesthetics of the face. The decision on the surgical approach to undertake should be based on the position, size, and suspected nature of the lesion. In case of well-capsulated intraorbital masses, position and size remain the main criteria to take into consideration when planning surgery.In this case, we focused on a lesion suspected to be a venous malformation. This is a well-capsulated slowly growing mass, which typically started to cause progressive painless proptosis associated with visual field deficit and eye movements' impairment in a middle-aged woman. The lesion presented an atypical anterior location, in the superomedial intraconal space, between SR and SO muscles.Recently, the medial orbital space has been addressed through a transnasal corridor.Recently, the “Pittsburgh group” has proposed an algorithmOur case presented a major difficulty, that is, a superomedial and anterior location. Due to the size and the particular position of the lesion, an exclusive transnasal approach would not have been possible due to the different plane of the ethmoidal and orbital roof that obstructs the complete visualization of the lesion. We would like to stress on the anatomical concept that the axial plane passing through the orbital roof is significantly above the axial plane passing through the fovea ethmoidalis, thus making the dissection of the upper part of the lesion  really complex and uncomfortable. However, we were able to initially dissect the lesion through the nose and complete the dissection after pushing the lesion downward, below the plane of the fovea ethmoidalis.We believe that the SE approach is a simple and minimally invasive open approach. The incision in the eyelid is quite invisible. As a technical advice, we can underline that a more inferiorly located incision is better from an aesthetic viewpoint given the fact this area is covered when the patient has the eye open. An eyebrow or subbrow incision is by far more evident. This external approach was not needed to visualize and dissect the lesion externally but just to push the lesion medially while pulling it from the nose to complete its resection. The periorbit has to be spared superolaterally, and the orbital content did not need to be entered. No muscle detachment was necessary.After 6 months, no enophthalmos was evident . This iCertainly, the lesion could have also been excised through an anterior transpalpebral approach, but we felt more comfortable using a dual-port approach. The final aim of our description is not to propose the “push-pull” technique as the standard of care in such cases but only to present its feasibility in really selected cases and in experienced hands. In term of minimally invasiveness, we would like to stress on the concept that what really matters is not to create damage to noble structures. We feel that the functional aspects represent the most important elements to be considered. In our view, it is not the presence of a skin incision that makes an approach invasive or not. However, we feel that our approach is a well-balanced solution in term of minimal invasiveness, efficacy, and safety. We presented our case just to show that it is possible to combine two different approaches, taking into account the benefits of every approach while minimizing negative aspects.Finally, from a biological viewpoint, the procedure was safe since the resection could be completed in a one-piece fashion. The patient did not experiment any recurrence since then up to the present day. Last consideration regarding the type of the lesion is that only lesions with expected weak adhesions can be approached through this technique. In lesions with strong adhesions, this kind of technique cannot be proposed. We would like to emphasize the use of cottonoids in this type of dissection. They are very useful in sparing noble structures surrounding the lesion and minimizing surgical trauma. They also allow the surgeon to perform a smooth dissection and keep the surgical field clean, absorbing the blood.In conclusion, the “push–pull” technique is a safe procedure, which might be considered a valid alternative to address selected superomedial IOLs. However, it should be attempted only by experienced teams able to convert the procedure into a more conventional route and able to face major complications.Of course, the real applicability of this technique still needs to be confirmed; however, we would like to highlight the importance of modulating the surgical approach according to the needs of the patient.


Erythropoietic protoporphyria (EPP) is a rare disorder caused by reduced ferrochelatase activity and shows incomplete autosomal dominant inheritance. Meticulous perioperative management can avoid characteristic complications. This report describes a case of a bleeding jejunal tumor in a patient with EPP.th day of hospitalization. Yellow filters were used to avoid operating room light-induced tissue injury. The tumor was located in the jejunum 30 cm from the Treitz ligament toward the anal aspect. The histopathological diagnosis was desmoid-type fibromatosis of the jejunum. Postoperatively, his hemoglobin levels were stabilized; however, his serum bilirubin level remained high. His serum bilirubin level gradually decreased following hemin injections (150 mg/day).A 49-year-old man with a history of EPP was admitted to our department with abdominal distention and severe anemia. Contrast-enhanced computed tomography revealed an abdominal tumor measuring 5 cm, originating from the small bowel wall or mesentery. Tumor resection was planned after correction of anemia. Red blood cell transfusion restored his hemoglobin to acceptable levels; however, his liver function worsened. Institution of liver support therapy achieved gradual reduction in his elevated liver enzymes; however, hyperbilirubinemia persisted. He underwent tumor resection on the 12Reducing heme synthesis and minimizing protoporphyrin generation are important perioperatively. Additionally, preventing operating room light-induced tissue burns and selecting appropriate anesthestic agents are important during surgery.The institution of appropriate treatment and adequate intra- and perioperative measures can ensure safe surgery in patients with EPP even under emergency conditions. Erythro2A 49-year-old man was admitted with abdominal distention to the hospital where this study was performed. He reported a several-year history of photosensitivity and EPP-induced liver dysfunction. Laboratory tests performed on admission are shown in l-cysteine hydrochloride hydrate), after which AST and ALT levels gradually decreased, although T-bil remained elevated. Once again, a reduction in hemoglobin level was observed, and although liver function had not recovered adequately, tumor resection was planned to control bleeding.After admission, RBC transfusion restored his hemoglobin to acceptable levels; however, serum levels of total bilirubin (T-bil), aspartate aminotransferase (AST), alanine aminotransferase (ALT) and the PP content of RBCs were elevated. He received daily injections of Stronger Neo-Minophagen C  were performed. The operation time was 104 min, and the intraoperative estimated blood loss was 55 mL.On the 12Histopathological examination showed hyperplasia of spindle cells in the muscularis propria of the small intestine. Immunohistochemical examination of the resected specimen showed that the tumor cells stained positive for α-smooth muscle actin and Calponin and negative for CD34, S-100, c-Kit, Desmin, ALK-1, DOG1, and Caldesmon stains. The Ki-67 labeling index was 3.0%. The final histopathological diagnosis was desmoid-type fibromatosis (DF) of the jejunum .Fig. 2a:His hemoglobin levels were stabilized postoperatively. He resumed oral intake on postoperative day (POD) 4 without digestive complications; however, his serum bilirubin levels continued to increase and hyperbilirubinemia persisted despite increased quantities of Stronger Neo-Minophagen C administered. His abdominal pain returned, and he showed elevated PP levels in RBCs. Liver supporting therapy alone could not treat this condition, necessitating injections of hemin at a dose of 150 mg/day from POD 21 to 24. After the initiation of hemin therapy, his serum bilirubin levels gradually decreased with resolution of symptoms. Hyperbilirubinemia was not observed, and he was discharged on POD 47. His clinical course is summarized in 3Meticulous perioperative management is required in patients with EPP to avoid complications. The literature contains a few case reports describing surgery in patients with EPP : howeverThe level of PP determines the degree of photosensitivity and hepatic dysfunction; therefore, maintaining low PP levels perioperatively can reduce the severity of EPP. Maintaining adequate hemoglobin levels is important because anemia triggers the activation of the heme biosynthesis pathway, thereby increasing PP production ,13. RBC Intraoperative management of EPP primarily involves prevention of operating room light-induced tissue burns and selection of the appropriate anesthestic agents. PP absorbs light from the blue portion of the spectrum (wavelengths 380–460 nM), which triggers the formation of free radicals that precipitate tissue injury ,18. The Several anesthetic agents are associated with EPP progression. Fentanyl, droperidol, vecuronium, and isoﬂurane are associated with a low risk; therefore, fentanyl, sevoflurane, and rocuronium were used in this patient. The safety profile of propofol is controversial, which precludes its use.Preoperative diagnosis was difficult in this case, and the lesion was considered a small bowel tumor such as a gastrointestinal stromal tumor. Surprisingly, the final diagnosis  was DF. DF are rare soft tissue tumors associated with trauma, gastrointestinal surgery, pregnancy, and oral contraceptive use . Patient4In conclusion, the institution of appropriate peri- and intraoperative measures can ensure safe surgery in patients with EPP even during emergencies.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.Not commissioned, externally peer-reviewed.Ethical approval from Hiroshima University.This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.All authors in this manuscript contributed to the interpretation of data, and drafting and writing of this manuscript. Yu-ki Takemoto is first author of this paper. Shoichiro Mukai is corresponding author of this paper. Yu-ki Takemoto, Shoichiro Mukai, Hiroyuki Egi conceived and designed the study and drafted the manuscript. Shoichiro Mukai first diagnosed. Yu-ki Takemoto, Shoichiro Mukai, Testsuya Mochiduki, Masatoshi Kochi, Hiroyuki Egi were engaged in patient’s care in our hospital including surgery. Hiroyuki Egi contributed to study concept, and review of the final manuscript and submission of the paper. All the authors read and approved the final manuscript.None of the authors have any commercial or financial involvement in connection with this study that represents or appears to represent any conflicts of interest.Shoichiro Muki.The manuscript does not report the result of an experimental investigation or research on human subjects.


