Craniopharyngiomas represent a rare group of intracranial tumors that often arise in the sellar/suprasellar region of the brain. Adamantinomatous craniopharyngioma is significantly more common than papillary craniopharyngioma. The former most often arises in children whereas the papillary craniopharyngioma is mainly limited to adults. We present the case of a 34-year-old female with visual disturbances and other vague complaints who was found to have a large lobulated sellar mass on neuroimaging studies. She was subsequently diagnosed with an adamantinomatous craniopharyngioma after undergoing transsphenoidal resection. We discuss the patient’s clinical, radiological, and pathological findings in correlation with the current literature and recommendations regarding this type of tumor. Given that adamantinomatous craniopharyngioma rarely presents in adulthood, especially in middle-aged adults, this case is considered rare, and we hope to increase awareness to include adamantinomatous craniopharyngioma in the differential diagnosis for sellar lesions in this age group. Craniopharyngiomas are rare, benign tumors that arise from the embryonic remnants of Rathke’s pouch. They include two distinct tumor entities, namely, adamantinomatous craniopharyngiomas (ACP) and papillary craniopharyngiomas (PCP) . TypicalThe typical manifestations of craniopharyngioma are a result of mass effects on nearby structures and include headache, vision changes, and numerous endocrine disturbances. The World Health Organization (WHO) Classification of CNS Tumours considers craniopharyngioma (both ACP and PCP) as CNS WHO grade 1 , althougWe describe a rare case of ACP in a middle-aged adult. We discuss the radiological, pathological, and clinical importance of this case while supporting the current literature and hope to increase awareness and knowledge for clinicians encountering this entity in the future.A 34-year-old female presented to the emergency room upon recommendations from her ophthalmologist due to ongoing  and now worsening blurry vision. Two weeks prior, she had numerous teeth extracted and noticed an acute worsening of her vision. Chronic ongoing issues included amenorrhea around age 19, weight loss of 15 pounds (2.3 kg) over several months , dizziness upon standing, frequent severe, throbbing, mostly right-sided headaches, and an achy pain over her forehead for two to three months, unique from the previously described headaches, unresponsive to any medications.Her medical history was significant for anxiety and migraines, treated with daily venlafaxine. Upon review of systems, she denied recent illness, shortness of breath, nausea, vomiting, fever, or chills. The physical examination was unrevealing. Ophthalmology consultation identified significant visual field restrictions in the left superior temporal, inferior temporal, and superior nasal areas. Extraocular movements were intact bilaterally. Visual acuity was found to be 20/200 on the right. The left side was evaluated with a hand motion. All other visual tests were within normal limits.Initial computed tomography CT is demonstrated below Figures , 2. MagnEndocrine and neurosurgery teams were consulted. The patient began taking hydrocortisone at physiologic doses which increased to stress doses at the time of surgery due to a low morning cortisol level. Additional endocrine testing revealed elevated prolactin and low insulin-like growth factor 1. Thyroid-stimulating hormone, luteinizing hormone, and follicle-stimulating hormone levels were within normal limits. The patient underwent transsphenoidal resection of the tumor. Intraoperatively, the tumor was found to be heterogeneous with both soft and firm areas; it completely filled the sphenoid sinus, and invaded the cavernous sinus walls, predominantly on the left side. The compressed pituitary gland and stalk were identified and preserved during the surgery. Postoperatively, the patient reported subjective improvement in her vision the same day.Pathologic evaluation of the tissue specimen revealed an adamantinomatous craniopharyngioma with typical basaloid palisaded epithelium, stellate reticulum, areas of wet keratin, and multiple cystic spaces, some containing mucinous material Figures , 7. MicrACPs are extremely rare in adults, with most adult cases presenting over 50 years of age ,10. As tIn an epidemiological study in the United States, PCPs were diagnosed in 5.5% of patients aged 0-29 years old, 30.6% in 30-59 years old, and 30.4% in patients over 60. PCP was found to be more common in adults; however, ACP was significantly more common overall when compared to its papillary counterpart. In addition, the incidence of craniopharyngioma was found to be the greatest amongst Blacks followed by Whites, Asian or Pacific Islanders, and American Indian/Alaska natives. There was no difference in incidence between genders [Craniopharyngiomas typically arise in the suprasellar area of the brain and vary in size. Tumors that have both a sellar and suprasellar component, as in our case, comprise around 53-75% of all cases . There aFrom a neuroimaging standpoint, the lobulated shape is a reliable feature in helping differentiate between ACP and PCP . LobulatCraniopharyngiomas often comprise cystic fluid to some degree. Therefore, their signal intensity on MRI can vary. Typically, solid portions of the tumor have high signal intensity on T1-weighted images. This is thought to be secondary to high protein content (motor oil cysts), hemorrhage, cholesterol, or a few calcifications . AccordiCalcifications are a defining feature of ACP and are more common in pediatric cases . CalcifiThe typical histopathological findings of the adamantinomatous craniopharyngioma include “squamous epithelium disposed in cords, nodules, and irregular trabeculae bordered by palisaded columnar epithelium” . Our casThe patient described numerous chronic abnormalities such as changes in menstruation, weight loss, dizziness, and headaches when she presented for evaluation. Although the etiology of these complaints had yet to be determined, the craniopharyngioma likely served as the underlying common source. Our patient also developed transient diabetes insipidus after her transsphenoidal resection which was successfully treated with DDAVP. She currently requires thyroid replacement therapy, a new medication addition after resection. A study done in 2019 found that the effect of the tumor on hypothalamic dysfunction, mass effect, and incidence of central diabetes insipidus was found to be lower in the adamantinomatous group when compared to PCP. This remained true whether the surgery had been completed or not. However, postsurgery hypothalamic and pituitary dysfunction and incidence of central diabetes insipidus were increased significantly for both tumor types. More than half of the patients typically complained of vision impairment at diagnosis, and 41-48% reported postoperative improvement in vision , as is tAlthough there have yet to be prospective treatment trials, surgical resection is typically the first-line treatment of craniopharyngiomas. Given that ACP tends to adhere more to its surroundings and can surround blood vessels, this poses a neurosurgical challenge and can limit cases attaining gross total resection . AdjunctStudies have shown that recurrence rates of craniopharyngiomas are highly dependent on the extent of surgical resection; however, tumors invading the hypothalamus are associated with significant morbidity. There was no difference in five- and ten-year survival rates between patients who have undergone gross total resection versus subtotal resection of the tumors with adjuvant therapy. However, patients who underwent subtotal resection without adjuvant therapy had significantly higher recurrence rates when compared to subtotal resection with advent therapy .β-catenin gene mutations have been documented in more than 70% of ACPs [BRAF V600E mutations have been found in the majority of the PCP cases but have not been associated with the adamantinomatous subtype [CTNNB1 mutations were found in 75-96% of the adamantinomatous subtype [There have been numerous studies evaluating the genetic alterations in craniopharyngioma that could be crucial to future treatment strategies.  of ACPs . BRAF V6 subtype , while C subtype . While s subtype .Craniopharyngiomas are a rare cause of intracranial tumor. ACP is more common overall, and most commonly occurs in children. Of the adult cases, it is rare to see this tumor diagnosed under the age of 50. We presented a rare case of ACP in a 34-year-old female. The patient underwent transsphenoidal resection with the restoration of the majority of pituitary function. This case serves to heighten our awareness of the occurrence of this type of tumor in younger adult patients and emphasizes helpful neuroimaging features, typical pathological findings, surgical techniques, and sequelae.


Glucagonoma is a rare neuroendocrine tumor (NET). Most glucagonomas are in the tail or body of the pancreas and are diagnosed at a metastatic stage. We report a case of an early recurrence after surgical resection of a glucagonoma and its management.We present a case of a 44-year-old female patient with no medical and surgical history, operated on in May 2018 for pancreatic glucagonoma revealed by skin necrolytic migratory erythema. The patient was regularly monitored by clinical exams and CT scans. In December 2020 (31 months postoperatively), we noticed the recurrence of the cutaneous lesions.Admission laboratory measurements demonstrated hyperglycemia as well as elevated blood Glucagon levels. Explorations showed 3 retro-pancreatic lesions. Based on these findings, we concluded that it was a recurrence of her glucagonoma. The patient was operated on by median laparotomy We performed a warshow's procedure.Pathology confirmed the endocrine nature of the 3 nodules. We are currently 6 months behind the surgery. The examination is strictly normal with no recurrence of the skin lesions so far.Surgical resection on a recurrent glucagonoma is what is unique in our case as we haven't found any case in the literature to our knowledge.What is also unique about our case is both the local aspect of the recurrence and the multiplicity of the tumors observed as multiple nodules around the tail of the pancreas. These lesions were not metastatic lymph nodes as confirmed by pathology. Probably it was an effraction of the big tumor at enucleation.Due to its rareness, there is no clear consensus on the management of glucagonomas therefore we chose to write our case in order to further enrich the literature to achieve one-day guidelines for glucagonomas treatment. •Glucagonoma is rare.•NME is considered as an important symptom.•Surgery is the only permanent solution.•Recurrence is frequent therefore follow up is important. Most glucagonomas are in the tail or body of the pancreas and are diagnosed at a metastatic stage. We report a case of an early recurrence after surgical resection of a glucagonoma and its management.This case report has been reported in line with the SCARE Criteria .2We present a case of a 44-year-old female patient with no personal medical and surgical history nor family history of multiple endocrine neoplasia, operated on in May 2018 for pancreatic glucagonoma revealed by skin necrolytic migratory erythema. The patient had a simple enucleation of the mass. Pathology concluded to an intermediate grade (G2) pancreatic neuroendocrine tumor with a Ki67 at 7%. The postoperative period was uneventful, and all cutaneous lesions disappeared.The patient was regularly monitored by clinical exams and CT scans. In December 2020 (31 months postoperatively), we noticed the recurrence of the cutaneous lesions.On admission, she had a good general condition, stable vital signs, no abdominal mass but with skin findings suggestive of necrolytic migratory erythema.Admission laboratory measurements demonstrated hyperglycemia  as well as elevated blood Glucagon levels . The serum levels of Chromogranin A, were normal (31ng/ml).MRI and CT-scan showed 3 retro-pancreatic lesions of 10, 13, and 16 mm in diameter. The octreotide scan was normal. Based on these findings, we concluded that it was a recurrence of her glucagonoma.The patient was operated on 28-05-2021 by an attending surgeon at our department by median laparotomy . On explWe performed distal pancreatectomy with spleen preservation and resection of the splenic vessels as shown in Warshow's technique . The posPathology confirmed the endocrine nature of the 3 nodules and concluded to an intermediate grade (G2) pancreatic neuroendocrine tumor with a Ki67 at 5%. We are currently 6 months behind the surgery. The patient has no functional complaints. The examination is strictly normal with no recurrence of the skin lesions so far.3Glucagonoma is a rare neuroendocrine tumor (NET), with an incidence of one in 20 million people. More recent reports suggest that they represent 2–7% of pancreatic neuroendocrine tumors. Most glucagonomas are in the tail or body of the pancreas [As it is pointed out in our case the necrolytic migratory erythema is a common symptom but lacks specificity which led to a late discovery of this tumor. Indeed, our patient was explored for chronic dermatitis for 6 years before the final diagnosis was found. This is concurring with the literature as a recent series found a median time of 39 months from onset of symptoms to diagnosis [What we find interesting in our case is the early recurrence of the resected glucagonoma.Indeed, our patient underwent surgery one year prior to its transfer to our department. The patient had a history of enucleation of a 14cm glucagonoma. The surgery, in this case, was insufficient as it is well known that enucleation is only indicated in small NET's [Surgical resection on a recurrent glucagonoma is what is unique in our case as we haven't found any case in the literature to our knowledge. The only reported case of recurrent glucagonoma wasn't resected due to vascular considerations. Other reported cases of recurrence were of metastatic glucagonoma .What is also unique about our case is both the local aspect of the recurrence and the multiplicity of the tumors observed as multiple nodules around the tail of the pancreas. These lesions were not metastatic lymph nodes as it was confirmed by pathology. Probably it was an effraction of the big tumor at enucleation.To confirm our curative procedure, clinical assessment and dosage of endocrine markers should be practiced. We will also perform CT scans every 3–9 months as suggested in the 2016 ENET's consensus guidelines.4Due to it’s rareness there is no clear consensus in the management of glucagonomas therefore we chose to write our case in order to further enrich the literature to achieve one day guidelines for glucagonomas treatment.There is no ethical committee in our country (Not applicable for this manuscript).No sources of funding.All authors contributed to the study design.All authors declare that they have no any conflicts of interest.1.Name of the registry:2.Unique Identifying number or registration ID:3.Hyperlink to your specific registration (must be publicly accessible and will be checked):Ouadi Yacine.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not commissioned, externally peer reviewed.


