Postoperative immunohistochemistry revealed ACTH and T-box Protein in T-cell positivity. With literature review, we found 5 reported cases of clival ectopic pituitary adenoma with Cushing syndrome. Clinical characteristics, imaging features, histology, and management of such masses have been discussed. In conclusion, ACTH-producing clival ectopic pituitary adenomas are rare, require differentiation from neuroendocrine tumors, and remit by multimodal therapy.The development of adenohypophysis by differentiating and detaching from the pharyngeal roof results in formation of a functional ectopic pituitary tissue around the Sella turcica. Of the ectopic sites in which pituitary adenoma occurs, clival adenomas are rare and the majority secrete prolactin. We report a case of ACTH-dependent Cushing syndrome. Magnetic resonance imaging revealed a clival mass with sphenoid sinus infiltration and  Cushing syndrome has remained 1 of the most difficult endocrine disorders to manage to date because localizing the source can be a challenge. Even in those patients with central localization, as confirmed by inferior petrosal sinus sampling (IPSS), ectopic location of a tumor around the sella turcica remains 1 of the reasons for disease persistence after surgery. In a review of 21 cases of ectopic ACTH-secreting pituitary adenoma in sphenoid sinus, preoperative diagnosis of ectopic pituitary adenomas (EPA) was not made in 9 (46%), and in 2 (10%) imaging did not show the ectopic location of the adenoma ). However, her insulin requirement, hypertensive medications, and potassium replacement reduced in postoperative period. She was started on IV liposomal amphotericin B in view of mucormycosis invading the nasal septum. Repeat nasal endoscopy after 1 week showed complete resolution of the lesion; hence, amphotericin was stopped after 10 days. After vitamin D supplementation, IV zolendronic acid was administered for osteoporosis.Repeat magnetic resonance scans after 1 month revealed reduction in the size of the tumor by 50%, with a residual lesion in the clival region. Her morning cortisol was 16 mcg/dL (441 nmol/L) and there was further decrement in diabetic and hypertensive medications. She underwent stereotactic radiosurgery for the residual clival mass 3 months after surgery. Six months after radiotherapy, her salivary cortisol was 4 mcg/L (11 nmol/L). Her diabetes and hypertension were well controlled with a single medication each. At the time of reporting—3 years after surgery—she has been keeping well without any further symptoms.Clival masses have a wide differential diagnosis and include chordoma, chondrosarcoma, plasmacytoma, metastasis, osteoblastoma, meningioma, developmental cysts, and EPA. EPA around sella are very rare and about 180 cases have been reported to date . In an eAll of the cases reported were in women. The presenting symptoms were predominantly classical Cushing syndrome phenotype except in 1 case (#2), in which it was purely mass related . Except for 1 patient (#4), all others had a macroadenoma with infiltration into surrounding structures.It has been hypothesized that the ectopic pituitary tissue arises because of failure of migration of pituitary tissue during embryonal period and, in that case, empty sella might be noted . None ofAn important investigation in ACTH-dependent Cushing syndrome with normal sellar imaging is IPSS. In a review of literature of ACTH-producing EPA, 100%, 83%, and 79% of suprasellar, sphenoid sinus, and cavernous sinus tumors had a central gradient, respectively. Caution needs to be exercised while interpreting IPSS with a central gradient in those having normal/empty sella because of shared venous drainage between ectopic pituitary tissues and the pituitary gland. In cases in which there are obvious radiological lesions around the sella, one cannot be sure of the source of Cushing syndrome (whether ACTH is from pituitary vs EPA). In case of occult radiological lesions around the sella (as was the case in patient #4), with IPSS showing central gradient, sellar exploration and surgery should be undertaken.8F-FDG and 8F-DOPA PET-CT negative but 68Ga-DOTATATE PET-CT positive tumor in the right sphenoidal sinus [18F-Choline PET-CT localized left maxillary sinus tumor in a patient in which initial 18F-FDG and 68Ga-DOTANOC scans were negative [18F-FDG was negative, 68Ga-DOTANOC localized the lesion and the lesion had higher standardized uptake value maximum compared with normal pituitary tissue.Normal