Dear Editor,A 67-year-old postmenopausal, multiparous female presented with a one-month history ofvulvar edema, with no vaginal bleeding or dysuria, reporting only local discomfort. Asolid, fixed, painless nodule was found on her vulva. Magnetic resonance imaging (MRI)showed an oval fibrous mass, with a hypointense signal in T1-weighted sequences and aheterogeneous, predominantly hyperintense signal in T2-weighted sequences . The for(1). In the internationalliterature, only 12 cases of SCC have been reported(2). Almost all of those casesinvolved multiparous women, between 50 and 80 years of age, with a vulvar mass, althoughwith various presentations(2): as a painful mass, in four cases; as a painful massaccompanied by abdominal pain, in three cases; as urinary dysfunction, in another fourcases; and as pain at the base of the penis with sexual dysfunction, in a rare caseinvolving a male patient.The first description of SCCs was in 1996 by Algucial-Garcia et al.(2-5). It is believed to be secondary todegenerative changes. It may remain stable or present minimal size reduction with onlyone case in 2015 in Japan, where there was complete and spontaneous regression after twoyears(2-5).An SCC is a rare form of ganglion cyst that begins on the inferior surface of thesymphysis pubis, consisting of a collagen capsule composed of gelatinousfibrocartilaginous tissue in degeneration, mucin, and debris(6-10). A diagnosis of SCC, which is based on clinical andimaging findings, depends on the amount of mucinous and cartilaginous material, whichresults in a heterogeneous aspect on MRI. Degenerative alterations in the symphysis pubis can beseen on X-rays(3).Imaging exams, MRI in particular, have garnered increasing attention in the assessment ofpelvic diseases(12): Such findings include asignal that is hypointense (in relation to that of the muscle) in T1-weighted sequencesand heterogeneously hyperintense in T2-weighted sequences, the lesion being in close,extensive contact with the symphysis pubis and presenting wall enhancement aftergadolinium administration, without internal enhancement(2).The MRI findings of SCC were first described in 2004 by Kim et al.. The differential diagnoses of SCC inpatients with a vulvar mass include lipomas; cysts of the urethra; Nabothian cysts;Bartholin gland cysts; Gartner duct cysts; paratubal cysts; cysts in the symphysis;subchondral pseudocysts in rheumatoid arthritis; and subchondral cysts. Other potentialdiagnoses include malignant tumors such as squamous cell carcinoma, Bartholin glandcarcinoma, chondrosarcoma, and melanoma of the vulva. In general, the differentiation is easy, depending, asit does, on the location and radiological characteristics of thelesion.The clues for a correct diagnosis of an SCC are a cystic lesion that is located on themidline and is in close contact with the symphysis pubis. The treatment of choice is resection, because the bulkycontent of the cyst precludes aspiration. No cases of recurrence have been reported,although none of the patients involved were followed for more than three years. In onecase, the SCC was not treated and there were no changes in its size or characteristicsafter two years of follow-up. In that case, the resection of the SCC was complicated byseparation of the symphysis pubis. Because SCC is a benign condition, all efforts shouldbe made to preserve the stability of the symphysis pubis.Preoperative biopsies of SCCs are reserved for cases in which there is a high suspicionof malignancy


Phyllodes tumors are spectrum of tumors ranging from benign to malignant. Malignant spectrum pose a management challenge for clinicians due to high risks of recurrence and metastasis. Malignant phyllodes tumor with brain, lung and adrenal metastases at the same time is rare.A 37 years-old unmarried female presented with an ulcerated huge lump in left breast with severe bleeding. Trucut biopsy showed necrosis with spindle cell proliferation with atypia for which she underwent modified radical mastectomy with final diagnosis of malignant phyllodes tumor. Three months after surgery, patient presented with headache, which on further evaluation showed masses in lung, right adrenal and brain.We presented a rare case of malignant phyllodes tumor with clinical, imaging and histological findings with metastases to multiple sites early in course and poor outcome of the patient despite margin negative resection. •Metastatic malignant phyllodes tumor have poor outcome.•Simultaneous metastasis to brain, lung and adrenal is rare.•Margin negative resection is feasible even in huge malignant phyllodes tumor. They are classified by WHO into benign, borderline and malignant variants based on stromal cellularity, cellular atypia and pleomorphism, mitotic index, stromal overgrowth, tumor margin, and the presence or absence of heterologous differentiation . The beh. They ar2A 37 years-old unmarried premenopausal female presented in the emergency of our hospital with complaints of bleeding from ulcerative lesion in the left breast for a couple of hours. The patient had history of ulcer in the left breast for past 2–3 weeks which developed over the underlying huge breast lump. The lump was present for past 5 months but rapidly grew over the last 3 months to current size. There was no history of coagulopathy, trauma, chronic liver disease or history of similar illness in first degree relatives. The patient attained menarche at the age of 15 years. She is a non-smoker and denies history of contraceptive use or alcohol intake.On examination in our emergency there was severe pallor with tachycardia. However the blood pressure was maintained. There was an oozing ulceration in upper outer quadrant of the left breast with underlying huge lump measuring 15 × 15 cm occupying the upper outer, lower outer and upper inner quadrants with deformed shape of the breast a. The skBefore the development of breast lump, the patient had itchy lesion at the same site. The patient unfortunately attributed all these events to that itchy lesion and sought help late. There was no history of headache, shortness of breath, abdominal distension or bone pain.On investigating the patient, she was severely anemic with hemoglobin of 5 gm% with normal total and differential counts. Coagulation parameters and liver function tests were normal. Chest X-ray showed no evidence of metastasis other than dense breast shadow on left side due to huge breast lumpa.Fig. 2. Histopathological examination showed maximum tumor size of 14 cm with increased stromal cellularity, loss of stromal-epithelial balance and frequent mitoses more than 45 per 10 high power fields  typical Postoperatively patient gained weight and was doing well. Three months after surgery, the patient started developing progressively increasing headache, nausea and vomiting for which she got admitted in our center. There was no local recurrence. However on further investigations, she had a huge cystic lesion with mural nodule in her brain b,c,d. Co3Phyllodes tumors are notorious for local recurrence and progression. Only less than 10% of these tumors grow larger than 10 cm and the largest reported case happens to be of 50 × 50 cm by Islam et al.  with metThere are two cases of malignant phyllodes with simultaneous brain, lung and adrenal involvement . Our casPatient attributed the illness to itching and sought treatment late when she already had ulceration and bleeding from the lesion. Increasing awareness is required especially in developing countries where health seeking behaviors are still poor and where people correlate ominous diseases with some trivial event that does not even have temporal relationship with the current illness.Closure of wound after excision of giant phyllodes tumor is a challenge. However total mastectomy with primary closure is still feasible in such cases while achieving negative margins as we did in our case. We do not image the head and spine routinely unless the patient is symptomatic in these areas. Our practice to screen for metastasis is to image abdomen and pelvis with ultrasound, and chest with X-ray first, proceeding to CT if any abnormalities are detected in these scans.One may argue that the lesions in chest or abdomen could be present during initial presentation although the chest X-ray and USG abdomen and pelvis were negative. But given the rapid increase in size of adrenal (tumor velocity), we strongly believe that there could not have been such lesions during initial presentation.We believe that atleast chest and abdomen should be screened with contrast enhanced CT especially in cases with malignant phyllodes tumor so as not to miss subtle lesions even if the patient is asymptomatic at these sites.Postsurgical management in margin negative phyllodes tumor is still controversial. A study with 30 malignant phyllodes cases do not recommend radiotherapy in margin negative resection . Althoug4Malignant spectrum of phyllodes is notorious for metastases with dismal outcome. Although the lungs and bones are the most common sites, there can be simultaneous metastases to brain, lung and adrenal as in our case. Contrast enhanced CT of chest and abdomen should be routinely done in malignant phyllodes tumors to look for metastasis.Not applicable as it is a case report.No funding was received from any source for this work.SK wrote the article. YPS and SK were the operating surgeons, AB and RS helped in literature review, acquisition of histological, radiological images and revising the manuscript. All authors read and approved the final manuscript.None.Not applicable.Suman Khanal, Yogendra P Singh.Written informed consent was obtained from 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.Not applicable as it is a case report.