Endoscopic endonasal approach (EEA) techniques have evolved significantly in recent years, with better techniques guaranteeing better surgical results in the treatment of sellar and parasellar region pathologies. Although the complications associated with the EEA have been widely described, with cerebrospinal fluid fistulas being the most commonly reported, some rare complications, such as vasospasm after surgery in extended approaches, turn out to be poorly understood. Here, we describe a case of middle cerebral artery delayed vasospasm associated with the resection of a non-functional pituitary adenoma via an EEA. The implementation of endoscopic endonasal approach (EEA) techniques for the treatment of lesions in the sellar and parasellar regions has increased considerably in recent decades, allowing a better understanding of this approach and critical technical advances in this technique. Likewise, this advance has made it possible to establish and understand better the complications associated with this approach. Vasospasm with delayed cerebral ischemia associated with EEA approaches is a rare complication occurring mainly due to the manipulation of suprasellar cistern structures. Due to the insufficient number of reports on this complication in the literature , vasospasm associated with the EEA is described using the principles of vasospasm associated with non-traumatic subarachnoid hemorrhage (SAH) -5. Here,A 35-year-old female with no previous medical records presented to our hospital with a two-month continuous headache and progressive visual loss history. In her initial neurological examination, an obvious visual acuity loss in the right eye (20/40 using Snellen cards) and a bitemporal campimetry defect were noted. Her laboratory tests showed secondary hypothyroidism and hypocortisolism. Initial magnetic resonance imaging (MRI) showed a sellar lesion compatible with a pituitary macroadenoma of Hardy-Wilson 2C and Knosp II classification Figure . A complThe patient attended the emergency department 11 days after surgery reporting an acute onset of right hemiparesis and global aphasia. Diagnosis of an ischemic stroke was made with a 20-point score in the National Institutes of Health Stroke Scale (NIHSS). A cranial angio-tomography was performed finding vasospasm of the left internal carotid artery (ICA) segments C5-C7, left anterior cerebral artery (ACA) segment A1 and left MCA segment M1. The perfusion tomography showed a decrease in the cerebral blood volume (CBV) and blood flow velocity (CBF) in the frontal opercular cortex as well as in the left precentral gyrus with an Alberta Stroke Program Early CT Score (ASPECTS) score of 7 Figure -4. We inIntracranial Doppler ultrasonography (USG) was performed showing increased flow velocities in the left ICA and MCA confirming the diagnosis of moderate vasospasm with delayed cerebral ischemia. The patient was admitted to the ICU and showed clinical improvement in motor and language deficits, being discharged seven days later with a modified Rankin score of 1. The follow-up angio-CT showed resolution of the vasospasm a month after discharge.The pathophysiology of vasospasm and associated delayed cerebral ischemia are well studied and are known to be an important cause of morbidity and mortality in aneurysmal SAH . VariousAnother theory proposed that in large-volume tumors with hypothalamic involvement, dysfunction or direct compression damage to the hypothalamus could cause a transient catecholaminergic response with subsequent production of spasmogenic substances (e.g. endothelin or angiotensin); likewise, the presence of polyuria due to the manipulation of the hypothalamus or pituitary stalk with subsequent hypovolemia would contribute to the establishment of ischemic damage secondary to vasospasm -15.In our case, the patient presented a favorable early evolution without any of the previously mentioned factors explaining the development of vasospasm and cerebral infarction; in addition, the event occurred 11 days after surgery, while in other reports, the average time to the onset of vasospasm after transsphenoidal surgery was 8 days ,16-17. OIn the cases reported in the literature, the anterior circulation was mainly affected, particularly, the ACA, followed by the proximal MCA and the supraclinoid ICA; posterior circulation vasospasm was reported less frequently  -5. In thDue to the low number of cases reported, there is currently no management consensus for the treatment or prevention of vasospasm in such cases; hence, the established management guidelines for vasospasm in aneurysmal SAH must be used . In thisThe standard management of these cases should include the use of oral nimodipine as a calcium antagonist and controlled hypertension to improve cerebral perfusion . The durEEA-associated vasospasm continues to be an uncommon and poorly understood entity that can have severe repercussions in postoperative patients, even in late stages, with considerable morbidity. In our case, the late presentation of this complication was a very rare finding, which is why we recommend a close follow-up of these patients during the first three weeks after surgery. Risk factors associated with surgery, as well as vasospasm predisposition post-surgery, must be detected in a timely manner to allow for adequate treatment in this select group of patients. More studies are required to establish a specific treatment plan for this complication. For now, the management guidelines established for aneurysmal SAH should be used. In our case, the early initiation of vasospasm treatment granted an excellent clinical outcome, achieving an almost total recovery of the patient.


Lipomas are common benign tumors, which are usually located in the subcutaneous tissue. It is relatively rare for lipoma to occur in the intrathoracic cavity, and it is clinically difficult to distinguish it from liposarcoma. We present the case of a 72-year-old man with a chest wall tumor preoperatively diagnosed as liposarcoma, with tumor enlargement with radiological image change to heterogenous and 18F-fluorodeoxyglucose positron emission tomography uptake. The tumor was resected along the chest wall, lung and diaphragm because of dense adhesions. The tumor was diagnosed as lipoma with fat necrosis and inflammatory changes. Lipomas of the intrathoracic cavity are relatively rare and are reported to be located in the intracardiac, pleural and intramuscular regions. There are few reports of chest wall lipomas, and it is always clinically difficult to distinguish them from liposarcomas . Herein,in situ hybridization examination for murine double-minute 2 was negative. Based on these findings, a chest wall lipoma was diagnosed. The post-operative course was uneventful. The patient was followed up for 24 months without evidence of recurrence.A 72-year-old man with a history of hypertension and left renal cancer (Stage I) was referred to us with a mass in the right chest wall on post-operative follow-up chest computed tomography (CT) for renal cancer. He had a smoking history of 20 pack-years and had no exposure to environmental fumes or dust. Physical examination results were unremarkable. The laboratory findings were within normal limits. Pulmonary function tests and cardiovascular examinations revealed normal results. Chest CT revealed mixed density mass (8.0 × 5.0 × 3.0 cm) located in front of thoracic wall in the third to sixth right intercostal space. The tumor can be revealed as thoracic wall fat (7.8 × 4.8 × 1.2 cm) on CT 1 year before , and it was progressively increased in size and the density changed . MagnetiLipomas are common benign tumors, and during treatment of deep-seated lipoma, differential diagnosis between well-differentiated liposarcomas should be considered  . LipomaSeveral critical radiological image findings have been reported for the differential diagnosis. Lipomas are discrete, encapsulated, homogeneous fatty masses on CT and MRI. On the other hand, thickened or nodular septa (>2 mm), prominent foci of high T2 signal and prominent areas of enhancement on MRI have been reported to be features suspicious of liposarcoma , 6. In aIn the present case, tumor enlargement with heterogenic change of the density, thickened septa >2 mm, prominent foci of high T2 signal and prominent areas of enhancement on MRI were seen on preoperative CT and MRI. In addition, the SUVmax of FDG-PET was higher than that of simple lipoma (SUVmax = 3.78). These findings led to a misdiagnosis for liposarcoma. Furthermore, intraoperative findings of dense adhesion between the tumor and surrounding structures strengthen our suspicion of liposarcoma. We could not find another case of intrathoracic lipoma of the chest wall with density change on radiological image and dense adhesion between the surrounding structures.Pathological findings in this case showed fat necrosis and inflammatory changes in the marginal area of the tumor, with foamy macrophages, and multinucleated giant cells. These changes may cause the malignant featured images, such as heterogenic density, enhancement on MRI and high SUVmax of FDG-PET. In addition, these changes may cause dense adhesion. Several lipomas with fat necrosis has been reported , but nonIn conclusion, we encountered a case of chest wall lipoma misdiagnosed as liposarcoma, with malignant features based on radiological image and intraoperative findings, and performed wide local excision. Fat necrosis and inflammation may be the cause of this clinical course.


