Primary neuroendocrine tumors (NETs) of the retroperitoneum are extremely rare. The purpose of this case report is to highlight the unusual growth pattern and radiologic features of primary retroperitoneal NETs.A 46-year-old woman was found to have a retroperitoneal cystic and solid mass during a physical checkup.The mass was mainly multiseptated in the cystic portion and had a bead-like, lobulated appearance. The solid portion showed restricted diffusion on diffusion-weighted imaging and obvious homogeneous enhancement. The cystic portion showed ring-like and septal enhancement. The patient was diagnosed with a grade 2 (G2) NET of the retroperitoneum after surgery.The patient underwent resection of the large retroperitoneal tumor.The patient returned 20 months later with tumor recurrence in the retroperitoneum. She was enrolled in a clinical trial for sulfatinib, and the mass was considerably reduced in size after 4 months. During a nearly 1.5-year follow-up, the mass gradually became slightly enlarged. The expression of somatostatin receptor 2 (SSTR2) was detected, and somatuline was administered as the current treatment.When a retroperitoneal mass presents as a well-defined cystic or solid hypervascular mass with a fibrous capsule, a primary retroperitoneal NET should be considered in the differential diagnosis. Small intestinal NETs are the most common NETs in the gastrointestinal tract, followed by pancreatic NETs. NETs found in the retroperitoneum are most often metastatic, and primary NETs of the retroperitoneum are extremely rare. We report a case of a middle-aged woman who was surgically confirmed to have a primary retroperitoneal NET, and the radiologic features of the NET were observed on computed tomography (CT) and magnetic resonance (MR) imaging. The purpose of this case report is to highlight the unusual growth patterns and radiologic features of primary retroperitoneal NETs. We also present a comprehensive review and summary of all reported cases in the pertinent English-language literature.Neuroendocrine tumors (NETs) are heterogeneous in nature and have varying growth rates.1.1This retrospective case report was approved by the ethics committee of The Second Affiliated Hospital, Zhejiang University School of Medicine. Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for editorial review.2A 46-year-old woman was admitted to The Second Affiliated Hospital, Zhejiang University School of Medicine, due to the discovery of a lesion during a physical checkup at a local hospital. Ultrasonography and CT revealed a cystic and solid lesion in the retroperitoneum. She had no prior surgeries, and her medical history and family history could not explain the mass.On admission, the results of the physical examination were unremarkable. Laboratory examination results were significant for hemoglobin (75 g/L) and D-dimer ). Other laboratory tests, including those for tumor markers carbohydrate antigen 199 (CA199), carbohydrate antigen 125 (CA125), carcinoembryonic antigen (CEA), and alpha fetoprotein (AFP), were normal.After admission, abdominal CT and MR imaging were performed. Abdominal CT revealed a retroperitoneal cystic and solid mass located near the midline. The mass was adjacent to the lower left liver and partly wrapped around the pancreas. The mass was mainly multiseptated in the cystic portion and had a bead-like, lobulated appearance. The solid portion showed obvious homogeneous enhancement, with an increase from 42 Hounsfield units (HU) to 73 HU after contrast injection. The cystic portion showed ring-like and septal enhancement Fig. A and B. Seven days after admission, the patient underwent resection of the large retroperitoneal tumor . An approximately 8-cm × 7-cm polycystic mass in the retroperitoneum was resected. During surgery, the retroperitoneal tumor was found to be located adjacent to the head of the pancreas, with a distinct border. The integrity of the pancreas was preserved. The solid portion of the mass was 3 cm in diameter and had moderate hardness. The mass wrapped around the left gastric artery, and the remainder had a well-defined margin. After removal of the tumor, the omentum, mesentery, diaphragm, retroperitoneum and pelvic cavity were explored by palpation, and no tumor-like masses were discovered.The resected mass was mainly cystic and solid. Cross-sections revealed that the mass had a “fish flesh” appearance and was soft. Microscopic examination revealed that the retroperitoneal mass was composed of oval and round epithelial cells with a papillary structure Fig. A. Fewer  The mass was considerably reduced in size after 4 months  NETs have now been officially defined as a subgroup of pancreatic high-grade (G3) NETs. Based on this classification, this patient was diagnosed with a well-differentiated, intermediate-grade (G2) NET.NETs constitute a group of tumors that originate from diffuse neuroendocrine cells throughout the body and are characterized by a wide spectrum of clinical manifestations. The classification proposed by the World Health Organization (WHO) was updated in 2010 to include the tumor grade and differentiation in the criteria. NETs are divided into well-differentiated, low-grade ; well-differentiated, intermediate-grade ; and poorly differentiated, high-grade  lesions.,8–13 . A slight female predominance was noted. These NETs were asymptomatic, or the clinical symptoms were nonspecific , and the symptoms resulted from the effect of the mass on adjacent structures. All patients had no manifestations of carcinoid syndrome, and no clinical evidence of hormone production (nonfunctioning tumors) was identified. Three patients (including our patient) had tumors that evolved asymptomatically and were incidentally diagnosed on an abdominal imaging examination. Kwon, and Ye et al) had tumors that were detectable on CT, and our patient underwent both CT and MR imaging. On imaging, the retroperitoneal NETs typically appeared as well-defined, hypervascular masses due to their rich capillary networks. Small tumors tended to be round or ovoid homogeneous solid masses. Large tumors were commonly lobulated and heterogeneous with cystic, hemorrhagic, and necrotic areas. Calcification was not observed. Most cases had a fibrous capsule and showed enhanced capsules in post-enhancement images from delayed phase imaging. These tumors were mainly cystic and typically had a hypervascular rim. The enhancement of the tumors was homogeneous, ring-like, or heterogeneous. In our patient, the mass was mainly multiseptated in the cystic portion and had an irregular shape with a bead-like, lobulated appearance, which was quite different from the other cases. Retroperitoneal NETs were classified as G1-G2. The Ki-67 and mitotic indexes were not provided in some early cases; thus, those cases could not be accurately classified. No cases were associated with lymphatic, hepatic, or other metastases. The retroperitoneal NETs were sporadic, and no cases were associated with any familial syndromes.Most retroperitoneal NETs were relatively large in size, with a median size of 8 cm (range 4–21 cm) at the time of detection. The retroperitoneum provides a large space for tumors to grow; thus, the tumors were not detected until they were very large. Only 3 patients , mediastinal and upper abdominal retroperitoneal lymphadenopathies (LAPs), and renal angiomyolipomas are common. A lymphangioleiomyoma is a cystic mass found in the lymphatic system. The signal intensities of the center area on T1-weighted imaging and T2-weighted imaging are homogeneous and similar to those of ascites. The peripheral region is immediately enhanced, and homogeneous delayed enhancement of the entire mass is observed. PRMC is an extremely rare neoplasm with a female predilection that is found almost exclusively in the lateral retroperitoneal spaces. This tumor is a retroperitoneal cystic lesion with solid mural nodules that show progressive enhancement on enhanced CT. Our patient was a 46-year-old woman without any familial syndromes. The tumor contained a solid portion and was mainly multiseptated in the cystic portion, with a bead-like, lobulated appearance. The tumor was located in the middle-upper central retroperitoneum. Therefore, the 3 aforementioned differential diagnoses were not supported.The differential diagnosis of a retroperitoneal cystic and solid mass includes plexiform neurofibroma, lymphangiomyomatosis (LAM) and primary retroperitoneal mucinous cystadenocarcinoma (PRMC). Plexiform neurofibromas are almost exclusively observed in neurofibromatosis type 1 (NF l). These masses are typically bilateral, symmetric, low-attenuation masses, and the lumbosacral plexus is the most common site in the retroperitoneum.–20 The previous case reports indicated that most retroperitoneal NETs grow slowly and have a limited risk for local invasion and metastasis. In this case, metastasis was not observed, and the patient was treated with surgical resection without postoperative adjunctive therapy. This patient experienced recurrence in the retroperitoneum 20 months later based on CT scans, and the mass gradually became enlarged with sulfatinib treatment. SSTR2 expession was detected in this patient, and somatuline  was used as a palliative treatment option. Recent research has demonstrated that SSAs exert antiproliferative effects and inhibit tumor growth by binding the SSTR2. SSA treatment may prolong both overall and progression-free survival in patients with NETs, and multiple trials have demonstrated high rates of disease stabilization upon treatment with SSAs. However, this patient showed recurrence in the retroperitoneum, which was not observed in previous cases, indicating that retroperitoneal NETs may have a risk for recurrence and metastasis. The prognosis of retroperitoneal NETs is relatively good, but the role of postoperative adjunctive therapy and the therapeutic options for recurrent NETs are still undetermined.Surgical resection is the first-line treatment for primary NETs and is potentially curative, even in cases of metastatic disease, regardless of the NET origin if at least 90% of the tumor can be successfully removed.4We present an extremely rare case of a primary NET that arose from the retroperitoneum. When a retroperitoneal mass presents as a well-defined cystic and/or solid hypervascular mass with a fibrous capsule, a primary retroperitoneal NET should be considered in the differential diagnosis.We thank Xin-Feng Yu for his excellent help in the preparation of the manuscript.Conceptualization: Dan Shi, Guo-Qiu Dong, Ri-Sheng Yu.Data curation: Dan Shi, Guo-Qiu Dong, Ke-Ren Shen.Formal analysis: Ke-Ren Shen, Yao Pan, Shu-Mei Wei.Methodology: Dan Shi, Ying Chen, Ri-Sheng Yu.Resources: Dan Shi, Guo-Qiu Dong, Ke-Ren Shen, Yao Pan, Shu-Mei Wei, Ying Chen, Ri-Sheng Yu.Supervision: Ying Chen, Ri-Sheng Yu.Writing – original draft: Dan Shi.Writing – review & editing: Shu-Mei Wei, Ri-Sheng Yu.