Asymptomatic lipoma only requires observation, whereas symptomatic lipoma requires treatment such as endoscopic or surgical resection. However, in case of multiple lipomas, with evident diffusion and malignancy, resection procedure and range cannot be determined.We experienced GI lipomatosis (multiple lipomas) diffusely existing from the duodenum to the small intestine and involved recurrent intussusception.47 year-old female was a history of open bowel resection for intestinal obstruction caused by intussusceptions of multiple small intestinal lipoma 11 years ago. EGD showed duodenal lipoma, and CT showed diffuse multiple lipomas from the proximal jejunum to the distal ileum. Another CT also showed intussusception of small intestine, but no signs of intestinal obstruction. Surgical procedures performed included diagnostic laparoscopy. All intestinal lipomas were resected with local excision, and duodenal lipoma was resected with ESD without any bowel resection.Multiple local excision  ESD for multiple GI lipomatosis have not been reported. The most problematic thing is that if extensive resection is performed to cut off all multiple lipoma, short bowel syndrome may occur. Determining the range to be cut remains unclear.Multiple local excision ± ESD seemed to be one of the methods in resecting multiple GI lipomatosis. In the future, cases and indications of surgery and resection method for GI lipoma should be accumulated and considered, respectively. There was no finding suggestive of malignancy at the time of palpation. All intestinal lipomas were resected with local excision, and duodenal lipoma was resected with ESD without any bowel resection, as a considerable amount of small intestinal resection complicates postoperative short bowel syndrome.No previous studies have reported the use of multiple local excision + ESD for the treatment of multiple lipomatosis; therefore, this case is reported along with a discussion of the relevant literature. This case report has been reported in line with the SCARE criteria .2Herein, we present a case of a healthy 47-year-old woman. In 2005, she underwent open bowel resection for intestinal obstruction caused by intussusceptions of multiple small intestinal lipoma. No other major complications were observed. Recently, she complained of abdominal pain, and after careful examination, EGD showed the 20-mm duodenal lipoma a, and di3With the recent advances in endoscopy and modern imaging technologies such as CT scan and MRI, more cases of lipoma are being diagnosed and treated. Current imaging modalities such as CT and MRI can provide an accurate diagnosis . GI lipoSymptoms observed in the GI lipoma become an indication for resection, and the method and range of resection remain controversial. The localized, small, solitary lipoma can be easily and safely removed using an endoscope, especially the duodenum and the proximal small intestine. Endoscopic treatment was performed using four methods: the unroofing technique, endoscopic mucosal resection (EMR), EMR after precutting (EMR-P), and endoscopic submucosal dissection (ESD) . In caseExtensive resection of all multiple lipomas can cause short bowel syndrome. Therefore, determining the exact range remains a problem. However, with excision, the total tumour resection can be performed without resecting the intestinal tract regardless of the number of tumours. All lipomas were resectable by combining ESD and local excision with plastic repair as in this case. Although the procedure requires long duration and is complicated, it seemed to be one of the methods of resecting multiple lipomas because it prevents short bowel syndrome and allows total resection.Lipomatosis is a benign disease, and mobility is good if it occurs in the small intestine; therefore, surgery under small laparotomy is indicated. Beside, in cases of multiple and diffuse lipomas, the entire position of the tumour cannot be determined preoperatively, and small umbilicus laparotomy would be an option in this case, considering resection, reconstruction, and suturing. However, as the duodenum has poor mobility and many adjacent organs were found, its surgical procedure becomes very complicated in cases of duodenal lipoma. Laparoscopic and robotic surgery may be suitable for duodenal lipoma, which is difficult to endoscopically resect. Laparoscopic approach is minimally invasive surgical procedure with minimal postoperative pain and short hospitalization . RoboticIn the future, cases and indications of surgery and resection method for GI lipoma should be accumulated and considered, respectively.　4This is an unusual case of ileo-ileo intussusception caused by GI lipomatosis in an adult. Multiple local excision ± ESD seemed to be one of the methods in resecting multiple GI lipomatosis.More case reports and surgical approaches can establish future progress in surgery.This case report did not receive any conflicts of interest.This case report did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.This case report was performed in compliance with the principles of good clinical practice outlined in the Declaration of Helsinki and federal guidelines. This case report was approved by the local ethic committee.This report was written informed consent was obtained from every participant or the participant’s guardian.Yasunori Yoshimoto, Takahisa Fujikawa, and Tsunenori Yamamoto greatly contributed to preoperative diagnosis, determination of surgical technique and surgery. Tomoharu Yoshida and Yasuyuki Shirai contributed to preoperative diagnosis and performed an endoscopic approach. Yasunori Yoshimoto, Tomoharu Yoshida were a major contributor in writing the manuscript and helped with the critical revision. All authors read and approved the final manuscript.This is a case report, not research studies.Yasunori Yoshimoto is the Guarantor.Not commissioned, externally peer-reviewed