Sarcocystis is an intracellular protozoan parasite that manifests as a sarcocyst within the muscle fibers of an intermediate host. Sarcocystis commonly affects animals; in fact, cases of sarcocystosis involving human hosts are rare and often undiagnosed. The two types of Sarcocystis species that may infect and utilize humans as a definitive host are S. hominis and S. suihominis, both of which predominantly involve the gastrointestinal system. The low prevalence of intestinal sarcocystosis among humans is rarely accompanied by symptoms, except for individuals who ingest large amounts of the parasite. This study presents an unusual case of tongue sarcocystosis, a site that has not been previously reported, that was treated successfully with oral Albendazole for two weeks. Sarcocystis is a genus of intracellular protozoan parasites that predominantly affect mammals and reptiles. Sarcocystis spp. fall under the family Sarcocystidae and the phylum Apicomplexa. S. hominis and S. suihominis use humans as definitive hosts and require two hosts to complete their life cycle, which includes sexual reproduction in the intestine and asexual reproduction in the tissue [Sarcocystis [Sarcocystis spp. infections in humans are usually asymptomatic and self-limited [e tissue . The precocystis . Sarcocy-limited . To date-limited . This stA 36-year-old male with an unremarkable medical history presented to our outpatient clinic; his chief complaint was progressively enlarging left lateral tongue swelling for the past four months. It was accompanied by odynophagia and pain while chewing. He denied dysphagia, dyspnea, or any constitutional symptoms. The patient had been working in the latex industry for six years with no history of tobacco smoking or alcohol consumption. Intraoral examination revealed soft swelling of the left lateral tongue measuring approximately 2 cm x 2 cm Figure ; additioOther areas of the oropharynx and oral cavity were normal, and no cervical lymph nodes were palpable. A biopsy of the lesion was performed under local anesthesia, and a histopathological examination Figure  revealedSarcocystis spp. in Nepal; consequently, most human infections occurred by ingesting raw or inadequately cooked meat of infected animals [Human skeletal muscle sarcocystosis is a rare condition that is diagnosed based on clusters of clinical criteria, including persistent myalgia, episodic weakness, subcutaneous nodules, and dermatomyositis. Laboratory findings of eosinophilia and elevated creatine kinase are also indicative of sarcocystosis. In certain cases, such clinical findings are linked to a history of traveling to known endemic locations. Moreover, sarcocysts in muscle biopsies can be readily identified by microscopic examination of hematoxylin- and eosin-stained sections . Tests s animals .S. hominis and S. suihominis, both of which are acquired by eating undercooked parasite-infected meat. This parasite reproduces sexually in the human intestine, which can cause symptoms of acute gastroenteritis; however, most infections remain asymptomatic [Sarcocystis spp. by ingesting oocysts or sporocysts in contaminated food or water [As previously mentioned, humans are one of the definitive hosts for ptomatic . Humans or water . In our Sarcocystis spp. infections. Such infections can be prevented by thoroughly cooking or freezing meat to kill the bradyzoites embedded in the tissue. Despite the lack of standard treatment regimens [Sarcocystosis is usually a self-limited and asymptomatic disease. To date, there are no definitive or specific treatment guidelines for human regimens , our patIn summary, sarcocystosis in humans usually affects the gastrointestinal tract and rarely involves the skeletal muscle. Our report highlights the importance of otolaryngologists remaining vigilant and scrutinizing patients with a history of traveling to a region where sarcocystosis is endemic. While this disease is usually self-limited, treatment with oral Albendazole should be considered for symptomatic cases.


Soft tumors of the penis are extremely rare in childhood and adolescence. Because there are no guidelines, each such case raises embarrassment over the extent and degree of aggressiveness of the diagnostic and therapeutic procedures. Herein, we describe a case of a teenager with a penile myointimoma along with a review of the literature. So far, only 10 cases have been reported in this age group.The 15-year-old boy presented with a 6-months history of a slowly growing, palpable firm nodule in glans penis. Physical examination revealed a palpable, well circumscribed, firm, whitish painless mass, around 1 cm in diameter within the glans. Ultrasound revealed hypoechogenic, hypoperfused poorly defined area inside the glans. MRI did not confirm any other pathologic mass inside the glans and corpora cavernosa. An excisional biopsy was performed; its immunohistological features indicated myointimoma.Given the rarity of this benign entity, the main importance is to distinguish myointioma from more clinically aggressive neoplasms. The key to a correct diagnosis is a careful histological examination, including immunohistochemistry. Local excision is safe and effective treatment modality. Myointimoma, also known as angiocentric myofibroblastic tumor, is a rare benign soft tissue neoplasm derived from intimal cells of the vascular spaces of the corpora cavernosa of the penis, histologically characterized by multinodular / plexiform myofibroblastic proliferation within the vascular spaces of cavernous bodies. The term myointimoma was first introduced by Fetsch et al.  in 2000 A 15-year-old Caucasian boy presented with a 6-months history of a slowly growing, palpable firm nodule within glans penis. Clinically he was completely asymptomatic and voided freely. The patient did not report any history of trauma, systemic connective tissue diseases or other autoimmune disorders. On physical examination, there was a palpable, well circumscribed, firm, whitish painless mass, around 1 cm in diameter within the glans . Ultrasound revealed hypoechogenic, hypoperfused poorly defined area inside the glans Fig. . MRI didWe obtained a macroscopically pale tissue sample measuring 10 × 8 × 5 mm Fig. . On the Due to the benign nature of the lesion, we did not perform staging for distant metastases and simply performed an outpatient follow-up. Three years after the excision, there is no local recurrence, no urethral stricture and a cosmetic appearance is good Fig. .This case report refers an additional case to the 10 previous reported cases of myointimoma in children and adolescents –5, . Desmin may be absent or show only focal reactivity. There is no reactivity for S-100 protein, CD31, CD34, ERG, epithelial membrane antigen (EMA) or neuron specific enolase (NSE). The plexiform growth pattern can be found in plexiform histiocytic tumor (PFHT) . Unlike The myointimoma structure may resemble myofibroma, a more common tumor in children. In contrast, it does not exhibit the exclusive intravascular growth; the growth is rather concentric around the small vessels. The tumor is composed of oval or spindle myoid cells . MyoperiEpithelioid hemangioma and hemangioendothelioma can be distinguished from myointimoma by immunostaining as the endothelial nature of the lesional cells can be confirmed by CD31, CD34 and ERG positivity. Another structurally similar pathology is a late phase of intravascular fasciitis (intravascular nodular fasciitis). Histologically, intralesional inflammatory cells between spindle myofibroblast cells, mucoid pools, a less compact stroma with more eosinophilic hyalinization, and obvious mitotic figures were observed [Both clinician and pathologist must be aware of this rare benign entity. The key to a correct diagnosis is a careful histological examination of the specimen, including immunohistochemistry. Local excision is safe and effective treatment modality.


Carcinosarcoma is a distinct neoplasm consisting of bidirectional differentiation toward epithelial and mesenchymal cells. Bladder localization is rare and the association with a rahbdomyoblastic component is exceptional. Few cases of bladder carcinosarcoma with rhabdomyoblastic differentiation have been reported in the literature. We present a case of a bladder carcinosarcoma in a 68-year-old man who presented with terminal hematuria and discuss difficulties of diagnostic and treatment. Accepted: 19 April 2022</p>Carcinosarcoma is a rare tumor . The diaA 68-year-old man, diabetic, presented for terminal hematuria associated with pelvic pain. His physical examination was normal. Ultrasonography and chest and abdominopelvic computed tomography (CT) scan revealed a urinary bladder tumor of 5 × 5 cm over the dome and the anterior wall with normal upper tract . Cystosc. The term ‘carcinosarcoma’ is used to refer to a malignant neoplasm whose overt epithelial histology is mixed with distinctive mesenchymal histology with heterologous elements [Carcinosarcoma of the bladder is a high-stage tumor at diagnosis and has a poor prognosiselements . The preelements . The hiselements . In our elements . These telements . Bladderelements . Howeverelements . Neoadjuelements . Howeverelements ,5. In thBladder carcinosarcoma is an aggressive tumor and has a poor prognosis. Radical cystectomy represents the best therapeutic option with better overall survival. Sometimes, external radiotherapy can be offered.The authors declare that there are no conflicts of interest regarding the publication of this article.We have no financial sources for our research.