We present a typical case of dermatofibrosarcoma protuberans(DFSP) with a complaint of a painful and various recurrence, make a review of the most important aspects. This is a rare tumor and we call special attention to the fact that its recurrence is extremely frequent, so there is absolute need to observe these patients periodically after surgery. We present a typical case of dermatofibrosarcoma protuberans (DFSP) with a complaint of a painful and various recurrence, make a review of the most important aspects. This is a rare tumor and we call special attention to the fact that its recurrence is extremely frequent, so there is absolute need to observe these patients periodically after surgery. It is a relatively uncommon soft tissue neoplasm of fibroblast origin. Slow growth and low aggressiveness are its characteristics. Its incidence is 0.8‐4.1 per million people annually.23. The patient underwent mass resection. The pathologic diagnosis of the extracted specimen was nodular fasciitis. The second recurrence was in 2016, the patient returned with a complaint of a painful mass in the right axillary region and underwent surgery—the pathologic result of the resected sample diagnosed as benign soft tissue tumor most compatible with myoxid fibroma. The following year, the patient presented with a similar painful mass in the same area where the ultrasound of the right axillary region had shown an isoechoic mass of 41.39 mm in the subcutaneous area. The patient underwent surgery again, and the removed mass sent for pathologic examination. The report shows myxoid fibroma recurrence.We present the case of a 22‐year‐old Caucasian patient who had referred to an outpatient clinic with a complaint of a painful and recurrent mass in the right axillary region. First, the patient reported an increasing size of the tumor during the preceding 6 months and denied any recent weight loss, fever, night sweats, or chills. There was neither personal nor familial history of malignancy but he had a lot of times of recurrency in the first refer. The patient underwent mass resection, but he does not have any pathologic report. The first recurrency was in 2013, the patient presented with a recurrence of the tumor in the previous area. The mass has a cystic heterogeneous of 29.20.30 mm and a volume of 9.5 cmEventually,In 2019, patients presenting with painful recurrence has the same complaint and also in the same area as the previous ultrasound, which displays a hypoechoic area of approximately 9.6.8 mm with a depth of 8 mm. Also, a lymph node of hypoechoic density (4.5 × 8 mm) observed adjacent to the lesion. Highly suspicious two hypoechoic nodules of 17 × 8 and 6 × 24 mm were seen in the right axillary region, suggesting a recurrent tumor invasion to the adjacent nodal lymph. Axillary tumor of the patient underwent FNA. The extracted specimens examined for pathology and IHC and according to pathologic findings and IHC, DFSP diagnosis made for the patient. The patient underwent mass resection for the last time, and the resected mass sent for pathological examination. The diagnosis results in myxoid form of DFSP. Also, the extracted specimen re‐examined by IHC where the tumor cells tested positive for SMA and B‐carenin, a part of tumor is positive for CD34 negative for S100, thereby setting the diagnosis of DFSP tumor markers were confirmed again and finally, the diagnosis approved as fibrosarcoma originating from DFSP , computed tomography (CT), and ultrasound to be useful in preoperative evaluations.Typical findings in ultrasound studies show hypoechoic or mixed hyperechoic, with mostly well‐defined margins or irregularities, but our ultrasound findings initially show an isoechoic mass with defined margins and subsequent recurrence of the lesions presented with hypoechoic defined margins mass.The gold standard to diagnose this type of neoplasm is a deep subcutaneous punch biopsy or incisional biopsy because the surface biopsy of the lesion cannot differentiate sufficiently from benign skin lesions. Re‐biopsy is recommended if our clinical suspicion was strong and unequivocal primary biopsy was performed although this is associated with a risk of tumor seeding.Histopathological studies usually show relatively uniform densely grouped fusiform cells, with elongated nuclei and without significant cytologic atypia or pleomorphism in characteristic storiform arrangement.9,20,21. In our case, tumor cells were positive for SMA and B‐catenin and part of tumor was positive for CD34 and negative for S100. As a result, the findings strongly favor DFSP.Immunohistochemically, tumor cells stain for vimentin, CD34, apolipoprotein D, nestin, and maybe for EMA. Desmin, S100 protein, FXIIIa, stromelysin III, HMGA1 & 2, tenascin, D2‐40, CD163, and keratins are negative. In myoid nodules, tumor cells stain for SMA. Fibro sarcomatous DFSP may exhibit a loss of CD34 and enhancing expression of TP53The American Joint Committee did not publish a TNM staging system since we used a system presented by Ugurel et al to evaluate the situation includes stage‐I—localized disease, stage‐II—lymph node metastasis, and stage‐III—distal metastasis.Our patient had a stage II disease because of lymph node metastasis and no distant metastasis.The standard treatment of localized masses is extensive local surgical resection with 2‐3 cm surgical margins and three‐dimensional resection of skin, subcutaneous tissue, and underlying fascia.The local recurrence rate tends to decrease with the increase in surgical margins. An alternative to extensive surgical resection is Mohs micrographic surgery which has a therapeutic success rate of 93%‐100%.Chemotherapy is not the standard treatment for this disease, but recent studies with Imatinib mesylate as Neoadjuvant have recommended in advanced and recurrent DFSP localized tumors. The recommended dose is 800 mg orally, which has also been approved by the FDA.After surgery, we recommended the patient to chemotherapy because of the recurrence and large volume of the lining mass. Recent chemotherapy is considered to be a wise choice based on patient concerns about recurrence, cosmetic problems, dysfunction, and even metastasis. In one study, the rate of cure in these patients following postoperative chemotherapy was 85%. However, recurrences usually occur in the first 3 years, half of which usually occur 1 year after surgery, which illustrates the importance of regular check‐ups.The NCCN guidelines recommend ongoing follow‐up with a focus on the lesion site every 6‐12 months, with a re‐biopsy of any suspicious symptoms or signs.4This is a rare tumor and we call special attention to the fact that its recurrence is extremely frequent, so there is absolute need to observe these patients periodically after surgery. This case motivated us to review the most important aspects of this rare tumor, besides presenting an interesting and typical case of DFSP with recurrence. All dermatologists and surgeons must know about the frequent recurrence of this tumor, sometimes even when excised with wide margins. For this reason, these patients must be observed periodically after surgery for a long time.Authors report no conflict of interest.All authors wrote and edited the manuscript equally.


Bilateral carotid artery ligation has been reported as a lifesaving procedure to control severe hemorrhage. However, reports are sparse and little information is available regarding the potential risks associated with this procedure. We report an ischemic brain infarct as a complication after vascular surgery. A 3-year old, male intact border collie was presented for acute onset of forebrain signs 5 days after bilateral carotid artery ligation. Multimodal brain MRI including morphologic sequences, MR angiography, diffusion- and perfusion-weighted images were performed. MRI revealed a well-defined intra-axial lesion of the left caudate nucleus, with increased T2 and decreased T1 signal intensity and moderate heterogeneous peripheral contrast enhancement. The cerebral blood flow was reduced relative to the contralateral caudate nucleus. Images were consistent with a subacute lacunar ischemic infarct of the left striate artery. Additionally, multiple arterio-arterial anastomosis arising from the vertebral arteries were visible in the angiography sequences. Ischemic infarct due to thromboembolism should be considered as a possible complication associated with bilateral carotid artery ligation. Collateral blood supply can develop as early as 5 days after surgery. BCAL is a lifesaving surgical procedure indicated in dogs with oral or maxillo-facial bleeding that cannot be stopped with standard hemostasis techniques –7. LigatWe report a striate artery infarct in a young adult Border collie 5 days after BCAL due to uncontrollable traumatic maxillofacial bleeding.A 3-year-old, male intact Border Collie was presented to the Veterinary Hospital of the Vetsuisse Faculty Zurich for profuse acute epistaxis as well as oral hemorrhage, occurring after playing with a stick. The origin of the hemorrhage could not be determined during oral examination and an underlying coagulopathy was excluded based on point of care clotting tests. For further workup, helical CT of the head and neck was performed under general anesthesia. The examination was performed with a 16-slice scanner  in 1.5 mm slices with the dog positioned in sternal recumbency and the head away from the gantry. Scanning parameters were set at 280 mA, 120 kV, 1 s rotation time, 0.7 pitch. Non-ionic iodinated contrast agent  was given intravenously, and post contrast scanning was programmed at 60 s after injection. CT showed accumulation of a large amount of heterogeneous soft-tissue attenuating material with few gas inclusions was present in the pharyngeal region, with focal contrast pooling seen immediately medial to the right pterygoid process . TherefoThe dog underwent emergency surgical exploration to manage the hemorrhage. The bleeding site was surgically inaccessible and the hemorrhage persisted despite local pressure hemostasis. A BCAL was performed as described by Goodman and Goodman . The bleImaging of the brain was performed with a 3 Tesla MRI scanner  using a 20-channel head/neck coil. The dog was positioned in dorsal recumbency and the scanning protocol included morphological sequences, diffusion-weighted imaging (DWI), magnetic resonance angiography (MRA), and perfusion weighted imaging (PWI) . For posThe conventional sequences revealed a well-defined lesion centered within the left caudate nucleus, extending ventrally, mildly laterally and rostrally and involved the basal nuclei  as well as the internal capsule. This lesion was T2- and FLAIR hyperintense and hypointense on precontrast T1 weighted images relative to spared cortical gray matter.Overall, there was no mass effect associated with the lesion except for a mild compression of left lateral ventricle. A thin FLAIR- hyperintense rim outlining the lateral contour of the rostral aspect of the left lateral ventricle was also noticed .Post contrast T1 weighted images showed moderate, heterogeneous, peripheral, faintly rim-like contrast enhancement and a central non-enhancing core. The sequences sensitive to the susceptibility effects caused by paramagnetic properties of hemoglobin degradation compounds (SWI) showed no signal changes in the described lesion.The lesion was hyperintense on the DWI sequence. The corresponding ADC map showed low signal intensity within the lesion core and increased peripheral signal intensity. Perfusion imaging revealed a well-defined, irregularly contoured area of decreased CBF within the left caudate nucleus .The CE- MRA and TOF sequences of the brain and neck showed the ligation of both common carotid arteries as abrupt tapering of the contrast enhanced arteries at the level of the caudal