Retroperitoneal cysts are a rare entity in the field of surgery and their management remains a clinical dilemma for many surgeons. This study addresses the clinical management of three benign retroperitoneal cysts which were presented in our hospital.The objective of this case series is to explore the different clinical presentation of the retroperitoneal cyst (RPC). This study also aims at different tools of investigations especially role of CT scan in the diagnosis of RPC and finally the different treatment options available for RPC.This is a single centered case series of three retroperitoneal cysts which were presented in surgical department of our hospital in the year 2016 and 2017. Total three patients were presented in this case series, two of them were females and one was male. Females were 13 years and 23 years age-old, male was 45 years old. Two of them admitted through the Outdoor Patient Clinic and one was presented to the surgical emergency. Informed consent from the patients and guardian (case1) has been taken. Research work has been reported in line with the PROCESS criteria.They were investigated and treated in our hospital. Two of them were diagnosed with retroperitoneal cysts preoperatively and one was diagnosed pre-operatively. In all three cases, surgery had been carried out and they had been observed in their early postoperative period and then followed up for two years. All biopsies had been followed and turned out to be benign idiopathic cysts. These patients have been followed up for 2 years after completion of their treatment.Retroperitoneal cysts in adults are an uncommon manifestation of abdominal pathology. Their presentation is quite vague. The rarity catches the surgeon off guard at times. Radiology plays a crucial role in defining these lesions and complete surgical excision remains the cornerstone of treatment for symptomatic and complicated patients. They ar22.1A 13-year-old young female presented to the emergency department with abdominal pain, constipation and repeated bouts of vomiting for the last three days. The pain was sudden in onset, colicky in character and intermittent in onset. Her past medical history showed that she was treated with anti-tuberculous therapy for pulmonary tuberculosis. She took anti-tuberculosis treatment for nine months and completed this course two years back. General physical examination showed a heart rate of 110 beats/min and a BP of 90/60 mmHg with obvious clinical signs of dehydration. Abdominal examination revealed mild distension and tenderness with a 5 × 10 cm intra-abdominal mass in the upper abdomen. Bowel sounds were exaggerated. Laboratory results showed a hemoglobin level of 8.7 g/dl and a Serum Creatinine of 1.2 mg/dl. X-ray abdomen showed air-fluid levels in the small bowel. Ultrasonography of the abdomen revealed a 15 × 18 cm hypoechoic mass in the center of the abdomen. These findings were suggestive of small bowel obstruction secondary to abdominal tuberculosis. The patient was resuscitated, and surgical exploration was planned. Exploration revealed a huge cyst with dimensions of 24 cm × 20 cm × 16 cm, arising from the retroperitoneum, projects into transverse mesocolon and displacing loops of the small intestine , Fig. 2.2.2A 45-year-old male presented to the outpatient clinic with abdominal distension and a central abdominal mass of three years duration. Mass is progressively increasing in size without any symptoms. His past medical history was non-contributory. General physical examination was unremarkable. Abdominal examination revealed a large, non-tender mass in the central abdomen which was extending to the right hypochondrium. All laboratory tests were normal. CT scan showed a huge retroperitoneal cyst occupying right hemiabdomen. The cyst was well circumscribed, unilocular and containing homogenous fluid in it. It was displacing right kidney . A provi2.3A 23-year-old female presented to the outpatient clinic with heaviness and epigastric discomfort of 2 years duration. She was under ultrasound surveillance in some other hospital. She denied any history of upper abdominal pain suggestive of acute pancreatitis. She underwent ultrasound-guided aspiration three times revealing two liters of clear fluid on each occasion. No record of biochemistry and cytology was available. All laboratory tests were normal including serum amylase level. On abdominal examination, there was fullness in the left hypochondrium. CT scan abdomen and pelvis showed the presence of a retroperitoneal cyst in the left hypochondrium, 15 × 10 × 12 cm in size with no mass effect on surrounding structures . It was 3The embryological and histopathological basis allows retroperitoneal cysts to be divided into the following categories: (1) Urogenital; (2) Meso-colic; 3) Cysts arising in cell inclusions; 4) Traumatic; 5) Parasitic; (6) Lymphatic [ Cysts ar Traumati ParasitiIts diagnosis remains elusive in terms of clinical symptomatology and poses a great challenge to the unsuspecting physician. About 50% of the cases usually present with vague symptoms like abdominal pain and distension . Other nRadiology serves as an indispensable tool to delineate these lesions and investigations including Computed Tomography are often required to define these lesions appropriately . It provSurgical intervention is required in cases where these cysts tend to become symptomatic and when inherent complications like perforation, infection, and malignancy are expected. Both trans-abdominal and extra-peritoneal approaches can be used to approach and excise the cyst depending on the location . A laparThe treatment of choice, however, remains complete excision with preservation of surrounding important structures it is related to . Other l4Retroperitoneal cysts in adults are an uncommon manifestation of abdominal pathology. The presentation is varied between asymptomatic to vague abdominal symptoms. The rarity catches the surgeon off guard at times. Radiology plays a crucial role in defining these lesions and complete surgical excision remains the cornerstone of treatment for symptomatic and complicated patients.5This study had a small number of cases. It was not a randomized clinical trial. This study was done in a single center.Note: Research work has been reported in line with the PROCESS criteria [criteria .No source of funding.Study was discussed in institutional review board of King Edward Medical University, Lahore and got approval from board with reference No.154 / RC/ KEMU Date: 01/ 01/2016.Informed consent from the patients, guardian (case 1) and institutional board has been taken.Danish Ali: Conceptualization, Methodology, Writing the original paper, Project administrationMuhammad Zeeshan Sarwar: Study concept, Review & editing the draft, Project administration, and Supervision.Fareeeha Manzoor: Data collection, Methadology, Data interpretation.Syed Asghar Naqi: Supervision.https://www.researchregistry.com/browse-the-registry.Research Registry UIN researchregistry4716.Danish Ali.Muhammad Zeeshan Sarwar.Not commissioned.None.