Cloaca is a rare, complex malformation encompassing the genitourinary and anorectal tract of the female in which these tracts fail to separate in utero, resulting in a single perineal orifice. Prenatal sonography detects a few cases with findings such as renal and urinary tract malformations, intraluminal calcifications, dilated bowel, ambiguous genitalia, a cystic pelvic mass, or identification of other associated anomalies prompting further imaging. Multi-disciplinary collaboration between neonatology, pediatric surgery, urology, and gynecology is paramount to achieving safe outcomes. Perinatal evaluation and management may include treatment of cardiopulmonary and renal anomalies, administration of prophylactic antibiotics, ensuring egress of urine and evaluation of hydronephrosis, drainage of a hydrocolpos, and creation of a colostomy for stool diversion. Additional imaging of the spinal cord and sacrum are obtained to plan possible neurosurgical intervention as well as prognostication of future bladder and bowel control. Endoscopic evaluation and cloacagram, followed by primary reconstruction, are performed by a multidisciplinary team outside of the neonatal period. Long-term multidisciplinary follow-up is essential given the increased rates of renal disease, neuropathic bladder, tethered cord syndrome, and stooling issues. Patients and families will also require support through the functional and psychosocial changes in puberty, adolescence, and young adulthood. A cloacal malformation is characterized by a single perineal orifice where the urologic, gynecologic, and gastrointestinal tracts meet. It represents one of the most complex congenital anomalies of the pelvic organs in females, with an incidence of 1 in 50,000 births ,3. The cPrenatal imaging findings associated with cloaca may be subtle. An accurate diagnosis can be made but requires a high degree of suspicion by knowledgeable fetal radiologists working alongside surgeons experienced with cloaca. The benefit of prenatal diagnosis, when possible, is the opportunity for providers to counsel parents in advance of delivery. Thorough counseling includes education regarding the anatomic variations in cloacal malformations, a discussion of any concomitant anomalies, outlining the priorities of neonatal management, and refining delivery planning to include immediate transfer to a tertiary care center. Early diagnosis allows families time to meet with experts in cloacal reconstruction who can accurately detail the surgical reconstruction and long-term functional outcomes from the colorectal, urological, and gynecologic perspectives. Prenatal diagnosis may also minimize potential complications by allowing time to develop a unified multidisciplinary plan for neonatal management that prioritizes key interventions and avoids delays in care due to unnecessary evaluations .The most common findings on prenatal ultrasound (US) in patients with cloaca are abdominal/pelvic cystic masses, hydronephrosis, hydroureteronephrosis, oligohydramnios, ascites, and distended or obstructed bowel . AdditioThe differential diagnosis for a female fetus with an abdominal cystic mass should include ovarian cysts, enteric duplication cysts, abdominal cystic lymphangioma, intestinal atresia, megacystis microcolon intestinalis, and hydrocolpos due to imperforate hymen or urogenital sinus that leads to a distended vagina filled with fluid. The likelihood of a cloaca diagnosis in such a patient increases when two or three cystic structures can be identified—a bladder and a distended single vagina, a septate distended vagina only, without a visualized bladder, a bladder and distended septate vagina, or a distended distal rectum .Ovarian cysts are the most common cystic lesions seen in female fetuses, but these are adnexal, lateral structures that occur with normal gastrointestinal and renal anatomy, and therefore can be distinguished from patients with cloaca. Bischoff et al. proposed the following imaging algorithm to work through the differential diagnosis of a cystic pelvic mass in a female fetus  8]. A m. A m8]. When US findings are suspicious for cloaca, a fetal MRI performed in the third trimester can delineate the anatomy of the fetus and confirm the diagnosis. MRI findings suggestive of cloaca include dilated distal bowel that does not extend below the bladder, lack of meconium signal at the expected rectal location, renal anomalies (most commonly hydronephrosis), abnormal appearance of the bladder, and hydrocolpos . An incrThe exstrophy–epispadias complex represents a more severe spectrum of congenital urologic, gynecologic, and gastrointestinal tract anomalies, which typically includes an abdominal wall defect with marked pubic diastasis. There is a high rate of associated spinal dysraphism, such as myelomeningocele. The exstrophy–epispadias complex can range from a mild defect of epispadias in 30% of patients, bladder exstrophy in 60%, or the most severe form of cloacal exstrophy in 10%, also known as the omphalocele–exstrophy–imperforate anus–spinal anomaly complex (OEIS) . CloacalWhen a prenatal diagnosis of a cloacal malformation or cloacal exstrophy is made, caregiver counseling is a crucial intervention to assuage parental concerns. Devising a strategy for effective and tactful delivery of this difficult diagnosis is a key first step in counseling.The advantages of a prenatal diagnosis include the ability to discuss the viability of the fetus, offering the option of terminating the pregnancy, and establishing parental expectations of the prognosis for bowel, urinary, and gynecologic function . In addiAlthough in-person counseling may be ideal, virtual visits allow families to receive expert counseling even when centers of excellence are not local, and web-based protocols have been described to help providers accomplish this ,23,24,25This should include a discussion of the suspected anatomy of the urethra, vagina, and colon connecting to a single perineal orifice, as well as the relative severity of cloaca compared to other ARMs in general. The provider should discuss the higher rate of associated anatomic anomalies and discuss patient-specific findings, such as VACTERL-associations, and the need for additional screening tests in the pre- and post-natal periods, and expectations for bowel and urinary control, and gynecologic function.Routine follow-up with the patient’s primary obstetrician, a maternal–fetal medicine specialist and prenatal sonography with the ability for advanced ultrasonographic monitoring continues through delivery particularly to monitor for oligohydramnios, renal status, and to assist with delivery planning. The frequency of ultrasound monitoring is dependent on the presence of coexisting fetal anomalies and/or oligohydramnios. The family can meet with the colorectal surgery, urology, gynecology, and neonatology teams for individualized counseling about perinatal interventions and long-term functional expectations.The diagnosis of cloaca alone does not necessitate a preterm delivery, preclude a vaginal delivery, or require delivery at an institution with advanced neonatal support. However, complicating factors such as coexisting fetal anomalies or severe oligohydramnios may warrant early and advanced delivery planning. Those with a high cardiopulmonary risk profile, for example, may be best suited for delivery at a hospital with level III or IV neonatal intensive care unit (NICU) capabilities. For others, the delivery may occur at the hospital of the family’s choice with a plan for expeditious transfer to a tertiary hospital with a NICU and subspecialty radiologic and surgical services. In those with anticipated spinal anomalies, particularly neural tube defects, or with severe abdominal distention, cesarean delivery may be recommended. However, for a majority of those with cloaca, vaginal delivery is safe and feasible . DeliverAfter delivery, the priorities of care are to ensure respiratory and hemodynamic stability, including assessment for possible intubation. Additional examination and testing will be performed as part of a unified plan among surgical teams to triage the necessary interventions to achieve cardiopulmonary stability and secure egress of stool and urine, including drainage of a hydrocolpos, if present. Monitoring of renal function and management of azotemia may also be required. Until surgical interventions are completed, the newborn may remain nil per os (NPO) with parenteral nutrition for several days to weeks; therefore, necessitating a plan to store breastmilk if the mother plans to breastfeed.Expected criteria required for discharge home are discussed, including appropriate recovery time following surgical interventions, a plan for oral or enteral feeds, regular stooling and colostomy care teaching, and adequate voiding or proficiency in intermittent catheterizations. Infants with cloaca requiring these maneuvers generally spend 2 to 3 weeks in the NICU, with a plan to perform reconstructive surgery in 3–12 months, depending on the infant’s growth and condition. The child will see their preferred pediatrician for routine well-visits to check growth and administer vaccines. Surgical subspecialty outpatient visits are scheduled to review interval testing such as sacral X-rays, serial renal ultrasounds, and spinal imaging. Plans for cloacagram and endoscopic evaluation for the delineation of the precise cloacal anatomy before reconstruction are outlined. Early patient-centered discussion of long-term functional outcomes helps to set expectations for families.Routine communication between the caregivers and the high-risk obstetrician facilitates the implementation of the care plan at the delivery center and at the receiving center for the NICU transfer to manage the newborn.Initial resuscitative measures of the neonate focus on achieving cardiopulmonary stability including a potential need for intubation. If a prenatal diagnosis of cloaca is not made, an imperforate anus may be discovered with a newborn exam, or even later in some cases. Vascular access is obtained to collect blood cultures, blood gas, hematologic and metabolic labs, and then to administer intravenous fluids as clinically indicated. Intravenous antibiotics such as ampicillin are recommended for prevention of urinary tract infections (UTIs) and broadened as required by clinical status. The team should prepare for a nasogastric sump tube insertion to evaluate for an esophageal atresia/tracheoesophageal fistula, present in 7–11% of patients with ARM ,26. HeigAn echocardiogram is obtained to assess for cardiac defects, present in up to 40% of patients with ARM, and to determine if hemodynamic consequence may require intervention or additional considerations for general anesthesia administration for operative intervention ,27,28.Proper examination of a newborn perineum is often difficult to perform in a NICU, and the importance of good lighting with proper positioning cannot be overemphasized. Surgical loupes to closely examine the external anatomy can be useful, if available, especially for a premature newborn. The proper technique to perform a female perineal examination is to elevate the edges of the labia upward and outward towards the examiner’s shoulders to aid in the exposure of small, hidden orifices . A clitoA thorough physical exam also evaluates the abdomen for distention and the presence of a palpable lower midline mass, which may represent a hydrocolpos. This should be confirmed with bedside US, along with a check for concurrent hydroureteronephrosis . An abdoInitial attempts to drain the hydrocolpos are performed by passage of a catheter into the common channel, followed by US confirmation of vaginal decompression by the catheter placement. If two hemivaginas divided by a septum are present, which occurs in 25% of cloacal malformations, one must ensure that both vaginas are sufficiently drained . If therWhen the hydrocolpos is resolved but the upper urinary tract dilation persists, a voiding cystourethrogram (VCUG) is helpful in diagnosing vesicoureteral reflux (VUR), ureteral ectopia, a long common channel, or a poorly emptying bladder . It is uUrologic anomalies are very common in patients with ARM. Those with cloacal malformations are especially susceptible, with urologic anomalies reported in more than 80% of cases . Of thesAfter initial stabilization of the patient’s cardiopulmonary status and drainage of a hydrocolpos (if present), the colostomy is created to divert the fecal stream. This can be performed at the same time as other indicated surgical procedures, such as tracheoesophageal fistula ligation or vaginostomy creation, typically in the first 24 to 48 h of life. A cystoscopy or vaginoscopy are not needed at the initial operation if the hydrocolpos and the urine are reliably draining. The ideal location in the colon for the stoma is the descending colon, just distal to the fixation to the left retroperitoneum and abdominal side wall, which helps to prevent prolapse, while leaving sufficient distal length for a future anorectoplasty . In the If a divided colostomy is made, the mucous fistula is created small and flat to avoid prolapse, and sufficiently away from the colostomy with a skin bridge, to allow for ease of pouching of the proximal orifice to prevent skin breakdown and decrease spillover a 35]. T. T35]. TLoop colostomies preserve the blood supply and have the benefit of an easier reversal with shorter operative times ,36. In rA low midline laparotomy may be performed to surgically drain a hydrocolpos at the same time as colostomy creation b. A tubeA transverse colostomy can be associated with increased urinary absorption via the fistula, leading to acidosis, increased prolapse rates, and difficulty distending the distal colon and rectum on contrast imaging a 31]. P. P31]. PAfter a colostomy is created, hydrocolpos is addressed (if present), and a bladder management strategy achieved, recovery can be rapid. Prolonged hospitalization often depends on comorbidities of the heart, lungs, or prematurity.After the colostomy is functioning and the patient is clinically doing well, the patient may be fed. A newborn with a cloaca and a colostomy who is not doing well postoperatively usually has an undrained, obstructed urinary tract, with or without hydrocolpos. Therefore, the first study to perform in such patients should be an US, which can evaluate for hydronephrosis, megaureter, a distended bladder, or hydrocolpos. In the case of a loop colostomy or if both stomas are being pouched together, distal stool spillage may lead to a UTI . AdditioAny remaining components of a VACTERL work-up are completed before discharge, including a spinal US to assess for tethered cord, and plain radiographs looking for vertebral, radial, or other limb abnormalities. Particularly in a patient who struggles to drain their urinary tract, a neurogenic bladder arising from a spinal cord anomaly should be suspected. An inconclusive or definitively abnormal spinal US warrants a consultation with neurosurgery, who will assist with timing the repeat spinal US or MRI. Impaired bladder dynamics may be associated with poor development of the sacrum and severe caudal regression; therefore, dedicated sacral X-rays are performed after 3 months of age, after the sacral bones have ossified . The finIf the Mullerian structures are not inspected during colostomy or vaginostomy creation, they may be evaluated during definitive repair, or if any future abdominal surgery is needed . Greater than 50% of cloaca patients have Mullerian anomalies, and require longitudinal follow-up with a gynecologist ,44,45. FThe patient’s follow-up plan with the primary pediatrician, multiple surgical subspecialists, and outpatient testing are arranged, with precautions to seek urgent medical attention for issues such as fever, feeding intolerance, or decreased stoma output with abdominal distention, respiratory difficulties, lethargy, or purulent drainage with erythema of the surgical sites.Prenatal diagnosis of a cloaca gives providers an opportunity to counsel parents about the likely diagnosis of cloaca, provide reassurance, and set expectations for the perinatal period and long-term functional outcomes. The first day of life focuses on the identification of associated anomalies and the triage of interventions to stabilize the infant’s cardiopulmonary status, divert the stool, secure drainage of hydrocolpos, and manage renal azotemia if present. Concomitant urologic anomalies should be anticipated, along with measures taken to diagnose and manage them in order to prevent renal damage and UTIs. A thoughtful colostomy with a plan in mind for future reconstruction, preserving the blood supply, and minimizing the risk of prolapse or distal spillage is essential. A collaborative model for planning and completing the reconstruction after the perinatal period is ideal to ensure the best outcomes for long-term bowel, bladder, and gynecologic function in these complex patients.