pituitary expresses somatostatin receptors (SSTR) 1, 2, and 5. Novel somatostatin analogs used in nuclear imaging binds to avidly to SSTR 2 and SSTR 5 . The role of functional nuclear imaging in the diagnosis of ACTH-secreting EPA has been evaluated in a few patients. One was a case of al sinus  and the negative . In our The closest differential for ACTH-producing EPA is carcinoid tumor in the head and neck region. Though rare, sphenoid sinus carcinoid tumors have been reported in the literature. The histopathological features overlap between the 2 in the form of differential patterns of growth within the tumor, mild to moderate fibrovascular stroma, small dense cells with nuclear crowding, and overlapping. Destruction of nasal epithelium, numerous mitoses, smudging, and crush artifact, if present, suggest a neuroendocrine tumor. Imaging characteristics do not differentiate the 2. Neuroendocrine markers may be expressed in both tumors—about 97%, 76%, and 71% of EPA express synaptophysin, neuron-specific enolase, and chromogranin. Pituitary-specific transcription factors  are unique and specific to EPA.Surgical outcomes have been reported in ACTH-secreting EPA. Remission rates were 94%, 76%, and 60% in sphenoid sinus, suprasellar, and cavernous sinus tumors respectively. Two patients (#3 and #4) underwent a second surgery for complete resection of a clival tumor and were in remission after the second surgery. Another 3 patients underwent extensive debridement first and required postoperative radiotherapy. Although cases #1 and #2 received external beam radiotherapy, our patient received stereotactic radiosurgery. The long-term follow-up was not available in cases #1 and #5 but the other 3 cases were in remission.In conclusion, we summarize the findings of ACTH-producing clival EPA in this paper. Testing for the pituitary hormone hyperfunction is of utmost importance in working up a clival mass and, in any case of doubt, immunohistochemistry must be done for accurate diagnosis. Because of bony involvement, extensive surgery and added radiotherapy might be required in these cases.Ectopic pituitary tissue by itself is rare and such tissue becoming adenomatous and functional is much more uncommon.Parasellar adenomas that secrete ACTH may show central gradient in inferior petrosal sinus sampling.Multimodal therapy requiring surgical debulking and stereotactic radiotherapy with or without medications to control cortisol excess remains the cornerstone of case management.S.K. was involved in diagnosis and evaluation. T.K. and J.R.J. were involved in surgical and postoperative management. J.K. reported histopathology and did immunohistochemistry. All authors were involved in drafting and revising the manuscript.


Sildenafil is a selective phosphodiesterase type 5 inhibitor used for the treatment of erectile dysfunction and pulmonary hypertension. It is available over the counter in many countries. While there have been a few reports of retinal vascular occlusion following sildenafil consumption, most cases have other comorbidities as risk factors for the disease, and the exact causal role of this drug in these conditions remains unclear.We present the case of a healthy 32-year-old Iranian man who developed combined central retinal vein occlusion and retinal artery occlusion following sildenafil exposure. The patient underwent a hypercoagulative state workup for possible underlying risk factors. Additionally, we conducted a literature search on PubMed using the keywords: retinal vein occlusion AND Sildenafil OR Viagra, retinal artery occlusion AND Sildenafil OR Viagra, retinal vascular occlusion AND Sildenafil OR Viagra. To obtain more objective results in the reviews, we employed an adverse drug reaction possibility algorithm. The patient was found to be otherwise healthy, and ancillary tests were unremarkable. A literature review identified seven reports of retinal vascular occlusion following sildenafil use. In most of these cases, the role of sildenafil was not clearly established. To the best of our knowledge, our case achieved the highest score based on the algorithm compared with previous reports.Sildenafil may be associated with severe retinal vascular accidents in otherwise healthy young individuals. Sildenafil is a selective phosphodiesterase type 5 (PDE5) inhibitor approved for erectile dysfunction (ED) and pulmonary artery hypertension (PAH) . It induRetinal toxicity and side effects of sildenafil, including transient blue/green