The aim of this article was to present our experience with CT-colonography evaluation of a case of giant colonic diverticulum. Despite the high prevalence of diverticular disease in the Western world, giant colonic diverticula are rare entities, with fewer than 200 cases reported in literature. A 63-year-old male presented to the radiology department with a history of abdominal pain over the hypogastrium and associated bowel disturbance worsening in the last few months.He received a positive faecal occult blood test for screening. An optical colonoscopy was performed, but turned out to be incomplete for the presence of a stricture at the level of the sigma, reporting the presence of numerous small diverticula in that tract. Routine blood tests revealed a mild microcytic hypochromic anaemia.Abdominal series consisting of posterioranteriorupright view and supine lateral view radiographs of the abdomen showed a large well-defined oval air-filled formation in the lower abdominal cavity . The remAccording to Italian guidelines for colorectal cancer screening , a CT colonography (CTC)  was performed: axial scans with sagittal and coronal reconstructed images showed a thick-walled, 7 cm cystic mass appeared to arise from the sigmoid colon .Circumferential thickening of sigmoid colon walls was noted, consistent with inflammatory changes in severe diverticular desease. Indeed, multiple small diverticula were reported originating from colonic walls, as well as surrounding mesenteric fat stranding.3D reconstructions on CTC dataset were obtained , showingTwo days later, for the purpose of a complete preoperative study, a CT after intravenous injection of non-ionic iodinated contrast material  was performed; the wall of the formation showed a weak delayed enhancement.The differential diagnosis includes sigmoid or cecal volvolus, pneumatosis cystoides intestinalis, intestinal duplication cyst, giant Meckel’s diverticulum and vesicoenteric fistula.Exclusion of alternative diagnoses can be reasonably made by CT scan, observing location and features of the cystic structure, first of all demonstrating its neck, a thin connection to the colonic lumen, well depicted by CTC.Swirling of the bowel and its mesentery is a typical CT finding in volvulus of the gastrointestinal tract, resulting in dilatation of intestinal loops caused by mechanic obstruction.1Pneumatosis cystoides intestinalis is an uncommon entity, in most cases an incidental finding. It represents an idiopathic cystic gas collection in the intestinal subserosa or mucosa.Enteric duplication cysts are unusual congenital abnormalities. They can occur anywhere along the digestive tract on the mesenteric side, involving more often the small intestine. They present as a simple fluid cyst with mass effect extending into the bowel lumen. Most duplication cysts manifest early, during the first year of life.Meckel’s diverticulum is the most frequent intestinal congenital anomaly, usually found within 100 cm of the ileocecal valve on the antimesenteric border of the ileum.Fistulous connections between the bladder and small or large bowel most often develop in the setting of inflammatory or neoplastic gastrointestinal or genitourinary diseases, leading to gas entering the bladder lumen.The patient underwent laparotomic sigmoid colectomy, with excision of the giant diverticulum and primary anastomosis, where a large air-filled cyst was found developing from the mesenteric border of the proximal sigmoid colon . Six weeDespite the high prevalence of diverticular desease in the Western world, giant colonic diverticula are rare entities, with fewer than 200 cases reported in literature.By definition, they are outpouchings of the colonic wall greater than 4 cm in size, the diameter can vary, with the largest described being 40 cm.2 The majority of GCD presents after the 6th decade of life.Approximately 90% of them develop from the sigmoid colon. They might be isolated, but in 85% of the cases, GCDs are associated with concomitant diverticulosis or diverticular disease.3 it remains difficult to establish any relation of cause and effect between GCD and cancer development.Clinical manifestation is variable, ranging from asymptomatic palpable abdominal mass to acute abdomen. Perforation and abscess formation are the most common encountered complications;4 classified GCD in three histological and pathogenetic subtypes. Type 1 diverticula  are pulsion diverticula, which gradually enlarge, representing outpouchings of the colonic mucosa and submucosa through the muscular layers of the wall, where the vessels pierce the muscularis (pseudodiverticula such as common colonic diverticula).McNutt et al5) are secondary to a subserosal perforation, leading to a walled off abscess cavity communicating with the bowel lumen and gradually enlarging. Their wall is composed of fibrous scar tissue, without a normal intestinal layer.Type 2 diverticula  most likely have a congenital origin and are formed by all the bowel layers.Type 3 .Ultrasound examination does not seem to be the radiological examination of choice for detecting a GCD: no significant abnormalities are usually detected.5 A contrast enema can demonstrate the communication of the diverticulum to the bowel lumen in almost 70% of cases.Diagnostic colonoscopy is frequently non-contributory: it is considered that the ostium of diverticular neck might be too tight to allow visualisation of the interior aspect.7Multidetector CT is the preferred imaging technique for GCD evaluation, as it is for other complications of colonic diverticular disease. CT demonstrates a smooth-walled gas-containing structure, rarely in association to marginal calcification. An air-fluid level can be sometimes observed. Coronal and sagittal multiplanar reformatted images are important for identifying the neck of the GCD, that is its connection to the bowel lumen; this finding is essential for a correct diagnosis.The wall of the cyst is usually smooth and regular, but if irregular or lobulated, then the possibility of an additional inflammatory or neoplastic process should be considered.8 it may be useful in GCD evaluation, but its role is difficult to establish, in relation to the rarity of this condition. In our single experience, no complications were encountered during the procedure; CTC offered a valid instrument to study the diverticulum’s anatomical features, providing as well a panoramic view of colon and remaining abdominal organs. Particularly, this technique proved to be useful to demonstrate the diverticular neck, well depicted thanks to colon distension also in 3D reconstructed images.The investigation of diverticular disease is a clear clinical situation where CTC can be safely proposed:9The prevalence of complications in GCD and their potential severity impose surgical treatment, even including asymptomatic GCD discovered fortuitously. This attitude can be supported by the low morbidity and mortality of elective colonic resections.By definition, giant colonic diverticula are outpouchings of the colonic wall greater than 4 cm in size.They are rare entities, in most cases associated to diverticular desease.The diagnosis is often challenging, because patient symptoms and laboratory findings are unspecific and overlap with other gastroenterological entities.Multi-detector computed tomography is the preferred imaging technique for their evaluation, essential for identifying the connection to bowel lumen.Written informed consent for the case to be published  was obtained from the patient(s) for publication of this case report, including accompanying images.


Background: Gastrointestinal malrotation arises from intrauterine events that occur early in the first trimester of gestation, and can result in a midgut volvulus that classically presents in the neonatal period with bilious emesis. Gastrointestinal malrotation can present clinically with symptoms such as chronic abdominal pain or bowel obstruction, or remain completely asymptomatic only to be discovered as an incidental finding much later in life during surgical exploration for other diseases. We sought to identify the prevalence of gastrointestinal malrotation in patients undergoing surgical exploration for hepatopancreatobiliary (HPB) malignancy and describe the operative considerations of these cases.Case Presentation: We performed a retrospective review of our prospectively acquired HPB surgery database from January 1, 2006, to December 1, 2013. We identified three cases of gastrointestinal malrotation out of a total of 1220 HPB cases reviewed, which represents 0.2%. We found two cases of gastrointestinal malrotation in the setting of pancreatic ductal adenocarcinoma and one case in the setting of cholangiocarcinoma. All three patients underwent exploratory laparotomy with resection of their respective primary tumors. We searched the English literature for cases of HPB malignancy in the setting of gastrointestinal malrotation.Conclusion: Our case series and review of the literature underscore the rarity and complexity of these cases. Up to 55% of individuals with gastrointestinal malrotation present within the first week of life and up to 80% present within the first month of life.1Abnormalities in gastrointestinal rotation occur fairly commonly in the general population with an incidence of 1/200 to 1/500 live births. They arise from events that occur 2 Concurrent congenital anomalies have been reported in up to 42% of patients with gastrointestinal malrotation, with the most common being duodenal webs/atresia (11%), Meckel's diverticulum (11%), and omphalocele (5%).3Symptoms of gastrointestinal malrotation in the neonate typically include either bilious emesis or other clinical and radiographic evidence of high-grade small-bowel obstruction.Although most cases of intestinal malrotation are discovered early, abnormalities of gastrointestinal rotation can persist into adulthood before becoming symptomatic. They may also be encountered incidentally at the time of workup for other diseases. Gastrointestinal malrotation in the adult can either present acutely with abdominal pain and emesis secondary to intestinal volvulus or it can present more insidiously with chronic vague abdominal pain and nausea typically from partial obstruction from intraperitoneal adhesions or “bands.”1 In this study, we report three cases of gastrointestinal malrotation that were discovered during operation for hepatopancreatobiliary (HPB) malignancy. Two of the patients had a concomitant pancreatic ductal adenocarcinoma (PDA) and one patient had a cholangiocarcinoma. We detail the clinical course of these patients, provide a discussion of the embryology that leads to such anomalies, and put this series in context with the English literature.Whether gastrointestinal malrotation in the adult predisposes patients to intestinal, pancreatic, or