Tongue rhabdomyosarcomas (RMSs) are extremely rare soft tissue tumors in the pediatric age group. The most common reported histopathology type is embryonal. To our knowledge, epithelioid type has not yet been reported in tongue RMSs. We report a case of an eight-year-old boy who presented with a painless tongue mass, and the biopsy demonstrated RMS epithelioid type. Head magnetic resonance imaging (MRI) was performed and showed peripherally enhancing mass with central cystic/necrotic component. Computed tomography (CT) scan of the neck showed involvement of the cervical lymph nodes, while metastatic workup was negative for malignancy. As the biopsy showed a positive margin, the patient underwent secondary resection. Moreover, he received adjuvant chemotherapy and radiotherapy. There was no evidence of the disease, and no metastasis was detected in a follow-up of three years. We also performed a literature review of pediatric tongue RMSs to assess the clinical presentation, histopathology, diagnosis, and management. Rhabdomyosarcomas (RMSs) are malignant tumors that originate from primitive mesenchymal tissue. They are the most common soft tissue tumor in children . Soft tiThe etiology and specific risk factors of RMSs are unknown; however, higher incidence was observed in children from families with low socioeconomic status, children exposed to radiation in utero, and with maternal use of recreational drugs -6. RMSs The differential diagnosis that should be considered includes other soft tissue tumors such as hemangioma, fibroma, rhabdomyoma, and lymphangioma . To evalFor staging, chest CT scan, bone marrow aspiration and bone scan can also be performed. However, the metastatic workup should be tailored to the clinical and histologic features. Clinical group and the tumor, node, metastasis (TNM) system are the most widely used classifications for newly diagnosed RMSs. The clinical group (CG) takes into account the tumor size, nodal involvement, and patient age. It has four categories, which take into account the extent of the disease .The treatment of oral RMSs consists of surgery followed by chemotherapy with or without radiotherapy. Chemoradiation following complete surgical resection is considered ideal, however, it is difficult to achieve complete excision. Oral RMSs are associated with a higher recurrence rate and metastasis compared to other types. With the multimodal treatment approach, the five-year survival rate reaches to 85% .We report a case of an eight-year-old male who presented with a small mass at the tip of the tongue that was excised with positive margins. He underwent a second resection of the mass with neck dissection and received adjuvant chemotherapy and radiation therapy. We also reviewed reported cases of tongue RMSs in the literature on the clinical presentation, treatment, and outcomes.An eight-year-old child presented to the referring hospital with a small swelling at the tip of his tongue noticed by his parents. According to medical records, there were no associated symptoms and signs such as pain, difficulty feeding, fever, or lymph node enlargement at the time of presentation. The patient had a family history of cancer in parents, aunts, and grandparents .Head magnetic resonance imaging (MRI) was performed before the patient was referred to our hospital. The medical records reported a 1.3 x 1.4 x 1.7 cm peripherally enhancing mass with central cystic/necrotic component Figures . The patAs a result, the patient was referred to our hospital for further management. Oral cavity examination revealed no swelling of the tongue, and only a healed operational scar was noted. All head and neck lymph nodes were non-palpable on clinical exam. Other organ systems examination was unremarkable. Head and neck computed tomography (CT) scan demonstrated an oral cavity filled with heterogeneous tongue without bone destruction at the level of the neck. Also, bilateral enlarged cervical lymph nodes at the left level I were noted and the largest measured 0.8 x 1.1 cm in the left cervical chain. Oropharynx, hypopharynx, and major cervical vessels appeared unremarkable. The larynx and trachea were patent. CT scan , bone marrow aspiration, and bone scan were all negative for metastasis. As the patient had a strong family history of cancer, whole exome sequencing (WES) was performed, and it was negative. A tumor board meeting consisting of an otolaryngology and head and neck surgeon, pediatric surgeon, radiotherapist, pediatric otolaryngology surgeon, histopathologist, radiation oncologist and pediatric medical oncologist was conducted. The members of the tumor board agreed on doing secondary resection. If the margins were negative, the patient will be treated as intermediate risk.The patient underwent wedge resection of the tongue with left neck dissection from level I to level III lymph nodes. Wider resection of the old lesion with resection of the new lesion was performed with a 0.5 cm margin from the mass. Negative margins were reported by a frozen section procedure intraoperatively. Primary closure of the defect was then done. Histopathology report showed epithelioid RMS, Grade 2, with a tumor size of 0.8 x 0.5 x 0.5 cm that extended deeply in the tongue tissue. The tumor pathological stage (pTNM) was pT1b, pN1. Histopathological analysis of the hematoxylin and eosin-stained material demonstrated diffuse growth of epithelioid neoplastic cells with hyperchromatic nuclei and high nucleus-cytoplasm ratio Figures , 4. ResePost-operative MRI revealed a residual area of abnormal signal with enhancement at the region of the surgical resection of the tip of the tongue on the left side, which was highly suggestive of post-surgical changes. Residual small lymph nodes at level II of the cervical area and left prevertebral region were noted as well.Postoperatively, the patient received the Children’s Oncology Group (COG) ARST 0531 chemotherapy protocol which consists of vincristine, actinomycin d and cyclophosphamide (VAC) alternating with vincristine and irinotecan (VI) for 40 weeks. He also completed radiotherapy to oral anterior tongue, bilateral cervical and retropharyngeal lymph nodes with a dose of 60 Gy in 40 fractions. During his follow-up for the last three years in our hospital, no evidence of the disease was noted in the clinical and radiological assessment done by serial MRIs.We reviewed all the cases reported in the English language on tongue RMSs in pediatrics using the PubMed engine. Twenty cases were reported, including our case, summarized in Table RMSs of the tongue are rare. Bras et al. reported 16 cases of oral RMSs between 1944 to 1984, only one case has tongue RMSs . The mosTreatment of tongue RMSs is multimodal including surgery plus chemotherapy, chemotherapy plus radiotherapy, and triple therapy . Chemotherapy has been used in all the cases as RMSs are chemosensitive, while surgery is considered mainly in patients with respectable tumors. Surgical resection includes excisional biopsy, partial glossectomy, and near total glossectomy depending on tumor size. Radiotherapy is reserved for the control of the locally advanced disease. In the case reviewed by Gurung et al., the patient received radiotherapy along with surgery and chemotherapy as histopathology showed invasion to the tongue muscle . SimilarRMSs of the tongue are a rare condition with very few cases reported in the literature. The most common presentation is a lingual mass, and due to its location, it is identified and diagnosed early in the course of the disease. The most common RMS is embryonal and to the best of our knowledge, to date there are no cases reported on epitheliod RMS. Treatment depends on the stage and involves surgical resection, chemotherapy, and radiation.