aspect of the second cervical vertebrae . The artThe diagnosis based on MRI was a subacute ischemic infarct of the left striate artery with mild associated perilesional cytotoxic edema and presence of multiple extracranial arterio-arterial anastomoses secondary to BCAL.Clopidrogrel anticoagulant therapy was converted to acetylsalicylic acid (3 mg/kg SID p.o.) because of financial concerns of the owner and the dog was discharged 1 day after the MRI. At re-check examination 1 week later, right sided postural reaction deficit was still present. Circling to the left was improved, but still intermittently present. Four weeks later the owner reported that the dog was back to normal but less resilient to exercise than before. Ten weeks after first presentation the dog returned to working again as a sheepdog 1,800 meters above sea level without any signs of subsequent damage.This is the first clinical case report with an MRI diagnosis of an ischemic brain infarct as a complication of BCAL. Studies using dogs as experimental models for BCAL report no neurological deficits, assuming that cerebral perfusion is not affected by the acute vascular ligation . The expIn humans, decreased CBF has been measured after temporary unilateral balloon occlusion of the carotid artery even in the absence of neurological deficits; nevertheless, this population has an increased risk for delayed cerebral infarction because of a poor flow reserve . In dogsRecently, use of state-of-art MRI sequences for diagnostic workup of vascular diseases in clinical veterinary medicine settings have been reviewed . We docuin vivo. Blood flow in both common carotid arteries was absent in the caudal cervical region and showed increased signal intensity again at the level of C2, branching normally cranial to this level. An extensive vascular network was seen in the vicinity of the ligation sites of the common carotid arteries, not only paralleling these arteries but also extending between the vertebral arterial system and the carotids and extending rostrally. Superficial arterio- arterial collaterals between the caudal auricular and the cranial thyroid arteries and the deep collateral vessel system connecting both vertebral arteries and the common carotid arteries were identified, consistent with the abovementioned experimental study (Collateral blood supply has been shown in an experimental canine study using corrosion casting 8 weeks after BCAL . Howeveral study . Numeroual study . Clear val study , 14.Albeit performing MRI according to the most recent recommendations and including new functional techniques, MRI findings fail to explain the pathomechanism behind the occurrence of the ischemic infarct and proving that the ischemic infarct reported in this case is a direct consequence of BCAL is not possible. However, the patient was a young and healthy working dog and besides risk factors related to the traumatic acute bleeding, no chronic diseases predisposing to stroke were identified. Determining a cause for ischemic infarcts is not possible in up to 50% of canine ischemic stroke patients , 33, 34.Platelet inhibition and anticoagulant therapies where only started after neurological deterioration and for this reason we do not know if this infarct could have been prevented by initiating this treatment directly after surgery. No recommendation exists in veterinary medicine regarding medical thrombus prevention in this type of surgery and it is beyond of the scope of a single case report to give treatment recommendation. Still this case report should raise awareness for strokes as possible complication after vascular ligation surgery in dogs. The use of anticoagulant therapy in the postoperative period should however be considered on a case by case basis weighing possible risks and benefits.Collateral blood supply to the brain can be visualized in MRA as soon as 5 days after BCAL. Ischemic infarct due to thromboembolism must be considered as a possible complication of this procedure.All datasets generated for this study are included in the article/Ethical review and approval was not required for the animal study because data were collected retrospectively. Written informed consent was obtained from the owners for the participation of their animals in this study.LK, AW-L, and KB contributed to conception and design of the study and wrote the first draft of the manuscript. LK collected clinical data. IL and AW-L wrote the imaging part of the manuscript. IL and AV wrote sections of the manuscript. AP, AV, AW-L, and KB corrected and finalized the manuscript. All authors contributed to manuscript revision, read, and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Primary spinal cord glioblastoma multiforme (scGBM) is an uncommon entity in pediatrics, and intracranial metastasis originating in spinal cord gliomas is very rare. A 7-year-old female presented with weakness in the limbs, paralysis of the lower limbs and incontinence. The initial MRI of the spinal cord revealed expansion and abnormal signals from T2 to T5. She was initially diagnosed with Neuromyelitis optica spectrum disorders and treated with high-dose glucocorticoid and gamma globulin. Four months later, her symptoms worsened and follow-up imaging showed multiple intracranial mass lesions. We performed a subtotal resection of the right thalamic basal ganglia tumor and gross total resection of the right frontal lobe tumor under microscopic examination. Histopathology revealed scGBM with intracranial metastasis and the molecular pathology diagnosis suggested H3K27M mutant diffuse midline glioma WHO grade IV, which had previously been misdiagnosed as a Neuromyelitis optica spectrum disorders. We review the literature of intracranial metastases originating from pediatric primary spinal cord glioblastoma multiforme and summarize possible methods of differentiation, including changes in muscle strength or tone, intramedullary heterogeneously enhancing solitary mass lesions and cord expansion in MRI. Finally, we emphasize that in unexpected radiological changes or disadvantageous response to the treatment, a biopsy to achieve a pathological diagnosis is necessary to discard other diseases, especially neoplasms. Glioblastoma multiforme (GBM) is the most common primary CNS tumor in adults but it is not common in children. However, among the group of malignant glial tumors, high grade glioma is the most frequent diagnosis in children. Furthermore, the incidence of intramedullary tumors is very rare in children; most are low grade gliomas . A spinaThis report presents an uncommon case of scGBM with intracranial metastasis closely mimicking Neuromyelitis optica spectrum disorders in both symptoms and MRI findings. The unique features of MRI and the clinical symptoms are summarized in the present article.A 7-year-old female patient was admitted to the hospital because of a 7-days history of weakness in her left lower limb, intermittent chest pain, and aggravation at night. The patient presented with a fever of more than 39 degrees centigrade, urinary and fecal incontinence and bilateral limb paralysis after 16 days, and her temperature was normal after high-dose glucocorticoid and gamma globulin intensive therapy, due to the suspicion of acute transverse myelitis. Gamma globulin 2 g/kg) and methyl prednisolone (20 mg/kg) were administered for 4 days. She underwent lumbar puncture, and a cerebrospinal fluid (CSF) assay showed that the protein level was 4073.4 mg/l. Then, her symptoms deteriorated in her upper limbs, and the patient had a seizure. Imaging examinations and urine culture analysis confirmed a diagnosis of pneumonia and urinary tract infection. She received meropenems and cephalosporins, but the neurological symptoms did not improve. Then, considering the mild remission from the initial intensive therapy, gamma globulin (2 g/kg) and methyl prednisolone (20 mg/kg) were administered again 5 days later. The CSF examination showed a growing CD19-positive T and B cell count (815 U/L). She was diagnosed with a Neuromyelitis optica spectrum disorder and was treated with gamma globulin (2 g/kg) on day 10 and Rituximab 0.3 mg on day 15. Her spinal cord and brain MRI findings revealed changes in accordance with the course and severity of the disease , 2. g/kg andThe patient presented to our institution with a 4-months history of weakness in her limbs. Her physical examinations showed bilateral lower limb muscular tension with an Ashworth Grade of 1; right and left upper limb muscle strength with grades of 4/5 and 3/5, respectively; bilateral lower limb muscle strength with a grade of 0/5; hypesthesia of superficial sense below C2 (mainly in the left limbs); non-cooperation on a deep sense examination; hyperfunctional bilateral biceps and triceps tendon reflexes (++); a hyperfunctional bilateral patellar and Achilles tendon reflexes (+++) with a grade of 3/4; a bilateral ankle clonus (+); and a positive bilateral Babinski's sign (+), which was suggestive of brain involvement. A brain contrast-enhanced MRI examination showed multiple intracranial lesions .We suspected a high-grade glioma and performed subtotal resection of the right thalamic basal ganglia tumor and gross total resection of the right frontal lobe tumor under microscopic examination. The pathological diagnosis was WHO grade IV GBM, and the molecular pathology diagnosis was H3K27M mutant diffuse midline glioma WHO grade IV .Postoperatively, the patient was treated with antiepileptic drugs and neurotrophic drugs, such as injection of mouse nerve growth factor. On a postoperative spinal MRI, there were multiple signals showing heterogeneous enhancement in the medulla oblongata, at the c3-7 level of the cervical spinal cord and margin, at the t3-6 level of the spinal canal, and at the t2-l1 level of the spinal cord and its margin. Patchy enhancement was observed in the soft tissue behind the thoracolumbar vertebrae, the bilateral cerebellum, the anterior pontine cistern and the fourth ventricle.Eleven days after the operation, the patient developed status epilepticus and coma. Despite conservative treatment, her general condition and neurological status did not improve. Her relatives asked that she be discharged 2 days later, and she died a month later.High-grade gliomas (HGGs) are the most common group of pediatric malignant CNS neoplasms, and midline tumor location in children is more frequent than in adults . As far MRI is an essential auxiliary diagnostic method that helps when judging the location of a spinal tumor and is very useful and helpful for tumor differentiation. The patient's tumors always showed mixed hypo-isointense signals on T1-weighted images (T1WI) and hyperisointense signals on T2-weighted images (T2WI). On MRI with contrast, scGBM presents with an irregular zone of contrast enhancement, and could be expressed as intramedullary heterogeneously enhancing solitary mass lesions and cord expansion in children . With thSpinal GBM is identified based on pathology parameters, such as its intracranial counterparts, including nuclear atypia, mitotic activity, vascular proliferation, and necrosis , 30, as Surgery is still the primary treatment for spinal GBM and related intracranial metastasis. However, there is controversy regarding the surgical extent of resection that should be achieved in high-grade spinal glioma patients. Ononiwu et al.  noted thThe role of radiotherapy and chemotherapy in GBM is still much debated. In their study of 158 high-grade spinal cord gliomas, Jiang Lui et al.  found thNeuromyelitis optica spectrum disorders (NMOSDs) are rare autoimmune diseases in which NMO-IgG selectively binds to aquaporin-4 (AQP4). NMOSDs mainly involves the optic nerve, spinal cord and central nervous system in the form of an inflammatory response in these areas. According to the new NMOSD diagnostic