biliary tract malignancy is unclear. Campbell et al. reviewed all of the cases of gastrointestinal malrotation at one institution and found a high association of biliary tract anomalies in addition to one case of pancreatic cancer.We queried retrospectively our prospectively maintained HPB surgery database at the Thomas Jefferson University Hospital for cases of HPB malignancy with concurrent gastrointestinal malrotation from January 1, 2006 to December 1, 2013. We analyzed patient demographics, preoperative studies, operative variables, postoperative complications, postoperative hospital length of stay, adjuvant therapy, and survival. For the literature review, we searched the English literature for all cases of HPB malignancy with concurrent abnormalities of gastrointestinal rotation from 1957 to 2013. We performed a PubMed search of the terms “pancreatic ductal adenocarcinoma,” “cholangiocarcinoma,” and “gastrointestinal malrotation.”We identified three instances of patients who underwent surgical resection of HPB malignancy with concurrent gastrointestinal malrotation during the study period at our institution. Their cases are presented hereunder.A 63-year-old man presented to the emergency department with a 1-month history of intermittent nausea, chills, and jaundice. Preoperative laboratories revealed an elevated total bilirubin and alkaline phosphatase. Serum tumor marker analysis revealed a normal carcinoembryonic antigen (CEA) but an elevated CA 19-9. The patient underwent a magnetic resonance imaging (MRI)/magnetic resonance cholangiopancreatography that revealed thickening of the proximal common bile duct with proximal biliary dilatation and an absence of distant disease. Incidentally noted on the MRI was that the third portion of the duodenum did not cross to the left of the superior mesenteric artery (SMA) and superior mesenteric vein (SMV), and the majority of the small intestine lies in the patient's right abdomen, whereas the large intestine lies in the left. Also, the SMA lies to the right of the SMV consistent with gastrointestinal malrotation . The patA 47-year-old man presented to his primary care physician with a 3-month history of intermittent epigastric abdominal pain, nausea, and vomiting. Routine laboratory analysis revealed normal liver function. The patient underwent an abdominal computed tomography (CT) scan, which revealed a 4.2 × 4.1 × 4 cm mass in the head and uncinate process of the pancreas. The scan also showed that the patient's entire colon was on the left side of the abdomen and the small bowel to the right . Tumor mA 67-year-old Caucasian woman presented to her primary care physician with a 1-year history of epigastric abdominal pain, occasional nausea, and emesis, and an unintentional 10 pound weight loss. An abdominal CT scan showed a 4.5 × 4.1 cm periampullary mass arising from the pancreas without evidence of metastatic disease. Interestingly, the CT scan also showed the colon to be in a dorsal position in relation to the SMA . Laborat4–7 There were five pancreatic tumors , two cholangiocarcinomas, and one ampullary adenocarcinoma. Four out of the eight cases involved classic gastrointestinal malrotation where the entire small bowel lies to the right of the superior mesenteric vessels and the colon to the left. In one case, the patient had both classic gastrointestinal malrotation and situs ambiguous and so the entire colon was to the right of the superior mesenteric vessels and the entire small bowel was to the left. Two of the cases had a reversed rotation where the colon was a retroperitoneal structure and lies posterior to the superior mesenteric vessels. Finally, one case had an incomplete rotation where the ascending colon was midline and the duodenojejunal junction was to the right of the midline with some of the distal small bowel present in the left abdomen.Gastrointestinal malrotation occurs as a failure in the process of normal gastrointestinal rotation and fixation during intrauterine development. Although typically discovered early in a patient's life, malrotation can sometimes quietly persist into adulthood. If symptomatic, adult patients may present with vague symptoms of chronic abdominal pain or with episodes of partial bowel obstruction. At times, adult patients may even present acutely with intestinal volvulus. Although rare, this condition requires clinicians and surgeons to be aware of the unique underlying embryology and anatomic considerations to treat these patients effectively. As our report of these three cases of gastrointestinal malrotation found in the setting of HPB tumors demonstrates, special considerations are required when treating these complex patients.8 Frazer and Robbins then provided an extensive review of the subject, dividing development into three stages in 1915.9 Dott published a review of three cases of various anomalies of intestinal rotation and reviewed the most common rotational anomalies that can arise during each stage of fetal development.10Mall at Johns Hopkins first described the complex embryogenesis of gastrointestinal development by using embryo models at different stages of development in 1898.11 At this time, rapid growth of the midgut loop and liver leads to a loss of abdominal domain with a consequent extrusion of the midgut loop into the root of the umbilical cord as a temporary and physiological hernia. The SMA runs from the dorsal aorta between the two segments of the midgut loop to the apex. The vessel divides the midgut loop into a prearterial and postarterial segment with their corresponding mesenteries. At this stage, the loop lies in the sagittal plane with the prearterial segment being anterior and postarterial segment being posterior.12 The cecoappendiceal bud appears on the postarterial segment during the fifth week. Whereas in the umbilical cord, the prearterial segment grows faster and longer than the postarterial segment, thereby acquiring a disproportionately longer mesentery. Meanwhile the cecum, ascending and transverse colon develop from the postarterial segment. Derangement of the first stage of gastrointestinal development mainly leads to failure of the viscera to return to the abdominal cavity, which leads to an omphalocele.2The first stage of gastrointestinal development starts at approximately the fourth week of gestation .11 At th11 The midgut loop returns to the abdominal cavity through the umbilical orifice. The prearterial segment enters first. The small intestine enters the abdomen on the right side of the superior mesenteric vessels and passes to the left side of the abdomen behind the mesenteric vessels.12 This movement of the small bowel pushes the hindgut and its mesentery to the left and superior. This will become the transverse and descending colon. After the cecum reenters the abdominal cavity, further elongation of the colon forces it down to the right lower quadrant. The second stage sees a 270° counterclockwise turn around the SMA, which causes the duodenum to cross behind the superior mesenteric vessels near their origin and the colon to cross the same point anteriorly.10The second stage of gastrointestinal rotation occurs at the beginning of the 10th week of development and consists of the rotation and reentry of the gastrointestinal tract back into the abdomen .9,11 The2 The jejunum and ileum consequently occupy the right lower quadrant. The ileum then enters the cecum in either the midline or the left lower quadrant that brings the duodenum and cecum to rest anterior and superior to the SMA, which leads to a very narrow mesenteric base as in Cases 1 and 2. This is classically characterized as true gastrointestinal malrotation and predisposes to the development of a midgut volvulus. Finally, reversed rotation results from a 90° clockwise rotation that brings the colon in a position dorsal to the SMA as in Case 3.10One major derangement of the second stage of gastrointestinal development can be failure of rotation of the midgut after returning to the abdominal cavity. This results in the small intestine lying to the right of the midline with the duodenum descending from its normally fixed position to the right of the SMA.11 Both the cecum and duodenum come to rest by fusion of their mesenteries to the posterior parietal peritoneum. Abnormalities of the third stage of intestinal rotation can lead to an excessively floppy cecum, which can predispose patients to the development of a cecal bascule or cecal volvulus.The third stage of intestinal rotation is characterized by the final elongation of the colon causing descent and fixation of the cecum .11 Both 13 The incidence of abnormal visceral rotation in the setting of HPB malignancy has not yet been reported. We found the prevalence of abnormalities of gastrointestinal rotation to be 0.2% in the setting of HPB tumors at our own institution.Previous reports have stated that abnormal visceral rotation occurs in less than 0.2% of patients undergoing exploration for bowel obstruction.Patients who have abnormal gastrointestinal rotation with an HPB cancer can present in a manner similar to patients with HPB malignancy alone. Vague abdominal discomfort accompanied by symptoms of biliary and visceral obstruction typically existed for more than several months in our patients. The abnormal visceral anatomy was identified preoperatively in all three of our patients. Abnormal abdominal visceral anatomy has an impact on the operation both in the challenges that it poses in identifying abdominal vasculature during oncological resection and in reconstruction after resection. Case 3 had a reversed rotation with a retroperitoneal colon and the duodenum anterior to the superior mesenteric vessels. We usually bring the jejunal limb up in a retrocolic position . The retWe and others have shown that resection for HPB malignancy in the setting of abnormal visceral rotation can be performed safely. In all of the cases, the abnormal intestinal rotation was discovered on preoperative imaging. There are hallmark features on CT or MRI that the HPB surgeon should look for, so as to be prepared for these rotational abnormalities. A classic malrotation exists when the small intestine is in a position to the right of the midline and the duodenum descending from its normally fixed position to the right of the SMA. A reversed rotation exists when the colon is in a position dorsal to the SMA. Great care and consideration must go into operative planning and technique for a safe dissection and reconstruction when these abnormalities of gastrointestinal rotation are found on preoperative imaging.In summary, our case series demonstrates that with an adequate understanding of typical anatomic abnormalities of gastrointestinal malrotation, procedures for HPB malignancies can be safely performed with appropriate adjustments in the operative technique.