Mitral regurgitation is a common valvular disorder found in the general population with varying degrees of severity. The symptoms of this disorder correspond to the severity of regurgitation as well as its associated complications such as arrhythmias. Suspicion of mitral regurgitation is based on physical exam findings with diagnosis generally requiring confirmatory findings on transthoracic echocardiogram. However, asymptomatic patients with mitral regurgitation and limited sensitivity of cardiac auscultation to detect a murmur confound the diagnosis. In this case, a patient presented with nonspecific symptoms of shortness of breath and abdominal pain in which a bedside point-of-care ultrasound (POCUS) in initial examination demonstrated severe mitral regurgitation and pulmonary edema. These findings expedited an intervention on the regurgitation, which highlights the importance of incorporation and early use of POCUS during physical examination. Mitral regurgitation is prevalent in the general population and is characterized by the spectrum of pathophysiology and underlying severity. A trivial amount of regurgitation can be found in up to 66% of otherwise healthy individuals in what is known as physiologic mitral regurgitation . Mild reA systolic murmur, more specifically an apical holosystolic murmur or late systolic murmur, should prompt suspicion of mitral regurgitation. The diagnosis is subsequently confirmed with a transthoracic echocardiogram. Other modalities such as a transesophageal echocardiogram or cardiovascular magnetic resonance imaging can be used if transthoracic echocardiogram imaging is suboptimal. The limiting factor in making the diagnosis lies in the physical exam as cardiac auscultation provides limited sensitivity for the detection of valvular disease -8. Some Point-of-care ultrasound (POCUS) during the physical examination can be used as a diagnostic modality to increase the diagnostic yield in mitral regurgitation. It has been demonstrated previously that handheld ultrasound is superior to standard physical examination in detecting substantial valvular disease . FurtherA 70-year-old female with a history of hypertension presented to the emergency room with complaints of abdominal pain, shortness of breath, and dizziness. Her symptoms had been present for four weeks prior to the presentation, which had prompted an abdominal MRI as an outpatient, which revealed a pancreatic cyst and a papillary mucinous neoplasm. On presentation, she was found to be hypoxic, requiring two liters of oxygen supplementation, and tachycardic to 108 beats per minute, with a blood pressure of 156/83 mmHg. On physical exam, she was found to have clear breath sounds, a nontender abdomen, and a systolic murmur on cardiac auscultation. Chest X-ray showed interstitial opacities, which were more pronounced in the lower lobe fields. Initial lab work was unremarkable.POCUS performed during the initial physical exam revealed diffuse B-lines consistent with pulmonary edema Video  and bilaMitral regurgitation is a common valvular disease that carries a range of symptoms that typically correlate with regurgitant severity -3. CommoIn this case, a patient presented with shortness of breath, which is a complaint that carries a broad differential, and was diagnosed with severe mitral regurgitation through POCUS on the initial physical exam in the emergency room. Though this patient may have ultimately been evaluated by a formal echocardiogram based on her symptoms and hypoxia, POCUS revealed urgent findings during the physical examination, which prompted an early intervention that may have been delayed when relying on cardiac auscultation alone.The key to this case is recognizing that POCUS, when integrated into standard physical examination, can improve the detection rate of significant valvular abnormalities in the absence of an abnormal physical exam ,10. WhenDiagnosis of mitral regurgitation relies on a high degree of suspicion based on a systolic murmur on physical exam with subsequent echocardiogram for confirmation. Cardiac auscultation has a low sensitivity in detecting significant valvular abnormalities and allows the risk of delaying a diagnosis until a patient becomes significantly symptomatic. When incorporated with a physical exam, POCUS increases the rate of detection of significant mitral regurgitation independent of symptoms or abnormal physical exam.


Hepatic angiosarcoma is a very rare disease, but it has a poor prognosis. Here, we report the case of a 77-year-old man who was referred to our hospital for suspicion of hepatocellular carcinoma and cancerous peritonitis. Based on the imaging findings, a diagnosis of spontaneously ruptured hepatic hemangioma was made. Six days later, the patient was transported to the hospital in a state of shock and an emergency transarterial embolization was performed. He underwent lateral hepatic segmentectomy 7 days later. Histopathologically, he was diagnosed with hepatic angiosarcoma. Fever was observed 21 days after surgery, and computed tomography was performed. Multiple liver masses, which ware increasing rapidly, were found, and hepatic angiosarcoma recurrence was confirmed. He requested home medical care and died at home 36 days after surgery. When a tumor diagnosed as a hepatic hemangioma by imaging has ruptured, the possibility of hepatic angiosarcoma should be considered. Hepatic angiosarcoma is a very rare disease with a frequency of 0.04–2% among primary malignant tumors of the liver; however, it is considered to have a poor prognosis . It is oHere, we report a case of spontaneously ruptured hepatic angiosarcoma resected after transcatheter arterial embolization, which was challenging to diagnose preoperatively.A 77-year-old man was referred to the Department of Gastroenterology at our hospital for suspicion of hepatocellular carcinoma and cancerous peritonitis. He was scheduled to be hospitalized for a thorough examination at a later date. However, 3 days after his first visit, he returned to the Emergency Department with a complaint of abdominal distension. He was hospitalized on the same day with a diagnosis of intra-abdominal bleeding due to a ruptured tumor in the lateral section of the liver. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a tumor with a maximum diameter of 8 cm in the lateral hepatic region and ascites around the liver and in the pelvic floor. Dynamic CT showed blood vessel-like early staining inside the tumor, and the contrast effect gradually enhanced . During The risk of rapid progression and metastasis is high among patients with hepatic angiosarcoma, and the mean survival time is reported to be 5.5–6.0 months . Our patHepatectomy is the treatment of choice for resectable hepatic angiosarcoma, but there are many cases with distant metastasis at the time of diagnosis, and radical resection is possible in few cases . AlthougHepatic angiosarcoma is sometimes difficult to distinguish from hepatic hemangioma on imaging , 12. TheIn conclusion, although spontaneous rupture of a tumor diagnosed as hepatic hemangioma by imaging is very rare, the possibility of hepatic angiosarcoma should be considered.


Among all GI tract cancers, they account for less than 1% of cases. Most patients become symptomatic in the later stages of the tumor’s course, often presenting with insidious anemia due to gastrointestinal bleeding and metastasis. The recommended management of solitary GIST is surgery, while larger or metastatic tumors that express c-KIT are managed with imatinib as either neoadjuvant or adjuvant therapy. Due to the progression of these tumors, they are at times associated with systemic anaerobic infection, which is an indication of malignancy workup. In this case report, we discuss a 35-year-old woman who was discovered to have GIST with possible hepatic metastasis complicated by pyogenic liver disease due to S A gastrointestinal stromal tumor (GIST) is one of the most common pleomorphic non-epithelial KIT- or platelet-derived growth factor receptor alpha (PDGFRα)-mediated mesenchymal tumors of the gastrointestinal (GI) system, and they account for less than 1% of all GI tumors . There iA 35-year-old woman with a history of genital herpes on valacyclovir presented to the emergency department (ED) due to sharp 10/10 left upper quadrant (LUQ) pain, fever, and loss of appetite. On presentation, she was febrile with a temperature of 101 °F and tachycardic with a heart rate of 108. The physical exam was notable for an ill-appearing woman with severe discomfort due to abdominal pain and LUQ tenderness on palpation. Her initial laboratory results showed severe anemia with hemoglobin 5.6 g/dL requiring a transfusion of 2 units of packed red blood cells, a white blood cell count (WBC) of 11.2 × 103/µL, sodium 130 mmol/L, and elevated alanine transaminase (ALT) of 101 IU/L and aspartate transaminase (AST) of 115 U/L.Following the initial stabilization of her hemoglobin, the patient spent the majority of her early hospital course reporting persistent LUQ pain unchanged in intensity. She underwent CT abdomen and pelvis (A/P) without contrast, which revealed a left upper quadrant nonspecific 5.5 cm × 4.2 cm mass containing a small amount of fluid and air, multiple unquantified hepatic lesions, the largest measuring 3.3 cm × 4.8 cm, which was suspicious for metastatic disease, splenomegaly with splenic infarcts, and possible masses of the pancreas or porta hepatis. Cancer antigen 125 was mildly elevated at 37 U/mL (reference range <35 U/mL), cancer antigen 19-9 was 6 U/mL (reference range <34 U/mL), and carcinoembryonic antigen was 1.7 ng/mL (reference range 0-2.5 ng/mL). The patient subsequently underwent a CT-guided liver biopsy for presumed metastatic disease by interventional radiology, with 5 mL of purulent fluid extracted and sent for culture. Preliminary results demonstrated rare Gram-positive cocci which speciated to Streptococcus intermedius. Her anaerobic blood cultures also contained Streptococcus intermedius. The echocardiogram was negative for valvular vegetation.The patient was initially started on cefepime, then switched to piperacillin-tazobactam, and later to ceftriaxone due to an unimproved clinical course and pending culture data. Following the speciation of her anaerobic culture, a peripherally inserted central catheter (PICC) line was placed, and the patient was switched to ertapenem, which led to a significant improvement in her leukocytosis. However, a repeat CT A/P performed seven days later, showed no marked improvement in the sizes of her lesions despite antibiotics, with the largest lesion approximating 5.8 cm × 4.6 cm. She then underwent esophagoduodenoscopy (EGD) and colonoscopy. While the latter was unremarkable, the former revealed a nodular mass in the third portion of the duodenum, from which a biopsy was obtained Figure . An MRI The patient’s genetic mutational analysis of her biopsied mass revealed very low levels of the c-KIT mutation on exon 9 [c.1504_1509dup (p.A502_Y503dup)] and negative mutations of D816V on exon 17 and PDGFRα. Immunohistochemical staining was notable for the presence of CD117, patchy staining for CD34, and negative staining for actin and desmin, which was consistent with GIST , tyrosine kinase receptor CD117, CD34, and sometimes actin ,7. ApproGIST is considered potentially malignant and is stratified from very low to high based on its clinical risk of malignancy and recurrence . FletcheGIST has been hypothesized to closely simulate the interstitial cells of Cajal (the pacemakers of the gut) or their precursors, but they are being increasingly recognized as arising from mesenchymal cells with multipotent properties . The mosBased on the pathobiology and molecular cascades involved in the development of GIST, an accurate histologic diagnosis and prognostication are essential before initiating treatment . GIST saImatinib is used to control advanced GIST via neoadjuvant uses for reducing tumor size and facilitating resection, and as adjuvant therapy to reduce recurrence in patients who are at high risk . ImatiniA review of the literature has demonstrated that primary liver abscesses when associated with primary GIST are often caused by commensal, obligate, or facultative anaerobic organisms . Alpha hAll in all, this case was demonstrably a diagnostic challenge, as we did not know whether the patient truly had metastases, although she was recommended to initiate treatment with imatinib. She was unfortunately lost to follow up with our institution as she transitioned her care to a facility closer to home. Nevertheless, despite her degree of diagnostic uncertainty, we recommend that pharmacotherapy be appropriately guided to manage both infection and malignancy, although there is no literature suggesting appropriate dosing or an antibiotic course in cases such as this.GIST is a mesenchymal tumor that can be found incidentally at advanced stages, often presenting with chronic, insidious anemia. The disease can also be associated with anaerobic infections involving alpha-hemolytic streptococci, which, if encountered first, should warrant workup for gastrointestinal malignancy. The treatment of GIST is often surgical for tumors that are deemed resectable; however, in non-resectable or advanced tumors, imatinib is the first-line treatment in patients with c-KIT mutations. In cases where patients present similarly to those described in this case report, we recommend that treatment be focused on managing both presenting problems.