criteria, we suggest some characteristics that may be used to differentiate them: (1) serum AQP4 antibody detection; (2) association with clinical symptoms and MRI findings typical of optic neuritis; (3) in NMOSD, few muscles are involved, while scGBM often manifests with changes in muscle strength or tone, such as progressive weakness or paresthesia in both lower limbs; (4) a fast-moving process is frequently observed in NMOSD; and (5) rapid and significant improvement after high-dose glucocorticoid treatment. However, due to similarities in clinical symptoms and imaging findings and the rarity of both diseases, we were unable to accurately differentiate scGBM and NMOSD. There were some major potential contributors to the misdiagnosis that occurred in our case: (1) the nonspecific presentation of symptoms and imaging findings, (2) the rapid progression of the disease over time, and (3) the slight improvement observed after glucocorticoid treatment. This improvement may have been due to the remission of the secondary inflammation of the tumor rather than the treatment for NMOSD. There are very few records of scGBM imitating NMOSD. We emphasize the necessity of biopsy or surgical pathology in differentiating between spinal cord inflammation and neoplastic disease.While acute transverse myelitis (ATM) is a relatively common disease, it is rare in children. Compared with scGBM, ATM may present with substantially higher protein levels in CSF  and a notable absence of T1 hypointensity on spinal MRI  is a rare aggressive non-Hodgkin lymphoma confined to the brain, spine, eyes, and leptomeninges. It represents only 4% of intracranial neoplasms, and 4–6% of extranodal lymphomas. Phenotypically, PCNSL is almost always a diffuse large B-cell lymphoma (DLBCL) . This diIntracranial metastases originating from pediatric primary scGBM are extremely rare, and their atypical symptoms and imaging findings are the main causes of their misdiagnosis as other conditions, such as NMOSD, ATM, or lymphoma. Therefore, we should fully consider the possibility of scGBM and pay attention to differential diagnoses. If a patient's clinical symptoms and MRI and other auxiliary examinations do not allow a definitive diagnosis, it is necessary to perform a biopsy or surgical pathology as soon as possible.All datasets generated for this study are included in the article/supplementary material.The protocol was approved by the medical ethical committee of Zhengzhou University. This patient's parents gave written informed consent. The authors affirm that written informed consent was obtained from the participant for the publication of this case report.DS: manuscript writing. DX: conception and critical review. QG and PH: image extraction and pathological review. FG: conception and critical review. All authors proofread and approved the manuscript.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Portal vein pneumatosis is the presence of air in the portal venous system, which is one of the classic radiologic features of bowel ischemia or necrosis. However, there are several other morbidities that can have portal vein pneumatosis as a complication. This is a case of a 44-year-old man who suffered from severe abdominal pain after chemotherapy for soft tissue sarcoma of his left hip. The physical signs, laboratory findings, as well as the portal venous pneumatosis sign of the CT scan strongly indicated the probability of bowel necrosis and subjected the treatment decision of the patient finally to laparotomy. However, nothing abnormal except a segment of swollen small intestine was detected. Caution should be kept in mind when encountering a patient with suspected bowel necrosis following chemotherapy since several chemotherapeutic agents could cause portal vein pneumatosis. Diagnostic laparoscopy might be a better option for such cases. Portal vein pneumatosis is the presence of air in the portal venous system, which usually presents along with pneumatosis intestinalis, and is regarded as one of the classic radiologic features of bowel ischemia or necrosis. Portal vein pneumatosis was first described in 1960, and the reported incidence of portal vein pneumatosis in necrotizing enterocolitis varied from 10 to 33% . Therefo2 day 1, every 2 weeks) after the admission. Severe abdominal pain with diarrhea and vomiting were the complaints 3 days after the initiation of the second cycle of chemotherapy. There were no jaundice, abdominal distension, melena, and hematochezia. Physical examination revealed diffuse abdominal tenderness with mild rebound tenderness and guarding. Mild fever (37.6°C) and tachycardia (125 beats/min) were also demonstrated. Laboratory test results revealed remarkable neutrophilia  with normal bilirubin. The examination of stool showed no white blood cells. Computed tomographic images (CT) showed air in the portal  in patients with bowel ischemia . The necThe original contributions generated in the study are included in the article/Supplementary Material, further inquiries can be directed to the corresponding author.Y-XZ and Z-HC: acquisition, analysis, or interpretation of data for the work. C-CC and YC: drafting the work. KY: final approval of the version to be published. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Pseudoaneurysm should be considered in the differential diagnosis when the computed tomography (CT) findings show a mediastinal mass in patients with a history of cardiovascular surgery even if such surgery occurred over two decades previously. Pseudoaneurysm should be considered in the differential diagnosis when the computed tomography (CT) findings show a mediastinal mass in patients with a history of cardiovascular surgery even if such surgery occurred over two decades previously. A 68‐year‐old Japanese woman with a surgical history of total aortic arch replacement for a distal aortic arch aneurysm 20 years earlier visited our hospital because of back pain for three days and haemoptysis. Chest non‐contrast computed tomography (NCCT) was performed because of a contrast agent allergy and showed an abnormal mediastinal mass‐like lesion in the subaortic region Fig. . The radAppropriate written informed consent was obtained for publication of this case report and accompanying images.


Orthokeratinized odontogenic cysts (OOC) are developmental odontogenic cysts characterised by an orthokeratinized stratified squamous epithelial lining. They were originally believed to be part of the spectrum of Odontogenic Keratocyst, but are now considered to be a distinct entity. They are rare, making up approximately 1% of all odontogenic cysts and they usually occur singly. In this paper we present two new cases of multiple OOCs, and compare them to previous case reports of multiple lesions. The clinical and pathological features are discussed, along with possible diagnostic pitfalls. Orthokeratinized Odontogenic Cyst (OOC) is a developmental odontogenic cyst characterised by a lining of orthokeratinized stratified squamous epithelium . They weOOCs are distinct from OKCs as they recur less frequently, with a recurrence rate of less than 2% , in compHistologically, OOC differs from OKC predominately by orthokeratinization and the presence of a granular cell layer, in comparison to the parakeratin observed in OKC . In addiIn this paper we describe two cases of multiple and bilateral OOCs, followed by a review of the previous literature on multiple OOCs.A 23-year-old male presented with three lesions, two in the mandible and one in the maxilla. An orthopantomogram (OPT) radiograph revealed three cystic lesions, the mandibular lesions were both well-defined and corticated Fig. . In the Histology of all three cysts revealed similar features Fig. , showingThe patient had no signs or symptoms to suggest NBCCS and did not meet the criteria for a diagnosis of NBCCS . A diagnA 20-year male presented for extraction of a partially erupted and disto-angular positioned upper right second molar. On routine pre-operative radiographs, it was noted that both the left and right upper third molar teeth were impacted, displaced and associated with large, expansile radiolucencies Fig. a, b. A CThe lesions were enucleated along with surgical removal of the maxillary impacted third molars.Histological examination of both the left and right specimens Fig.  showed cA diagnosis of bilateral OOCs was made. To our knowledge, there is no evidence of recurrence at 24 months.These cases highlight the diagnostic difficulty of multiple OOCs, due to their clinical presentation being very similar to a number of other odontogenic lesions. In both cases OOC was not suggested in the clinical differential diagnosis, with the favoured diagnosis being OKC or dentigerous cysts.Both cases occurred in males, which is in agreement with previous reports that show a male predominance , 7. OOC A review of OOCs showed that 93% of cases were unilocular  and thisOOCs commonly present in association with an unerupted tooth , 7 and tAttempts have been made to explore the pathogenesis of OKC and OOCs using immunohistochemistry, with a number of studies reporting lower Ki67 expression, indicating a lower proliferation rate, in OOCs in comparison to OKCs –12. It hUnlike OKCs, there is no evidence that multiple OOCs are associated with NBCCS , 14. TheTable In summary, the two unusual presentations of OOCs outlined in this paper highlight the possibility of OOCs occurring bilaterally in either the mandible or maxilla. As OOC is a relatively new entity, raising the awareness of these lesions and their ability to mimic bilateral dentigerous cysts is of importance. Further work is required to elucidate a possible link to other associated lesions in certain patients.


Syringoma is a benign adnexal tumor that originates from the acrosyringium. Eruptive syringoma is a rare variant that manifests in the form of skin-colored or brownish, shiny, angulated papules that occur in successive crops. Dermoscopic evaluation reveals brownish regular pigment network and tiny whitish dots between adjacent papules that correspond histologically to multiple eccrine ducts lined by a double-layered epithelium, giving a paisley tie appearance. This case of eruptive syringomas presenting with brownish papules on the neck highlights the importance of histopathological and dermoscopic evaluation.A woman in her late twenties presented with a 7- to 8-year history of asymptomatic papules on the neck. The papules developed episodically, in clusters . She hadA 4-mm punch biopsy was obtained from one of the papules. Histopathologic analysis revealed multiple eccrine ducts and solid nests of epithelial proliferation within the dermis. The ducts were lined by a double-layered epithelium. Amorphous eosinophilic material could be appreciated in some ductal lumina. Some ducts showed small, comma-like extension of epithelial cells. The stroma was collagenous .Dermoscopic evaluation  of a papule revealed brownish regular pigment network and tiny whitish dots between adjacent papules . Based oSyringoma is a benign adnexal tumor that originates from the acrosyringium . The comHistopathological findings include eccrine ductal proliferation in a dense fibrous stroma. There is formation of nests and cords of double-layered epithelial cells. The outer epithelium is often elongated into comma-like structures, resembling a paisley tie pattern. Dermoscopic features include a delicate pigment network and multifocal whitish areas . The pigThe lesions are benign and usually remain static. Treatment modalities include dermabrasion, excision, electrodesiccation, chemical peeling, and oral and topical retinoids. The outcome is usually unsatisfactory. This case of eruptive syringomas presenting with brownish papules on the neck highlights the importance of histopathological and dermoscopic evaluation.