Intraosseous lipomas are very uncommon, benign primary bone lesions with an incidence of <1%. The overwhelming majority occur within the intramedullary canal. We present an uncommon intracortical intraosseous lipoma with 3 T MRI findings to help differentiate this from other differential diagnoses. Intraosseous lipomas are very uncommon, benign primary bone lesions with an incidence of <1%. The overwhelming majority occur within the intramedullary canal. We present an uncommon intracortical intraosseous lipoma with 3 T MRI findings to help differentiate this from other differential diagnoses.A fit and well 57-year-old male with no previous history of recent or past trauma presented with an incidental painless thigh mass and was referred to our tertiary referral sarcoma centre for assessment. Initial plain radiographic assessment  delineatIn view of the characteristic findings and the absence of any aggressive features, no needle biopsy was required for this patient, which would have been the usual management pathway for the assessment of an unusual bone lesion. As this was the first presentation, a single follow-up MRI examination locally at 6 months was recommended at the bone tumour multidisciplinary team meeting. Unfortunately, it has not been possible to contact the patient since his imaging review and no follow-up imaging is available at present.1 that usually occur within the fourth and fifth decades of life.3 They can be mildly painful but are predominantly asymptomatic and found incidentally. Intraosseous lipomas often occur within the femur, usually proximally at an intertrochanteric or subtrochanteric position, with the tibia, fibula and calcaneus being the other common locations. The overwhelming majority of cases describe lesions occurring in the intramedullary canal, with only a few reported cases demonstrating an intracortical location,6 as shown in this case.Intraosseous lipomas are very uncommon, benign primary bone lesions with an incidence of <1%3 but this has not been widely reported in the intracortical variety.6 The MRI also demonstrates no continuity of the lesion with the intramedullary canal marrow, helping differentiate it from potential post-traumatic entry of marrow into the cortex and other healed lesions such as a fibrous cortical defect or infection.Plain radiography demonstrates a well-defined intracortical lesion of low internal density with no evidence of previous injury or aggressive features. Subsequent MRI examination confirms a lesion confined to an intracortical location, with comparable cortical thickness both deep and superficial to the lesion, in contrast to a periosteal lipoma that would present as a mass adjacent to bone. The MRI also confirms the completely homogeneous fat signal of the lesion, which is helpful in differentiating it from intracortical fibrous lesions such as osteofibrous dysplasia that would typically demonstrate more heterogeneous signal from their non-fatty components. The more common intramedullary intraosseous lipoma can demonstrate additional imaging characteristics, including calcification and cystic change,In keeping with the few cases reported in the literature, our case did not demonstrate any aggressive features, with absence of non-lipomatous elements, periosteal reaction and associated extraosseous mass. For this patient, recognition of the homogeneous and uniform fat signal characteristics within a painless, non-aggressive lesion to demonstrate an intracortical intraosseous lipoma resulted in the patient avoiding unnecessary needle biopsy or surgical resection and the associated morbidity.Intraosseous lipomas are very uncommon, benign primary bone lesions with an incidence of <1%.The vast majority occur in the intramedullary canal but, as in this case, rarely, it can be present in an intracortical location.completely lipomatous signal characteristicsintracortical location without any communication with normal intramedullary bone marrowcomparable cortical thickness, both superficial and deep, around the lesionnon-aggressive imaging features.Features alluding to an intracortical intraosseous lipoma include:


Aggressive angiomyxoma is a very rare benign tumor for male population with fewer than 50 cases reported since the description of this tumor. Most documented cases of aggressive angiomyxomas were found in genital, perineal, and pelvic regions in women of child bearing age. We report a case of a massive (> 20 cm) aggressive angiomyxomas in a man who presented with perineal swellings. Macroscopically the mass was highly vascular and lobulated with high similarity to plexiform neurofibroma. Microscopic examination revealed a hypocellular tumor comprising bland oval and spindle-shaped cells along with vessels of varying calibre. The accompanying stroma was myxocollagenous. Immunohistochemical staining showed CD34 and focal estrogen receptors positivity and negative staining for S100, actin, desmin, and progesterone receptors. The histologic and immunohistochemical features favored the diagnosis of aggressive angiomyxoma. Despite the rarity of such tumor in the male population, aggressive angiomyxoma should be considered in the differential diagnosis when encountering chronic para-perineal lesions. Aggressive angiomyxoma is a rare, locally infiltrative mesenchymal tumor occurring mostly in women of productive age, but it is occasionally reported in men. It carries a high risk for local relapse, hence the need for differentiating it from the other mesenchymal perineal tumors. We report a case of a massive aggressive angiomyxoma extending from retro-pubic area anteriorly to buttock posteriorly.A 46-year-old man presented to our clinics with a mass located to his left buttock. He observed the gradual enlargement of this lesion for 2 years with no eliciting event. The lesion did not cause any neurological, urological, or colorectal symptoms. His main complaints were discomfort while sitting and embarrassment for cosmetic reason. The patient was a nonsmoker, nonalcohol drinker, and otherwise healthy with no family history of any cancer. Positive surgical history included a bilateral hip replacement 10 years ago post motor-vehicle accident complicated by multiple episodes of seroma. Physical examination showed a large left buttock mass with overlying intact skin and several neighboring scars from the previous hip surgery. It was soft, compressible, and nontender in consistency. Magnetic resonance imaging (MRI) from another hospital was reported as a large (20 × 10 × 10 cm) solid, lobulated lesion, spanning from retro-pubic anteriorly to left buttock posteriorly, well confined to subperitoneal space  have been reported in women, but none in men so far.4 Patients often present with nonspecific symptoms that are frequently misdiagnosed with more common entities, such as a Bartholin cyst in females, lipoma, or hernia. Therefore, it is not uncommon for delayed presentation and most aggressive angiomyxoma is reported large (> 10 cm). In our case, the patient suspected the lesion was a complication of seroma after his hip replacement surgery and thus allowed its growth to 23 cm in diameter. Preoperative diagnosis is often difficult because of the rarity of these neoplasms and lack of specific features on imaging studies.5 Hence, most cases are diagnosed by histopathological examination after surgical excision.5Aggressive angiomyxoma is a rare mesenchymal tumor that most commonly arises in the vulvovaginal region, perineum, and pelvis of women. The tumor was first described by Steeper and Rosai16 Immunohistochemically, the majority of reported cases show positivity for desmin in the myoid bundles and/or stromal cells, whereas actins and CD34 may be variably positive.7 In female patients, the tumor cells are characteristically positive for estrogen and progesterone receptors, suggesting a hormonal role in the development of the tumor and some therapeutic advantage using hormone agonists has been proposed.3 The significance of association with female hormone receptor still remains unclear. The tumor did not stain consistently positive with these receptors reported in male patients,4 but might occur in some male series.3Histologic examination reveals a hypocellular and highly vascular tumor with a myxoid stroma containing cytologically bland stellate or spindled cells. Immunohistochemical stains demonstrate S100, desmin, and progesterone receptor negative with CD34 and focal estrogen receptor positive. Histologically, the hallmark of aggressive angiomyxoma is vessels of varying calibre haphazardly scattered throughout the parenchyma.6 Interestingly, a review of 111 cases of aggressive angiomyxoma determining if radical resection is necessary by comparing patients’ risk of recurrence based on margin status. The data showed no statistical difference in remaining disease-free between groups of patients with positive and negative resection margin results .8 Even though complete surgical resection is the desired goal, incomplete removal is acceptable when significant operative morbidity is anticipated or when preservation of fertility is a concern.8 Hormonal therapy is recommended in the setting of neoadjuvant therapy after incomplete surgical resection.7 Recurrence mostly occurs within 5 years after complete surgical resection.6 Owing to the clear demarcation but proximity of rectum and anus, wide excision was not possible in this patient. No adjuvant or neoadjuvant therapy was offered in this case. However, considering the risk of recurrence careful follow-up and imaging modalities have been proposed.The standard treatment for aggressive angiomyxoma is surgical excision, although achieving negative resection margins is difficult because of the infiltrative nature of the tumor and the often absence of a defined capsule.Aggressive angiomyxomas should be included in the differential diagnosis of large para-perineal tumors. This case signifies the extreme rarity of the tumor in males and highlights its typical histologic findings and its adequate management considering its locally infiltrative nature.