Lymphomas are a heterogenous group of cancers of the lymphatic system in which disease primarily arises in lymph nodes. Extranodal disease is common; however, musculoskeletal involvement is rare. Imaging plays an important role in the diagnosis and staging of all lymphomas. In this case series, we present examples of musculoskeletal involvement of lymphoma encountered at our institution. We outline the clinical presentation and imaging findings of each and use these cases to review the features that can help to differentiate lymphoma from other musculoskeletal lesions. Disease primarily arises in lymph nodes but can occur in nearly any tissue, with extranodal disease present in approximately 25–40% of cases.3,4 Musculoskeletal involvement is rare and is usually the result of secondary spread but may also represent primary extranodal disease.4–7Lymphomas are cancers of the lymphatic system, accounting for approximately 2% of all cancer diagnoses each year and are ranked the sixth most common cancer type in Ireland.In this case series, we present seven cases of biopsy-proven lymphoma, each of whom had musculoskeletal lesions on initial imaging. We discuss in turn the imaging findings of each including radiographic, CT, MRI and positron emission tomography (PET). Finally, we review the typical imaging features of lymphoma in muscle and bone, and outline the characteristics that differentiate these from other malignant lesions within the musculoskeletal system.T2 weighted imaging and hypointense on T1 weighted imaging relative to adjacent skeletal muscle , multifocal PBL, or disseminated lymphoma with secondary osseous involvement that is either (a) within or (b) greater than 6 months from initial diagnosis. Diffuse large B-cell lymphoma accounts for the majority of osseous lymphomas.9–11 Clinical presentation includes bone pain (61%), soft tissue swelling (31%), pathological fracture (22%) and typical B-symptoms (13%), although these are rare in PBL.12 PBL and multifocal PBL typically affect the appendicular skeleton, with multifocal PBL occasionally involving the axial skeleton.10 Secondary osseous lymphoma preferentially affects the axial skeleton.13Lymphoma of bone, sometimes termed osseous lymphoma, accounts for 5% of extranodal lymphomas and represents 7% of all bone malignancies.5 A permeative or “moth-eaten” appearance is the most common.9 This appearance can also be seen with infection, Ewing sarcoma, osteosarcoma, multiple myeloma and metastases from other malignancies. Features that can indicate advanced local disease include cortical destruction, periosteal reaction and extraosseous soft tissue masses.9 Periosteal reaction patterns can be linear, lamellated or disrupted. Sclerotic lesions can occur, most frequently with Hodgkins lymphoma, however, lytic lesions remain the most frequently encountered in all subtypes.13,14 The ivory vertebra sign representing diffuse sclerosis of a vertebral body is a radiological sign associated with osseous lymphoma but can also be seen in a number of conditions including osteoblastic metastases from prostate and breast cancer as well as Paget disease and osteosarcoma.15Radiographic features of osseous lymphoma are variable and non-specific. Patterns can be normal, lytic, sclerotic or mixed lytic/sclerotic.10,15 CT provides a more detailed assessment of osseous lymphoma, with better visualisation of cortical and trabecular destruction, periosteal reaction and extraosseous extension. One particular feature on CT that is suggestive of lymphoma is the relative absence of cortical destruction which is more commonly seen with osteosarcoma.16Bone scintigraphy is useful for the detection of multifocal osseous involvement however findings are non-specific.17 Abnormal marrow generally exhibits low intensity on T1 weighted images and high intensity on T2 weighted images. This, however, is not specific for lymphoma and low signal intensity on T2 weighted images can be seen.17,18 MR imaging is also useful in evaluating extraosseous soft tissue masses which again demonstrate low signal intensity on T1 weighted images and high signal intensity on T2 weighted images.9MR imaging in osseous lymphoma is particularly useful for assessing marrow involvement.19,20 As with osseous lymphoma, patients may present with localised soft tissue swelling, pain and/or B-symptoms.21 The thigh and upper arm are the most common sites of disease which may be a focal mass or diffuse infiltration of the muscle.21–23 Ultrasonography, while non-specific and of limited use for staging, does play an important role in evaluating a solid mass/enlarged muscle, particularly for guiding biopsies. There are no specific sonographic features of muscular lymphoma, however, soft tissue lesions are usually solid, hypoechoic and can have either well-defined or poorly defined borders.21 CT findings for muscle involvement are non-specific. Muscle enlargement can be seen, with the abnormal tissue often isodense to normal muscle and enhancement post-contrast is variable.24,25 CT is, however, useful for identifying extent of disease and bony involvement/destruction. MR imaging is the most useful imaging modality for the assessment of muscular lymphoma. Either a discrete mass, or diffuse muscle infiltration may be seen, with the abnormal tissue isointense on T1 weighted images or hyperintense on 2T weighted images to surrounding muscle tissue.6 Contrast enhancement can be diffuse homogenous or heterogenous exhibiting peripheral thick band-like enhancement or marginal septal enhancement. Features that are suggestive of skeletal muscle lymphoma include muscle enlargement with long segmental or multicompartmental involvement, deep fascial involvement, skin thickening, subcutaneous stranding and traversing vessels.26Muscular lymphoma accounts for 1.4% of all lymphomas and similarly, this can be due to disseminated lymphoma, local extension or primary muscular lymphoma.Lymphoma represents a significant proportion of malignancies each year and timely identification is crucial given the often favourable outcomes if diagnosed early. Extranodal disease is common and can occur in almost any tissue. Bone and muscle involvement is rare, yet remains an important differential for musculoskeletal lesions as these can be the only presenting symptom. Imaging findings are often non-specific; however, certain imaging features, outlined in this case review, can help to characterise such lesions.Lymphoma is an important differential to consider for new bony/muscular lesions, either as primary musculoskeletal lymphoma or secondary to disseminated disease.Radiological diagnosis is challenging due to the heterogeneity of the disease and multiple modalities should be employed.For osseous involvement, radiography and CT are useful for determining the pattern of destruction and extent of extraosseous spread, while MR imaging can demonstrate marrow involvement.In muscular lymphoma, MR imaging is the most useful modality. Muscle enlargement with multicompartmental involvement and traversing vessels are features suggestive of lymphoma.