An 84-year-old male, with a large gouty tophus over the left elbow for 20 years, developed a new left elbow area mass about three months ago and presented to the emergency department (ED) with complaints of bleeding from that left elbow area mass after a small blunt injury. Initially diagnosed as olecranon bursitis, it had the worsening size and atypical growth associated with persistent leukocytosis. After being evaluated by multiple specialties, including infectious diseases, orthopedic surgery, and rheumatology, it was decided to do a biopsy, which resulted in the diagnosis of histiocytic sarcoma. Histiocytic sarcoma is a very rare disease belonging to histiocytic disorders that originate from histiocytic cells that are of hematopoietic origin. Histiocytic sarcoma may occur as a sporadic extranodal neoplasm or may be a complication of hematologic malignancies such as follicular lymphoma or acute lymphoblastic leukemia. Histiocytic sarcoma (HS) is an aggressive neoplasm with a rapidly progressive course. With only a few hundred cases reported in the medical literature, HS contributes to less than 1% of all hematopoietic malignancies [Clinical presentation is mostly a neoplastic mass, extranodal in location, commonly involving the skin. Other sites involved are the spleen and gastrointestinal tract. Associated signs and symptoms are nonspecific systemic symptoms such as fever, weight loss, anorexia, fatigue, hepatosplenomegaly, lymphadenopathy, rash, and pancytopenia. Diagnosis is made by the excisional or incisional biopsy of neoplastic tissue. Treatment options include local surgery, radiotherapy, and chemotherapy, depending on the stage of the disease. Being so rare, diagnosis is usually delayed and the overall prognosis of histiocytic sarcoma is very poor.Here we submit a case report of a rare presentation of a very rare disease.An 84-year-old man with a past medical history of gouty arthritis, hypertension, chronic kidney disease stage III, cardiomyopathy, and atrial fibrillation presented to the emergency room with complaints of bleeding from the left elbow area. The patient had developed a mass about three months ago over the left elbow and was evaluated by his orthopedic surgeon, who diagnosed it as olecranon bursitis. Bleeding started after his elbow bumped into his car door. Vital signs at the time of presentation: blood pressure 99/60, pulse 100 per min, temperature 36.6 °C, respiratory rate 18 per min, and saturation 98%.Physical examination showed a soft tissue mass of about 6x6 cm over the left elbow, just below the olecranon process, with a small area of skin necrosis and oozing of blood Figure . The patThe patient was admitted and treated for possible sepsis based on sepsis criteria. Coumadin was held and bleeding was stopped with a pressure dressing. Needle aspiration was done from the elbow mass with mild serosanguinous sterile fluid. Blood and fluid cultures were negative. Infectious diseases, orthopedic surgery, and rheumatology were consulted for the left elbow area mass. The patient had persistent leukocytosis Table  even thoDue to his advanced age with multiple comorbidities, including decompensated heart failure, along with worsening renal failure complicated by severe protein-calorie malnutrition and deconditioning, the patient became severely deconditioned with declining quality of life. Considering his overall prognosis, the patient and family together opted for comfort measures only and the patient passed away with hospice.Histiocytic sarcoma is a rare neoplasm of mature histiocytes, presents as a painless mass most commonly extranodal in origin, and can arise as a primary tumor or as a transformation of a pre-existing low-grade B-cell lymphoma. About 2% to 8% of chronic lymphocytic leukemia cases can transform into a histiocytic sarcoma .The diagnosis of histiocytic sarcoma is made after an incisional/excisional biopsy based on the immunohistochemical delineation of the characteristic expression of one or more histiocytic markers. Specifically, the presence of macrophage-associated antigens, CD68, and lysozyme immunophenotypically confirm the histiocytic lineage. Also, CD163 has been recently characterized as a more specific marker for histiocytic neoplasms. In cases of histiocytic sarcoma arising from B-cell lymphomas, there may be a retention of some B-cell markers . TypicalThe standard of treatment is surgical with local resection. Adjuvant radiotherapy and chemotherapy are used especially for multifocal presentations. Bone marrow transplantation is an option depending on the presentation and immunophenotypes -6.Histiocytic sarcoma is an aggressive tumor with about 50% mortality. It is a rare epithelioid malignancy often misdiagnosed with other sarcomas and cases of non-Hodgkins lymphoma. Histiocytic sarcoma may arise primarily as a soft tissue tumor and tumor size may be a prognostic factor. Hence, high clinical suspicion and vigilant pathological examination with specific immunohistochemical phenotyping are essential and play an important role in early diagnosis and, possibly, prognosis.


Colonoscopy is a low-risk procedure performed for screening and diagnostic purposes. About 15 million colonoscopies were carried out in the United States in 2012 with this number projected to increase. Injury to the spleen as a complication of colonoscopy is still a rather rare occurrence. We report a case of significant splenic injury, American Association of Surgery for Trauma (AAST) grade III with hemoperitoneum, in a patient following diagnostic colonoscopy, managed conservatively without the need for invasive or salvage surgical procedure. Colonoscopy is a low-risk procedure for screening, diagnostic, and therapeutic purposes . BleedinA 72-year-old female presented to the emergency room (ER) with complaints of left upper quadrant (LUQ) abdominal pain and weakness 8 hours after a diagnostic colonoscopy was performed to investigate chronic constipation. Her past medical history was significant for two episodes of unprovoked deep vein thrombosis including one episode with associated pulmonary embolism for which she was on oral anticoagulation with warfarin. The patient was advised to stop warfarin 7 days before her colonoscopy and to use enoxaparin for bridging therapy. Prothrombin time (PT) and the international normalized ratio (INR) measured on the day of the procedure were 19.0 sec and 2.0, respectively. Colonoscopy was performed under monitored anesthesia care (MAC) with propofol. Normal findings were reported with no technical difficulties encountered nor any therapeutic intervention performed or biopsies taken. The duration of the procedure was 16 minutes. At discharge, she remained asymptomatic, and warfarin was resumed.On return to the ER, examination revealed normal vital signs, a distended abdomen, and left upper quadrant (LUQ) abdominal tenderness. The rest of her physical examination was unremarkable. Laboratory examination showed a hemoglobin concentration of 8.1 g/dl, PT 15.3 s, INR 1.6, and activated partial prothrombin time (aPTT) of 22.3 s. Platelets were 322000 U/L at presentation. The rest of her labs were within normal limits. A computed tomography (CT) scan of the abdomen and pelvis with contrast revealed perisplenic heterogenous and hypodense fluid collection suspicious for hemorrhage or subcapsular hematoma with free fluid extending into the pelvis  during MAC, and use of anticoagulation in our patient, we cannot conclusively say why our patient had injury to the spleen during diagnostic colonoscopy, given there are no clearly identifiable risk factors that predict its occurrence. Due to weak evidence in data concerning risk factors and nonspecific symptoms , 13, we Splenic injury still is a rare but sometimes fatal complication following colonoscopy. Given the lack of properly identifiable risk factors and variation in its presentation, this rare but potentially fatal complication can present a diagnostic dilemma for clinicians and requires a high index of suspicion in patients with postprocedural pain following colonoscopy. Greater awareness is needed amongst healthcare professionals, including family physicians, emergency room doctors, midlevel staff, and internists about this potentially deadly but rare complication following colonoscopy. Increased awareness may help improve outcomes and decrease mortality.


GISTs are often asymptomatic or cause mild abdominal pain or GI bleeding. Rarely, an exophytic GIST may rupture leading to intraperitoneal bleeding. Surgical resection with negative margins is the mainstay of treatment although patients presenting with tumor rupture are at higher risk of dissemination and recurrence.This is a case report of a ruptured gastrointestinal stromal tumor (GIST) presenting as spontaneous hemoperitoneum. The patient was a 63-year-old female with a past medical history of hypertension and ulcerative colitis who presented to the emergency department with worsening epigastric pain. The patient denied history of trauma, previous surgeries, or forceful vomiting. She was not on anticoagulation. Vital signs at presentation were stable. A CT scan of abdomen/pelvis revealed a large amount of fluid in the upper abdomen with high attenuation material adjacent to the greater curvature of the stomach concerning for hemoperitoneum. Diagnostic laparoscopy revealed a significant amount of blood along the upper abdominal viscera. The procedure was converted to an upper midline laparotomy after identifying a necrotic, extremely friable 7 x 6 x 3 cm pedunculated mass with active hemorrhage on the posterior aspect of the greater curvature. A wedge resection was performed to remove the mass with grossly negative margins. An intraoperative frozen section revealed a stromal tumor with spindle cells. Final pathology revealed a pT3N0M0 stromal tumor with histologic spindle cells and a high mitotic rate (24/5 mm Gastrointestinal stromal tumors (GISTs) comprise the largest subgroup of mesenchymal neoplasms . The majIn this case report, we present a rare occurrence in which an undiagnosed GIST presented with tumor rupture and spontaneous hemoperitoneum.The patient was a 63-year-old female with a past medical history of hypertension and ulcerative colitis who presented to the emergency department with worsening epigastric pain. The pain started four days prior with one episode of non-bloody emesis, nausea, and one episode of dark stool. She denied previous history of abdominal surgery or trauma. She was not on any anticoagulant. Vital signs were within normal limits. On examination, the patient was diffusely tender to palpation, especially in the epigastrium. Initial lab work was unremarkable except for a mild leukocytosis of 12.3 k/uL and hemoglobin of 11 g/dL. A CT scan of abdomen/pelvis with intravenous contrast showed a large amount of blood in the upper abdomen  negative margin. Lymphadenectomy is not required due to the fact that these tumors rarely metastasize to lymph nodes. Tumor rupture, as in our patient’s case, increases the risk of recurrence and can lead to intraperitoneal spread. Due to the expression of c-kit in the majority of these tumors, tyrosine kinase inhibitors such as imatinib are effective in both the neoadjuvant and adjuvant settings in increasing progression-free and overall survival in advanced tumors .2) and presence of tumor rupture. She will undergo CT scanning of the chest, abdomen, and pelvis every three to six months for three to five years followed by annually thereafter.Our patient presented with nausea, vomiting, and acute onset epigastric abdominal pain with CT evidence of hemoperitoneum. She had no prior abdominal surgical history and no free air was identified on imaging to suggest a perforated ulcer. The preoperative CT scan did not clearly identify the mass due to the surrounding intraperitoneal hemorrhage. Once the source of the bleeding was found to be secondary to a friable gastric mass at the time of operation, a GIST was suspected. As previously mentioned, surgical resection is the mainstay of therapy for a GIST and the mass was resected with grossly negative margins. Postoperatively, the patient was referred to medical oncology and imatinib therapy was initiated with a planned duration of at least three years given the high mitotic rate (24/5 mmIn conclusion, a ruptured GIST should be in the differential when a patient presents with spontaneous hemoperitoneum without an obvious underlying cause. In patients with resectable disease, the mainstay of treatment is palliative surgical resection along with the addition of tyrosine kinase inhibitors for advanced tumors. Unfortunately, patients with a GIST who present in this manner have shorter overall survival and are at an increased risk of recurrence despite surgical and medical therapy.