Perineal groove is a well-defined clinical entity that belongs to a broader group of anorectal malformations. It is characterized by a non-epithelialized mucous membrane that appears as an erythematous sulcus in the perineal midline, extending from the posterior vaginal fourchette to the anterior anal orifice. The defect is gradually cicatrized, unless there are complications like infection, defecation disorders, trauma, and bleeding. The differential diagnosis includes several other conditions like trauma, infection, irritant dermatitis, lichen sclerosis, and ulcerated hemangioma. Since it is a rare malformation, it is often misdiagnosed and its presence often elicits unnecessary diagnostic workup and intervention. In this respect, neonatologists, dermatologists, or pediatric surgeons may under- or overestimate it. We report four cases of perineal groove out of 2250 live births at a Greek Maternity Hospital between September 2016 and April 2019. The “high” incidence of perineal groove cases in our Department allowed us to familiarize with this rare defect and minimize our clinical interventions. Perineal groove is a well-defined but rare entity that belongs to a broader group of anorectal malformations. It has rarely been described in the literature, with less than 30 cases published over the last four decades ,2. As thSigned written consent forms to publish their data and photographs were obtained from all the patients’ parents or legal guardians.+6 weeks and a post-natal follow-up ECHO at 40 days of life, with no other abnormal findings. On the first physical examination, a midline defect was noticed on the newborn’s perineum, which formed an erythematous sulcus resembling a rupture that expanded about 1 cm from the posterior vaginal fourchette to the 12 o’clock position of the anterior anal opening. Both external genitalia and the anal orifice were intact and normally positioned. No active blood or passage of meconium via the vaginal opening was observed. The obstetrician placed four absorbable sutures in the malformed perineum, as he thought the defect represented tear of the area, and the newborn was transferred to the neonatal unit for prompt pediatric surgical evaluation. Frequent and meticulous hygiene of the affected area was maintained and a course of intravenous antibiotics was administered. The newborn remained stable throughout hospitalization with no evidence of infection. Parents refused to attend follow-up outpatient clinic , and therefore we followed-up the case by frequent phone interviews; according to information provided by them, the infant had a full cicatrization of the perineal defect, with no defecation issues or local infection incidences.A full-term female newborn with a birth weight (BW) of 3440 g was born at 39 weeks of gestation via cesarean section (CS) for breech presentation from a 37-year-old mother Gravida 3 Para 3 (G3P3); Apgar score was 6 and 10 at 1st and 5th minute of life, respectively. Baby was apneic at birth and received five inflation breaths, followed by good response with vigorous crying, good muscle tone and heart rate. The pregnancy was uneventful apart from a single echogenic intracardiac focus that was detected on the second trimester anatomy scan and was confirmed, both by fetal echocardiography (ECHO) at 22A full-term female newborn with a BW of 3540 g was born at 39 weeks of gestation via CS for breech presentation from a 35-year-old mother G1P1; Apgar score was 9 and 10 at 1st and 5th minute, respectively. On the ultrasound of the second trimester’s anatomy scan, mildly dilated lateral ventricles were detected, a finding that was confirmed by a cranial ultrasound on the 4th day of life. The first physical examination revealed a mildly erythematous sulcus extending from the posterior vaginal fourchette to the anterior anal orifice . The dia+4 weeks of gestation by vacuum-assisted vaginal delivery, due to nuchal cord, from a 31-year-old mother G1P1; Apgar score was 8 and 9 at the 1st and 5th minute, respectively. The pregnancy was uneventful apart from repeated detection of positive IgM antibodies for cytomegalovirus (CMV) from 12 weeks onwards. The neonate had a normal cranial ultrasound on the 2nd day of life and urine PCR (polymerase chain reaction) for CMV was negative. At birth, the presence of a mucous membrane in the midline of perineum from the posterior vaginal fourchette to the anterior anal orifice was noted. Pediatric surgical consultation confirmed the diagnosis of perineal groove. As the streak appearance of the anal canal was missing at the 12 o’clock position due to the presence of mucosa, anal stimulation was performed, showing a concentric contraction of the anal sphincter which was intact. The vaginal opening was also normal with no fistulae (A full-term female neonate with a BW of 3500 g was born at 39fistulae . The neoA term female neonate with a BW of 2930 g was born at 38 weeks of gestation by CS, due to fetopelvic disproportion, from a 31-year-old mother G1P1; Apgar score was 9 and 10 at the 1st and 5th minute, respectively. The pregnancy was uneventful. The first physical examination revealed an erythematous mucous membrane extending from the posterior vaginal fourchette to the anterior anal orifice . The defPerineal groove is a rare congenital malformation of the perineum characterized by a non-epithelialized mucous membrane that appears as a wet sulcus in the perineal midline, extending from the posterior vaginal fourchette to the anterior anal orifice . The lesThe diagnosis is set on clinical grounds, by the presence of the aforementioned lesion, as it is otherwise asymptomatic . It is uMisdiagnosis is a common phenomenon in these cases, since the differential diagnosis includes several other conditions like infection, irritant dermatitis, lichen sclerosis, ulcerated hemangioma, perianal pyramidal protrusion, and mainly trauma . Biopsy Concerning its embryological origin, the groove’s location in the place of the perineal raphe indicates that it derives from abnormal midline development, which even explains the short distance between the anus and the genitalia, or the presence of bifid scrotum and hypospadias noticed in a male newborn . AccordiTypically, perineal groove remains stable and resolves spontaneously within the 1st year of life, with complete epithelization of the sulcus between 1 and 2 years of age [In conclusion, within a 31-month period we had four cases of perineal groove in our perinatal institute, all in female neonates. In all cases, the medical history of the parturients, along with the perinatal history, were not associated with the respective malformation. Due to the rarity of the condition, neonatologists or pediatric surgeons may under- or overestimate it. This may result—if underestimated—to a higher risk of infection due to defecation disorders and injury or hemorrhage of the groove with minimal handling. On the contrary, with aggressive surgical treatment, it may lead to cicatrization complications. The “high” incidence of perineal groove cases in our Department allowed us to familiarize with this rare entity and minimize our clinical interventions. With appropriate counseling and follow-up, our neonates’ clinical course was uncomplicated.