DICER1 gene, which shows variable expressivity. To our knowledge, spinal hamartoma has never been described in individuals with DICER1 syndrome. Here, we describe a rare association of congenital spinal hamartoma and DICER1 syndrome in a 5-week-old infant, with molecular findings suggestive of the implication of DICER1 in the pathogenesis of this tumor.Congenital spinal hamartomas are rare benign tumors. They are mostly seen in infants and are typically asymptomatic at presentation. Spinal hamartomas have not been associated with any known cancer predisposition syndrome. DICER1 syndrome is a well-characterized cancer predisposition syndrome caused by a germline mutation in the  Congenital midline spinal hamartomas are a rare but increasingly well-described clinical entity. They are benign tumors, characterized by a well-differentiated and mature overgrowth of local ectodermal and mesodermal elements, present in a disorganized manner . Spinal DICER1 (DICER1 syndrome OMIM #601200) is a rare tumor predisposition syndrome, with tendency to develop a wide range of benign and malignant tumors beginning in childhood and continuing into adulthood. DICER1 syndrome is caused by a germline pathogenic variant in the tumor suppressor gene, DICER1 . TypicalDICER1 . Central01200 is DICER1 variant and a second somatic hit in the spinal hamartoma.Herein, we present the case of a 5-week-old infant with an extremely atypical presentation of congenital midline spinal hamartoma of the cervical spine, who was found to harbor a pathogenic germline A 5-week-old full-term female infant presented with progressive bilateral upper limb weakness and diminished spontaneous movements beginning at the age of 1 week. Her perinatal history was unremarkable, with no history of traumatic delivery and a normal physical exam at birth. At presentation, there was bilateral upper limb flaccid hypotonia on the right more than the left, absent grasp reflex in both hands, absent bilateral upper limb deep tendon reflexes and loss of upper limb sensation bilaterally. Magnetic resonance imaging (MRI) of the brain and spine revealed a cervical spine extramedullary lesion extending from C2-C6 with extension through several neuronal foramina on the right Figure 1Based on her age and radiographic features, neuroblastoma with cord compression was suspected. Staging Computed tomography (CT) of the neck, chest abdomen and pelvis were unremarkable. Her urine catecholamines (HVA and VMA) were both within normal limits on two occasions. She received a single cycle of carboplatin and etoposide as well as dexamethasone on an emergent basis. The patient continued to demonstrate symptomatic cord compression, so a decision was made to undergo surgical resection of the lesion to protect her cord from ongoing damage and obtain a histologic diagnosis.Intra-operatively, a well circumscribed cartilaginous tumor was identified on the right side of the dura. A gross total resection of the extradural component was achieved and a small intradural residual tumor was left as it was adherent to the nerve root. Pathologic examination revealed a benign lesion with similar features in the intraspinal, extradural and intradural components. The lesion showed anastomosing islands of hyaline cartilage with loose fibrous stroma (including nerve-like tissue) in between . Results revealed a heterozygous pathogenic variant in the DICER1 gene designated c.3118_3119insCA (p.IIe1040Thrfs*27), which is predicted to result in a frameshift and premature translational stop site, expected to result in an absent or disrupted protein product. This variant was reported as pathogenic given its predicted functional consequence and has not been previously reported in the literature nor in population controls (gnomAD). There was no tumor specimen available from the mother for sequencing.The evolution of this family’s cancer history prompted further evaluation. A salivary sample was obtained from the mother for germline DICER1 finding, the patient was enrolled in our institutional precision oncology program KiCS (SickKids Cancer Sequencing) Program, through which germline and tumor samples were sequenced using a clinically validated DNA cancer sequencing panel . The same heterozygous germline frameshift variant in the DICER1 gene was detected in the patient who at that point was 3 years of age.Following the mother’s confirmed germline DICER1 gene, namely, c.5113G>A (p.Glu1705Lys). This is a somatic hot spot variant and is frequently identified somatically in DICER1 associated tumors such as Sertoli–Leydig cell tumor and pleuropulmonary blastoma  were noted to develop other neoplasms . DICER1 quencing . Since to expand . NCMH wasyndrome . In 2014mutation . Furthered tumor , 13, 14.henotype . This vaOur patient developed a congenital spinal hamartoma, which is an extremely rare benign, but locally aggressive, tumor of infancy and childhood. Intracranial hamartomas are commonly associated with neurofibromatosis type 1, consisting of neural-crest derived neurons, glial cells and Schwann cells . CongeniDICER1 is a tumor suppressor gene which encodes an RNase III-family endonuclease responsible for cleavage of precursor microRNA into active miRNA (DICER1 RNase IIIb domain missense variants have shown that they lead to defective cleavage of 5p-derived miRNAs from the pre-miRNA loop sequence resulting in the retention of pre-miRNA loop sequence in DICER1 mutant cancers (DICER1 germline pathogenic variant was a c.3118_3119insCA frameshift. The second hit identified in our patient’s tumor was a missense mutation in the DICER1 gene (c.5113G>A (p.Glu1705Lys)). This variant is one of the hot spot mutations in DICER1 and is frequently identified somatically in Sertoli–Leydig cell tumors, pleuropulmonary blastomas and other cancer types. It is often found with a loss of function germline DICER1 variant in patients with DICER1 tumor predisposition syndrome (ve miRNA . Tumorigve miRNA . Functio cancers . This is cancers . Our pat>A p.Glu105Lys). TDICER1 drivers, differentiating its natural history from sporadic forms of spinal hamartoma.It is interesting to note that our patient’s spinal hamartoma is quite atypical in many ways. First, the hamartoma developed in the cervical spine, contrary to the thoracic and lumbosacral predominance that is generally described in the literature . AdditioDICER1-associated hamartoma has been described in the nasal cavity, liver and now spine. As described in this report, congenital spinal hamartoma is a newly associated, albeit rare manifestation of the DICER1 syndrome phenotype.In summary, The original contributions presented in the study are included in the article/Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. Written informed consent to participate in this study was provided by the participants’ legal guardian/next of kin. Written informed consent was obtained from the individual(s), and minor(s)’ legal guardian/next of kin, for the publication of any potentially identifiable images or data included in this article.RH, data acquisition and original draft preparation. WL, carried out molecular genetic studies and annotation. HC, pathologist provided histology images. MS, radiologist provided MRI images. DM, AV, and AD, conceptualization, writing- review and editing. 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.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Management of small bowel obstruction varies depending on the cause and clinical status of patients. While most cases can be managed conservatively, a not-insignificant proportion of patients undergo surgical intervention. Laparotomy has long been the default approach for entering the abdomen in cases requiring surgical intervention, with laparoscopy largely being avoided due to abdominal distension and the risk of perforating bowels on entry.We present here the case of a 54-year-old woman who presented with signs and symptoms as well as radiological evidence of a closed-loop small bowel obstruction in her right lower quadrant. Following a brief period of nasogastric decompression, her abdominal distension improved, allowing for a laparoscopic entry where a pericaecal hernia was noted to be the cause of her obstruction. Extensive adhesiolysis without the use of an energy device successfully allowed for the reduction of the bowel contained within. No bowel resection was performed and the patient was discharged home on day 3 following her procedure.This case report successfully demonstrates the utility of using laparoscopy as an alternative to laparotomy in patients with a small bowel obstruction secondary to an internal hernia. Internal hernia is an important consideration as a differential diagnosis in patients with intestinal obstruction. Defined as a protrusion of abdominal contents through a peritoneal or mesenteric orifice, internal hernias most commonly manifest as small bowel obstruction in adults . Of the A 54-year-old woman presented to the emergency department with a one-day history of colicky right iliac fossa pain with migration to the epigastrium. The pain was associated with vomiting and exacerbated by oral intake. The patient had previously undergone multiple laparoscopic procedures, including appendicectomy, cholecystectomy, gastric band insertion, band removal, and sleeve gastrectomy. On examination in the emergency department, the patient’s observations were within normal limits. A soft, distended abdomen was noted with tenderness in the right iliac fossa. A computed tomography (CT) scan was performed in response to a lack of relief from analgesia and demonstrated a closed-loop small-bowel obstruction with multiple transition points around a single loop in the right lower quadrant, lateral and posterior to the caecum, likely secondary to adhesions from previous appendicectomy Figure . A nasogLaparoscopic Hasson entry was achieved through the umbilicus, where CT imaging showed omental space with no bowel in the vicinity. A right colon adhesion plate to the right lateral abdominal wall with a small, tight opening around the caecum was found to have created a cocoon through which a 20-cm length of mid-small bowel herniated Figure . An a-trThis patient’s history and examination were suggestive of a small bowel obstruction. While her CT scan was useful in providing a diagnosis of a closed-loop bowel obstruction as well as an appropriate laparoscopic entry site, it did not detect the specific presence of a pericaecal internal hernia. Internal hernias are defined as a protrusion of a viscus, usually small bowel, through a peritoneal or mesenteric aperture within the abdominal cavity. Internal hernias are uncommon and typically manifest with a small bowel obstruction. Approximately 0.6-5.8% of small bowel obstructions in adults can be attributed to internal hernias, and pericaecal hernias are rarer still, constituting up to only 6-13% of all internal hernias .Pericaecal hernias emerge through an aperture that develops within the peritoneal recess formed by local folds of the peritoneum . Four suLaparotomy has long been the standard operative approach to managing acute small bowel obstructions, particularly those caused by internal hernias . LaparosTo the best of our knowledge, this is one of the few reported cases of a pericaecal internal hernia with small bowel obstruction being managed successfully through laparoscopy. Fewer than 40 cases of pericaecal hernias have been reported in the literature, with the vast majority being managed through laparotomy. Only eight cases have reportedly been managed laparoscopically ,9,12.Even though it is rare, the consideration of a pericaecal hernia causing small bowel obstruction as a diagnosis is important in the patient presenting with right lower quadrant pain, particularly when signs and symptoms of obstruction are present. We believe this case successfully demonstrates the utility of using a CT scan in diagnosing small bowel obstructions and surgical planning in patients with undifferentiated abdominal pain. It also validates the practicality of laparoscopic surgery as an alternative to laparotomy in managing pericaecal hernias causing small bowel obstruction in patients where nasogastric decompression is possible.


Appendiceal mucinous tumors are rare, with variable malignant potential, and they are usually found incidentally. Clinical symptoms are nonspecific. Rarely, appendiceal mucinous neoplasm causes bowel obstruction and makes diagnosis more difficult. We present a case of an 84-year-old female who came to our emergency department having had abdominal fullness and constipation for 5 days. Ileus, due to an affected adhesion band, was diagnosed initially, and symptoms improved gradually under conservative treatment. However, 3 months later she presented to the emergency department again with abdominal pain and distension; small bowel obstruction due to adhesion was again diagnosed. Recurrent bowel obstruction prompted emergent surgery. Operative findings showed a whitish appendiceal tumor adhering to and directly invading the adjacent ileum, with a segment of herniated small bowel wedged in between, causing the obstruction. Upon reviewing the initial computed tomography scan, the dilated tubular structure of appendiceal tumor was misrecognized as small bowel loop; there was no surrounding inflammatory sign, leading to diagnosis difficulty. Instead of a common cause of bowel obstruction, such as adhesion band, this case revealed bowel obstruction can be caused by the direct invasion of an appendiceal tumor. Awareness of this condition with careful image evaluation of small bowel obstruction is essential for diagnosis.


Primary osseous Rosai-Dorfman disease (RDD) is a rare and benign disease that can pose diagnosis challenges.We report the case of a 29-year-old woman who presented with pain in her left patellar region for the past 6 months with no other clinical sign especially no lymphadenopathy associated. A surgical excision was done. Histopathology confirmed the diagnosis of a primary RDD patellar disease. The patient was followed up for 2 months without any recurrence. The aim of this study was to present a rare case of RDD in a patellar location and to review clinicopathological features, therapeutic modalities, evolutionary aspects and prognosis of a primary patellar RDD.The diagnosis of a primary osseous RDD without associated lymphadenopathy should be kept in mind when a sclero-lytic lesion is found. Excision of the lesion is the gold standard of the treatment. •Isolated patellar RDD is rare.•The diagnosis is made on histopathological examination of resected tissue•Prognosis is good and disease course is not aggressive Cell blood count (CBC), ultrasound and conventional radiograph of her knee were normal . Right kThe work has been reported in line with the SCARE 2020 criteria 3This case describes the clinical, radiological and pathological course of an isolated patellar RDD.Primary osseous involvement of RDD is rare, representing in roughly 10 % of cases and occurs in adulthood The imaging manifestations of RDD in bone are not specific. Bone lesions in RDD are typically osteolytic with margins varying from sclerotic to permeative on conventional radiographs Numerous neoplastic and inflammatory benign diseases can overlap with RDD's imaging symptoms. The most prevalent diagnosed benign patellar tumor is the giant cell tumor (GCT). Imaging features of GCT are typical on radiographs including a pathologic fracture, a soap bubble appearance, a sclerotic and radiolucent lesion, a fracture of the femur and the tibia, and an osteolytic lesion of the patella with bone degradation. MRI shows an abnormal extension and lesion of the patella, and there may be some evidence of adjacent tissues and sclerotin Histopathological examination with immunohistochemistry must be performed to confirm the final diagnosis of bone RDD. In our case, the patient underwent a biopsy and surgical resection. Microscopically, RDD is characterized by a proliferation of histiocytes that express S-100 protein and CD68 and are typically negative for CD1a Similar histiocytic diseases such as Langerhans cell histiocytosis and Erdheim-Chester disease can mimic RDD. Microscopic examination and immunohistochemistry help make the right diagnosis. Langherans cell histiocytosis is characterized by slightly foamy histiocytes with groovy nuclei mixed with inflammatory infiltrate containing eosinophils. Langherans cells express typically CD1a Treatment for primary RDD of bone is focused on relieving painful disease sites or avoiding consequences such pathologic fracture. Primary RDD of bone is not known to have a mortality risk. The most frequently mentioned therapies in these cases are surgical excision or curettage and bone grafting 4This case presentation highlights the isolated patellar location of RDD. Clinical manifestations of patellar RDD are not specific. MRI imaging are adjunct techniques that could be helpful to guide the diagnosis but remain insufficient. Definitive diagnosis relies on the typical histopathological findings by the identification of the characteristic histiocytes showing emperipolesis expressing S-100 protein.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.No.Not commissioned, externally peer-reviewed.All the authors read and approved the final version of the manuscript.Farah Sassi (MD): conception, acquisition of data, literature research and preparing the manuscriptHaythem M'rad (MD): acquisition of clinical data, preparing and revising the manuscriptLinda Belhaj Kacem (MD): SupervisionBoubaker Sassi (MD): revising the manuscript criticallySamia Hannachi (MD): acquisition of data, manuscript editingSoumaya Rammeh (MD): final approval of the version to be publishedSassi FarahThe authors report no declarations of interest.