Parastomal hernia is a common complication of patients living with an enterostomy. However, a parastomal hernia involving the gallbladder is a rare condition with only eight cases documented in the literature.We report the case of a 69-year old female who underwent an open right hemicolectomy with creation of a colostomy and terminal ileostomy. She presented with parastomal swelling and pain 16 months later. A computed tomography scan revealed a parastomal herniation of the gallbladder. We elected to proceed with a cholecystectomy and hernia repair, the patient was asymptomatic at her last follow-up.A systematic search of the literature found eight previously published cases. This condition primarily affects elderly females. Five patients were treated surgically and three conservatively, all with a favorable outcome. In frail patients without complicating factors, a conservative treatment approach with surveillance may be safe. We chose a surgical approach due to the symptomatic nature of the presentation and the gallstone containing hernia. This is the first case of a parastomal gallbladder herniation containing a large gallstone.This report should help broadening the physician’s differential diagnosis in dealing with patients with symptomatic parastomal hernias and provide an example for diagnosis and management of this complication. In Switzerland, about 1200 new stomas are created annually and 1000 patients per million inhabitants live with a stoma . AsymptoMost PSH involve a reducible segment of the omentum, colon, or small bowel, more rarely are cases involving herniation of the stomach . In cont2A 69-year-old female patient with a past medical history of hypertension and coronary heart disease as well as corticosteroid-treated chronic obstructive lung disease and a Body Mass Index of 25 kg/m2 underwent an urgent explorative laparotomy with right hemicolectomy and ileotransversostomy due to ischemic cecal necrosis. We performed a relaparotomy with creation of a colostomy and terminal ileostomy due to an anastomotic leakage on postoperative day six after the initial operation.After an uneventful postoperative rehabilitation, the patient presented to the outpatient clinic 16 months after the initial operation with parastomal swelling, tenderness and pain. The physical exam revealed a parastomal hernia under Valsalva in the erect position and an asymptomatic incisional hernia of the former median laparotomy.Due to the symptomatic nature of the parastomal hernia, we decided to perform an abdominal computed tomography scan (CT) . The exaOur clinical suspicion of a parastomal hernia was substantiated through the results of the CT scan. The finding of a gallbladder herniation was a surprise but the preoperative diagnosis helped with the planning of the procedure.After elective laparotomy, the gallbladder was carefully reduced out of the parastomal hernia  followed3Parastomal herniation of the gallbladder is uncommon. We conducted a systematic search of the EMBASE and MEDLINE databases with the following terms ‘gallbladder’ and ‘parastomal hernia’. We found 12 published articles in this search of which we included seven in this article. One additional manuscript was identified through bibliographic analysis. In total, we identified eight other cases of parastomal gallbladder herniation  [[7], 8. The aveOf the previously described cases, three were successfully treated conservatively despite an initially symptomatic presentation ,10,13. EThe high incidence of PSH with its subsequent adverse effects on quality of life, the need for surgical repair, and increased cost provide the reason to prevent PSH formation rather than treat its consequences . In accoDiagnostic tools including clinical  as well as radiological methods such as ultrasound and CT have been reported for the diagnosis of PSH . Most pr4Our report and the summary of the previous literature may promote the physician's awareness for a differential diagnosis in elderly females with symptomatic PSH, increase the understanding of this rare condition and guide its diagnosis and treatment.There are no actual or potential conflicts of interest capable of influencing judgment on the part of any author.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.This retrospective case report is exempt from ethical approval at our institution.Written informed consent was obtained from the patient’s son 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.BM, PL, PAC, RV were involved in the conception of the work.PL and RV were involved in the surgical treatment of the patient.BM and PL drafted the manuscript, RV and PAC critically revised the manuscript.All authors read and approved the final manuscript.This is not human subjects research.Beat Moeckli, MD.Not commissioned, externally peer-reviewed.


Meckel's cave (MC) epidermoid cysts are relatively uncommon lesions. In cases where surgical excision is indicated, resection is often carried out via a frontosphenotemporal craniotomy from an anterolateral approach or a temporal craniotomy with or without a petrosectomy for a lateral corridor; both of these routes are associated with brain retraction and potential neurovascular injury. The anterior location of MC in the middle cranial fossa makes safe access via posterior fossa-based approaches—such as the retrosigmoid approach—challenging as well. Here, we present the cases of two patients diagnosed with epidermoid cysts in MC who underwent surgical resection via an endoscopic endonasal transpterygoid approach. Near-total resection was achieved in both cases, with only mild transient neurologic disturbances postoperatively. Radiographically, no evidence of residual disease was noted in either patient. We further review the nuances of an extended endoscopic endonasal approach to these lesions. Epidermoids are cystic lesions that are generally considered to be benign lesions that occupy the CSF space. On magnetic resonance imaging (MRI), they characteristically appear hypointense on T1-weighted and hyperintense on T2-weighted sequences . Open suA 64-year-old woman was sent to our clinic for evaluation of a 2-year history of right-sided facial numbness. Her symptoms involved all divisions of the trigeminal nerve. She had initially presented to our institution 12 years prior with an MRI of the brain revealing both a large right cerebellopontine angle mass, suggestive of a vestibular schwannoma, as well as a right middle cranial fossa lesion radiographically consistent with an epidermoid cyst. She underwent resection of the vestibular schwannoma at our institution and remained asymptomatic during several years of clinical follow-up. Ten years following resection of the vestibular schwannoma, she began to experience progressive right-sided facial numbness, which persisted for two years. MRI of the brain at this time revealed mild radiographic progression of her known right-sided MC lesion, measuring 2.1 × 1.3 cm in greatest dimensions . Given tA 26-year-old woman with a history of congenital bilateral sensorineural hearing loss was found to have a large right-sided cerebellopontine angle mass extending anteriorly to the middle cranial fossa into the region of MC. The patient had previously undergone a right temporal and petrosal craniotomy for resection of this mass; histopathological analysis at that time demonstrated an epidermoid lesion. Given technical difficulties in resecting the anterior most aspect of the epidermoid via an open craniotomy, an endoscopic endonasal right transpterygoid approach to the remaining epidermoid cyst in MC was undertaken Figures 2. CN VI w5The endoscopic endonasal transpterygoid approach has been well described previously and is beyond the scope of this paper . Both paMC is a dural recess in the posteromedial portion of the middle cranial fossa. Lesions in this region are uncommon overall and can be varied in their etiologies . Space-oThe PubMed electronic database was searched from inception until December 2019 for English language studies using the search terms “Meckel's” and “epidermoid.” A total of 25 peer-reviewed articles were identified, reviewed, and included in our analysis. No articles were excluded. Only one of these 25 articles describes one patient with an epidermoid in a series of 8 patients with Meckel's cave tumors undergoing the endoscopic endonasal approach, substantiating the limited literature available on this topic . EpidermThe most commonly used surgical approach to MC has been the pterional approach, providing anterolateral access to MC but concomitantly associated with risk of damage to traversing neurovascular structures along a narrow operative corridor bounded by the internal carotid artery, the petrous apex, geniculate ganglion, and trigeminal nerve branches, amongst others , 18. FurThe endoscopic endonasal approach to the cranial base has undergone a tremendous transformation over the past few decades, heralded by the advent of newer more sophisticated tools and instruments to allow access to the region. Careful consideration of anatomic corridors along with appropriate patient selection continues to allow surgeons to expand the use of the endoscope in addressing more complex regions in the anterior cranial fossa , 23. TheAn endoscopic endonasal approach, importantly, provides an anteromedial corridor to the trigeminal ganglion with reduced need for trigeminal nerve manipulation required to maximize operative views . SeveralAs epidermoid cysts are exceedingly rare intracranial lesions, documentation of successful operation on them in Meckel's cave via an endoscopic endonasal approach is limited. Endoscopic-assisted techniques have been shown to be useful in the visualization of epidermoid tumors in several studies , 28. In In both of our cases, near-complete resection was achieved based on intraoperative impression and neuronavigational confirmation. Minor residual epidermoid remained in both cases, but postoperative MRI completed within 48 hours of surgery revealed no appreciable residual. Both patients were discharged from the hospital on postoperative day three without any major complications. Minor, transient neurologic deficits/symptoms relating to CN V (V2 pain) and VI  were noted in patients 1 and 2, respectively. Transient V or VI deficits have commonly been described following resection of MC tumors through craniotomy approaches [While only limited to two cases, our results suggest that the endoscopic endonasal approach to MC utilizing a transpterygoid approach may represent a safe alternative to open surgical approaches for epidermoid cysts at this region. Particularly, in our second case, an open craniotomy approach would have likely been associated with a lower rate of resection, given the marked anterior extension of the epidermoid and narrow working corridor. Given the proximity of the cranial nerve III through VI and the carotid artery, there is still potential for neurovascular injury with the endonasal approach. Utilization of image guidance to confirm intraoperative findings, the usage of neurophysiological monitoring to provide real-time feedback, judicious use of intraoperative ultrasound, and careful review of the preoperative imaging can help minimize such injuries. Additionally, such cases should only be considered and performed by multidisciplinary teams of neurosurgeons and otolaryngologists that are very experienced with endoscopic endonasal skull base surgery.The endoscopic endonasal approach can be utilized for the safe and effective resection of MC epidermoid cysts. Intraoperative neurophysiological monitoring is very helpful in these particular cases. These approaches should only be considered by experienced, multidisciplinary teams following careful review of preoperative imaging. Additional follow-up and studies will be needed to determine long-term outcomes.


Hepatic mesenchymal hamartoma is a rare hepatic tumour seen in the paediatric population. Although this entity has a variable imaging appearance, it has a favourable prognosis if diagnosed and managed correctly. This case presents the ultrasound and computed tomography findings of an 11-month-old patient who presented with a history of progressive abdominal distension and an elevated alfa fetoprotein level on biochemistry. The case describes how a confident perioperative diagnosis could be made on the basis of characteristic imaging features. Hepatic mesenchymal hamartoma is an uncommon, benign, paediatric tumour, most commonly encountered during the first 24 months of life. Because of an excellent prognosis, distinguishing this tumour type from other liver masses is essential, and confident perioperative diagnosis could be made on the basis of characteristic imaging features.An 11-month-old male patient presented at the department of paediatrics with a 3-month history of progressive abdominal distension. The child was otherwise systemically well, born at term and had no significant background medical history. Clinical examination revealed an upper abdominal mass lesion, non-tender and firm to palpation. Ensuing abdominal ultrasound showed a complex solid-cystic upper abdominal mass lesion, with concern for possible hydatid disease. Initial biochemistry revealed mild elevation in gamma-glutamyl transferase (GGT) with a marginally elevated alfa fetoprotein (AFP) level of 44.4 µg/L . Hydatid serology, blood cultures and confirmatory human immunodeficiency virus (HIV) testing were negative. Further imaging was then preformed.Contrast-enhanced abdominal computed tomography (CT) in the portal venous phase demonstrated a large, well-demarcated intraperitoneal mass , measuriBased on the above-mentioned imaging findings, the initial differential diagnoses included that of primary hepatic lesions as well as extra-hepatic mesenteric lesions. The most likely diagnosis within each category was a hepatic mesenchymal hamartoma and mesenteric lymphatic malformation respectively.The child was referred to a tertiary institution for further management. Because of delay in follow-up, a repeat abdominal CT was performed approximately 4 weeks after the initial study. This was carried out to evaluate for interval growth and assist in surgical planning. It revealed the mass to have shifted into the left-sided abdomen, completely separable from the right and left liver lobes, with displacement of the bowel to the right . A thin Subsequent laparotomy confirmed a large pedunculated mass arising from the caudate lobe of the liver. The lesion was excised and sent for histological evaluation. Macroscopic pathological assessment showed solid components with the appearance of haemorrhagic liver parenchyma with areas of haemorrhage as well as cystic components, the largest measuring 30 mm × 25 mm × 20 mm. The cysts contained yellow serous fluid with a smooth, unilocular inner cystic wall. Areas of necrosis and calcification were not identified.Microscopic and immunohistochemical evaluation showed clusters of hepatocytes, without cellular atypia, along with numerous bile ducts of variable proportions, haphazardly arranged within an oedematous fibromyxoid stroma. The stroma consisted of spindled fibroblasts with numerous vascular spaces interspersed with the bile duct. The cysts were lined with a single layer of columnar epithelium, also without atypia. Areas of acute haemorrhage were noted, with prominent vascular ectasia. No granulomatous inflammation, viral cytopathy, parasites, protozoa or features of invasive malignancy were visualised. These findings are in keeping with a histological diagnosis of hepatic mesenchymal hamartoma.A 3-month follow-up ultrasound revealed a normal liver with no residual or recurrent tumour.This article followed all ethical standards for a research without direct contact with human or animal subjects.1 A slight male predominance is documented. As the term ‘hamartoma’ implies, it should rather be considered a developmental malformation than a true neoplasm. Very rarely these hamartomas can undergo malignant degeneration into undifferentiated embryonal sarcoma. Mesenchymal hamartomas were first described in the literature by Edmondson in 1956.2 Initially, these lesions were variably named as hepatic or giant lymphangiomas, bile duct fibroadenomas, and cavernous lympangiomatoid tumours before being grouped together under the term ‘mesenchymal hamartoma’. Association with polycystic kidney disease, congenital hepatic fibrosis and biliary hamartomas has been described.4Hepatic mesenchymal hamartoma is an uncommon, benign tumour with an excellent prognosis, which may be encountered during paediatric imaging, typically in patients aged between 1 month and 4–5 years.6 Serum AFP levels can be within the normal limits, but can also be elevated because of the proliferation of hepatocytes in the neoplasm.7 Elevated GGT, but otherwise normal liver function tests, is also reported.3Clinical presentation varies on the basis of tumour size. Smaller hamartomas are usually asymptomatic, with a painless mass lesion incidentally discovered on abdominal examination. Pathologically, the neoplasm develops and progressively enlarges because of mesenchymal proliferation, with the development of multiple fluid-containing cysts contributing to the rapid enlargement of mass. Whilst well-circumscribed, this mass is unencapsulated.10Regarding serum AFP levels in the paediatric population, these levels are routinely used as an important biomarker for hepatoblastoma, where values are typically very elevated . However, this is limited in terms of sensitivity and specificity for the diagnosis of malignancy, as it is a tumour-associated and not a tumour-specific protein. A mild elevation in serum levels can be seen with other malignant and non-malignant conditions, such as hepatocellular carcinoma, mesenchymal hamartoma, cirrhosis, viral and chronic active hepatitis, and ulcerative colitis, to name but a few. In patients with hepatoblastoma, 5%–10% of these may have a low or even normal AFP level. Concentrations are also age-specific, with high levels observed in neonates and exceedingly high levels in preterm neonates. Levels usually decline and reach normal concentrations at 8–12 months of age. Alfa fetoprotein levels should thus be correlated with imaging and histopathology findings whilst taking the patient’s age into account.1Mesenchymal hamartomas are commonly large at the time of diagnosis, weighing over 1 kg, and most commonly arise from the right lobe of the liver. These lesions arise from the mesoderm within portal tracts. Pathological hallmarks are clusters of normal hepatocytes in a matrix of porous mesenchyme, which allows accumulation of fluid as it degenerates, predisposing to cyst formation. Additional cystic elements are attributable to dilated biliary ducts.2 Plain abdominal radiographs may show hepatomegaly or a paucity of bowel gas in the right upper quadrant, suggesting a hepatic mass lesion. Sonography is the modality of choice for initial assessment in the paediatric age group. Right upper quadrant findings can vary from large, septated cysts to small cysts with thick septations, with varying degrees of associated solid echogenic tissue, in relation to the liver and separable from the biliary tree. Computed tomography confirms sonographic findings, and better delineates the extent of the mass lesion and relationship to other intra-abdominal viscera. The appearance is commonly that of a well-defined solitary mass lesion of hepatic origin, either completely intrahepatic, partially extrahepatic, and with an exophytic or pedunculated pattern of growth. Enhancing soft tissue elements are usually seen surrounding a central area of simple fluid attenuation, with variable degrees of internal septation and loculation. This multi-cystic configuration is commonly labelled as a ‘swiss cheese appearance’. Completely solid, stromal predominant tumours are rarely seen and are usually smaller lesions. Haemorrhage is uncommon. Calcification, although it may very rarely occur in these lesions, is much more commonly seen in other tumours. Computed tomography appearance may be deceiving in instances where cysts mimic tumour necrosis seen in malignant hepatic neoplasms, and ultrasound or magnetic resonance imaging (MRI) is helpful to differentiate simple anechoic cysts from liquefied tissue.4Radiological imaging plays a major role in both diagnosis and planning the surgical approach to the lesion, as clinical and biochemical features are nonspecific.1 Magnetic resonance imaging has a value in confidently establishing hepatic origin in cases of large, pedunculated, predominantly cystic lesions and to accurately delineate solid and cystic components, septations and relations to surrounding structures – especially the intrahepatic vasculature and the biliary system. Appearance may vary depending on composition. The T1 signal intensity of cystic components may differ based on the concentration of protein in the fluid but usually approximates water intensity on T2-weighted imaging. Solid components may appear hypointense to adjacent liver on both T1- and T2-weighted sequences because of fibrosis. Enhancement post-gadolinium is usually mild and confined to the septal and stromal elements.4Scintigraphy with technetium-labelled sulphur colloid confirms the presence and position of either a solitary or multiple photopenic masses, as the hamartoma lacks Kupffer cells. This modality is otherwise non-specific regarding further delineation.11 Intrahepatic considerations are simple congenital hepatic cysts, which are generally rare in children and are not septated, or form part of the spectrum of polycystic kidney disease, where renal cysts are also present. Choledochal cysts are also not septated but are coupled with a degree of biliary dilation. Infectious foci, such as abscesses, have a different clinical picture with more complex internal content. Amoebic abscesses and hydatid diseases are also considered, but biochemical assessment helps in the diagnosis. Mesenchymal hamartomas with a larger degree of soft tissue may mimic cystic hepatoblastomas, the focal form of infantile hepatic haemangiomas, cavernous haemangiomas (adult-type hepatic haemangiomas), teratomas or hepatocellular carcinomas in older patients. Cavernous haemangiomas usually demonstrate a typical pattern of avid peripheral enhancement with centripetal fill-in, but differentiation could be challenging. Teratomas commonly contain fat and calcification. Calcification is also common in hepatoblastomas and infantile hepatic haemangiomas, which are more solid in nature. Alfa fetoprotein is significantly elevated with hepatoblastoma. Generally, the relatively poor enhancement of the solid components of cystic mesenchymal hamartomas helps in differentiation from these lesions. Undifferentiated embryonal sarcomas, an even rarer entity, may be indistinguishable from cystic mesenchymal hamartomas.4Differential diagnoses include benign and malignant hepatic or intra-abdominal masses, as summarised in 6 There is a strong association with undifferentiated embryonal sarcomas, which are especially aggressive and have a poor prognosis.13 Long-term follow-up is necessary post-resection. Treatment with surgical resection is usually curative and yields admirable results. Partial lobectomy is commonly needed to remove larger tumours. The large size of these tumours makes the surgery technically challenging. If respiratory distress is the presenting complaint, percutaneous catheter drainage of large cystic elements is frequently performed as a temporising measure prior to surgical intervention.3These neoplasms may undergo malignant transformation, and, as a result, complete surgical excision, with clear margins, remains the mainstay of treatment.Hepatic mesenchymal hamartoma is a rare hepatic tumour with a variable imaging appearance that has an excellent prognosis if identified and managed appropriately. In rare instances, malignant degeneration to undifferentiated embryonal sarcoma could arise whilst having a similar imaging appearance. A high index of suspicion should be asserted in all cases of asymptomatic, cystic liver masses in children and a clear understanding of the most important differential diagnoses is imperative.


Extramedullary plasmacytomas (EMPs) are uncommon plasma cell tumors that develop in soft tissue as isolated tumors without osseous involvement while secondary lesions are associated with systemic multiple myeloma (MM). Primary extramedullary lesions are most commonly found in upper respiratory tract, gastrointestinal tract and lymph nodes. They can be found either in patients with history of MM or preceding the manifestation of a systemic disease. Orbital manifestation of the lesion is rare but conjunctival involvement is very unusual. The reported cases in the English-written literature are only five cases. Herein, we report the sixth case of primary EMP in a middle-aged adult who presented with a lesion confined to the conjunctiva, unremarkable present and past medical history, and confirmed tissue diagnosis. In addition, a review and summary of the previously reported cases in the literature is presented. We aim to attract the attention of ophthalmic surgeons to consider plasmacytoma within the differential diagnosis of a conjunctival lesion. •Extramedullary plasmacytomas (EMP) are isolated primary soft tissue plasma cell tumors without osseous involvement.•Secondary lesions are associated with systemic multiple myeloma.•The conjunctiva is a very unusual location for primary lesions.•We are reporting the sixth case of conjunctival primary EMP.•All cases including ours are summarized. Primary lesions are uncommon plasma cell tumors that develops in soft tissue as isolated tumors without osseous involvement, while secondary lesions are associated with systemic multiple myeloma (MM) . Primary2A 44-year-old Pakistani male, who was not known to have medical illnesses, and walked into our academic institution with a right lower lid inner palpebral swelling for one month. The mass was painless and progressively increasing in size. There was no history of tearing or discharge. The patient reported an unclear history of ocular surface surgery 5 years prior to his presentation for excision of a smaller conjunctival lesion of unknown nature in the same location. No histopathological details about that lesion were available and no diagnosis was obtained from the history. He was not receiving any regular medications and he was not a known smoker. He was not a good historian especially because of language barrier, however limited family history was obtained and was not found to be significant.Upon examination, the visual acuity was 20/20 in both eyes with unremarkable ocular examination. The patient had a right lower lid mass in the medial half of the palpebral conjunctiva measuring 20 mm × 20 mm. The mass was painless, non-tender, reddish in color, soft and semi-cystic in consistency with smooth surface that trans-illuminates but with dim content a & b. NoThe patient was consented for an excisional biopsy of the mass, which was completely excised by an experienced oculoplastic surgeon through conjunctival approach under local anaesthesia within one week of his initial presentation. During excision the lesion was causing remarkable distention of the lower canaliculus without destruction. Probing was done and showed patent nasolacrimal duct, and mini Monoka stent was inserted through the right lower punctum, and the wall of the distended lower canaliculus was trimmed off and sutured over it. Inspection of the excised lesion was suggestive of cavernous hemangioma and the specimen was sent for histopathological examination. The patient tolerated the procedure well and was discharged the following day on topical medications c. He shaOn gross examination the lesion measured 2 × 1.8 × 0.4 cm. Cross sectioning revealed a solid and pale cut surface. Histopathologically, the conjunctival epithelium was thin and shows metaplasia with loss of goblet cells. The substantia propria showed diffuse infiltration mostly by mature neoplastic grade 1 cells resembling normal plasma cells with rare mitotic figures and few immature, binucleated and giant plasma cells d. ImmunoMM work up was advised and the patient was referred to the hematology/oncology team for systemic workup. However, the patient was lost to follow up. He was approached by the treating team and his particular condition was fully explained. However, taking into consideration that he is far from his family, the patient elected to go back to his home country for further investigations. This case report has been prepared in compliance with the updated SCARE 2018 criteria . A gener3Primary extramedullary plasmacytomas are generally rare and orbital involvement is even less likely. Solitary plasmacytoma can be found in patients who have a history of MM or in asymptomatic cases where the diagnosis is not yet established, as in our patient ,3. HowevThe demographics of patients with EMP orbital involvement are similar to the typical MM with an age ranging between 40 and 70 years of age and almost equal male to female ratio ,6. HowevGrossly, the lymphoplasmacytic tumors appear soft with poor circumscribed borders, unlike our case which had well-defined lesion confused as a cavernous hemangioma. Under the microscope, large uniform round plasma cells with displaced nuclei and variable mitotic activity can be seen . The his4Conjunctival primary EMP is very rare, yet should be considered in the differential diagnosis of conjunctival lesions. The lesion can be associated with MM thus systemic workup, follow up and patient counselling are recommended and are essential. Since this can be the first presentation of MM, related morbidity can be significantly reduced through early diagnosis and intervention.A general written informed consent was taken from the patient, which includes permission for anonymous use of information and photos for the purpose of publication.Not commissioned, Editor review.Case reports do not require Ethical approval in our institution. This case report was prepared in accordance with the ethical standards and the Helsinki Declaration. No trial of new drugs or therapy is applicable in this case.A general written informed consent was taken from the patient, which includes permission for anonymous use of information and photos for the purpose of publication.No funding was received.First author: Collection of data, literature search, and manuscript drafting.Second author: Literature search and sharing in the manuscript drafting.Third author: Histopathological examination and final tissue diagnosis.Fourth author: Histopathological review of slides, taking images, and overall review for editing of the manuscript as the corresponding author.Fifth author: Supervision and provision of clinical data and surgical images.This is a case report, which does not require a registry.Dr. Hind Manaa Alkatan.None.Authors do not have any conflict of interest related to this case report.


Lipomas are extremely common benign tumors that occur in a variety of locations. However, lipomas in the bronchus are exceptionally rare and account for a very small number of all bronchial tumors. Diagnosis of an endobronchial lipoma can be challenging at times, as they may present with overlapping symptoms of central airway obstruction, pneumonia, or dyspnea.We present an 82-year-old male with a 40-pack-year history of smoking who presented with recurrent pneumonia and signs of airway obstruction. Imaging studies showed an obstructive lesion. The biopsy sample revealed adipose cells. Eventually, the lesion was successfully resected. The surgical specimen ruled out malignancy and confirmed the diagnosis of lipoma.Timely identification and differentiating a malignant lesion from benign lesions, like lipoma, is crucial to management. Tracheobronchial neoplasms are not very common. Malignant tumors account for most of the obstructive neoplasms in the tracheobronchial tree. Benign tumors arising from mesenchymal tissue are far less frequent -3. HamarAn 82-year-old Caucasian male with a 40-pack-year history of smoking, obesity, diabetes mellitus, hypertension, and three prior episodes of pneumonia within the last 12 months presented to the primary care physician with recurrence of his symptoms of dyspnea on exertion and wheezing. The patient was last treated for pneumonia four months ago. He denied fevers, orthopnea, paroxysmal nocturnal dyspnea, recent sick contact, or travel. He had a minimal non-productive cough. No weight loss was reported. The patient's vital signs, including resting oxygen saturation, were stable. Physical examination was remarkable for wheezing on the right side of his chest. He had no elevated jugular venous pulsation or lower extremity edema. No lymph nodes were palpable. Laboratory studies revealed normal blood counts, cardiac markers, and chemistry panel. Blood gases showed very mild compensated hypercapnia. A chest radiograph was done which revealed no new infiltrate but the resolution of the prior right-sided pneumonia. The patient was prescribed a short-acting bronchodilator, considering his long history of smoking. Upon follow-up evaluation, his symptoms did not improve and he had continued wheezing on the right side of the chest. A computed tomography (CT) scan of the chest was ordered, and the patient was referred to a pulmonologist. The CT scan showed a lesion in the right mainstem bronchus, predominantly fat density, most likely lipoma  of 67%, with mid-expiratory flow reduced to 42% of predicted and minimal, if any, reversibility with bronchodilators. Flow volume loops did show some signs of fixed obstruction  of the chest was performed which also confirmed fat density Figure .The scan also revealed a small defect within the lateral wall of the right mainstem bronchus with an extension of fat into the mediastinum, which limited the option of bronchoscopic resection. The patient underwent further mass resection, mediastinal dissection, and bronchoplasty. The surgical specimen confirmed lipoma with no evidence of malignancy. The patient did well postoperatively with a resolution of his wheezing. When a patient presents with recurrent pneumonia in a particular anatomical region, ruling out an anatomic abnormality or obstructive lesion is crucial. Obstruction or airway narrowing can be extrinsic from compression by neoplasms, lymphadenopathy (infective or neoplastic), and malformations involving big vessels or intrinsic caused by tracheomalacia, stenosis or strictures of the tracheobronchial tree, bronchial tumors, and occasionally foreign body impaction . MalignaDyspnea, chest pain, cough, and post-obstructive pneumonia are the chief presenting signs and symptoms . Lobar cConfirmation is achieved by biopsy or resection. Relieving obstruction and restoring the patency of the airway is the primary objective of therapy. Treatment modalities include endobronchial resection or surgical approaches. Less invasive, endobronchial techniques, like electrosurgical snaring and laser therapy, are the first choice for small lesions < 4 cm) and proximal lesions with no evidence of extraluminal extension on imaging studies (CT and MRI)  cm and p-11. SurgEndobronchial lipomas are rare tumors causing bronchial obstruction. Biopsy and resection are necessary in all cases, regardless of the symptoms, for timely identification and differentiation of malignant tumors from benign ones.


Assessing the cause, severity of bleeding and strategies to control bleeding is crucial. We describe a rare case of a patient who was presented with epistaxis and left ear haemorrhage, as a probable complication of a ruptured internal carotid artery pseudoaneurysm. The massive haemorrhage compelled blood transfusion and clinical intervention. The diagnosis of internal carotid artery (ICA) pseudoaneurysm measuring 2.9 cm x 3.7 cm was concluded by computed tomography. Several coils were used to embolize the internal carotid artery pseudoaneurysm and arrest the bleeding with the guidance of an angiography. Coiling the pseudoaneurysm is highly recommended. Yet, the best methods to completely treat aneurysm are still in question. After the clinical intervention, the patient remained symptom-free and no episodes of bleeding were noted. Pan Afr Med J. 2021;38:239. doi: 10.11604/pamj.2021.38.239.28017Vascular lesions are severe complications caused by an invasive tumour, blood dyscrasia, penetrating trauma or blunt, or iatrogenic origin . PseudoaA 66-year-old woman presented with epistaxis and haemorrhage of the left ear without any obvious inducement for a period of one week. The patient has a 7 year history of hearing loss, mastoiditis, left tympanic membrane perforation and cervical necrotizing fasciitis which prompted a surgical debridement. A pseudoaneurysm was not observed during that time. The coagulation profile revealed that the haemoglobin level on admission was 60 g/L. An otoscopic examination revealed a blood-stained left external auditory canal. The tympanic membrane was intact. A laryngoscope examination revealed blood stained secretions in the posterior pharyngeal wall. Left pharyngeal oedema and a swollen uvula was observed. Neck contrast-enhanced computed tomography (CT) scan showed a slightly high-density shadow visible in the left parapharyngeal space, pharyngeal space and internal carotid artery area measuring 2.9 cm x 3.7 cm . OropharThe patient underwent ICA coil occlusion. The angiogram was obtained to confirm the pseudoaneurysm and check the flow . With thPseudoaneurysm treatment using coil embolization from the external auditory canal is effective and a secure option to usual surgical ligation of the affected artery, as illustrated in our case. No patient has been reported with associated functional impairment after coil embolization procedure. In our case, iatrogenic and injury are the possible causes of the pseudoaneurysm. The diagnosis of pseudoaneurysm should highly focus on the past history of injury and also a physical examination. Physicians should refrain from using a diagnostic needle aspiration due to the risk of bleeding and the related dilemma of dealing with such bleeding in an orifice setting.Contrast-enhanced computed tomography (CT) can illustrate a vascular enhancement with a round lesion. It can outline the site and degree of the lesion and also disclose any coexisting pathologies which cannot immediately be diagnosed clinically. Still, CT has inadequate detective sensitivity in some cases due to artefact from metallic fragments. Apart from the limitations of a CT scan, we still pursue to utilize it as a basic screening option ,4. The cEndovascular, surgical and conservative management is the treatment options for the pseudoaneurysm . We opteAn ICA pseudoaneurysm should be taken into consideration if there is extreme epistaxis and a past history of a neck injury, even though it occurred many years ago. Pseudoaneurysm from the external auditory canal and its branches are rare in the head and neck. This case illustrates that a pseudoaneurysm can be treated by coil embolization in a secure, fast and beneficial manner.