vision, blurred vision, and photosensitivity , have beA 32-year-old Iranian healthy man presented to us with sudden vision loss in the right eye 3 hours following 100 mg sildenafil consumption. The history for thrombophilia, diabetes mellitus, and hypertension was negative. No familial history of hypercoagulative diseases was noted. The patient reported no vigorous exercise and severe dehydration. Visual acuity was 20/200 OD and 20/20 OS. Intraocular pressure (IOP) was 12 mmHg OD and 14 mmHg OS. Ophthalmic examination revealed a three-plus positive relative afferent pupillary defect (RAPD) in the right eye. In fundus examination, a diffuse retinal hemorrhage, cotton-wool spots, subretinal exudation, dilated and tortured veins, and prominent optic disc swelling were evident. The extreme severity of exudation and retinal edema resulted in macular folding  injection for severe macular edema and hemorrhage. After three monthly intravitreal bevacizumab injections, the macular edema resolved but the vision did not improve due to severe macular ischemia and optic disc atrophy. To assess the ADR probability, we implemented the Naranjo algorithm Table  and the Sildenafil is a potent PDE5 inhibitor with a weak PDE6 inhibitory effect that is generally prescribed for ED and PAH; in many countries, it is available at pharmacies without physicians’ affirmation. The effects of sildenafil on the eye are usually divided into two groups: the dose-dependent effect of PDE6 inhibition on photoreceptors by controlling the level of cGMP that causes reversible blurred vision, blue–green tinge vision, light sensitivity, and decreased color vision, and the effects that are results of PDE5 inhibition; most of these are related to change in vascular structure and the coagulation system that can induce serious and irreversible retinal and optic nerve catastrophes. The effect of sildenafil on retinal and optic disc blood flow is controversial. Grunwald  showed tWe reported a 32-year-old healthy man with central retinal vein occlusion (CRVO) and retinal artery occlusion (RAO), 3 hours following 100 mg sildenafil use. Since the patient was otherwise healthy without known risk factors for vascular disease, negative ancillary test results directed to hypercoagulative states, and in addition the close temporal relation between sildenafil intake and incidence of the disease, we speculated that the drug could play a causal role in this clinical scenario.We employed the Naranjo algorithm  Table 11, for thet al. reported a 36-year-old man with CRVO, NAAION, and cilioretinal occlusion following sildenafil use [Retinal vein occlusion (RVO): In 2007, Gedik afil use . The patafil use , it is sThis was the most similar case to ours in the literature, but our case was more severe with extensive intraretinal hemorrhage, prominent optic disc swelling, exudative retinal detachment, and retinal ischemia that extended to the equator.et al. reported a 74-year-old man with pulmonary hypertension who presented with CRVO, 72 h after taking sildenafil [et al. reported a case of CRVO and cilioretinal occlusion in a 40-year-old, otherwise healthy man following 2-day consumption of sildenafil [et al. nor Hafidi et al. performed ancillary tests in this regard, and the “cause and effect” relation remains unclear.Pinto ldenafil . Hafidi ldenafil . While ret al. reported the first case of branch retinal artery occlusion (BRAO) in a 69-year-old man after taking a 100 mg Viagra pill [Retinal artery occlusion (RAO): In 2000, Tripathi gra pill . The autet al. reported the case of a 51-year-old man who experienced hemiretinal artery occlusion after using sildenafil during sexual activity [Bertolucci activity . The autet al. documented a 54-year-old man who developed NAAION and cilioretinal occlusion a few hours following the consumption of 200 mg of sildenafil [Akash ldenafil . The patet al. reported a 62-year-old man with NAAION and BRAO following an overdose of sildenafil [Sinha ldenafil . The patIn 2016, the Food and Drug Administration (FDA) reported the retinal vascular occlusions associated with phosphodiesterase type 5 inhibitor use. By the end of 2014, 82 cases of RVOs and 24 RAOs had been reported, with 32 and 12 of them having other risk factors and comorbidities, respectively. The FDA postulated that these diseases are multifactorial and recommended that ophthalmologists ask patients with retinal vascular occlusion about their PDE5 inhibitor consumption .et al. showed a decreased vessel density of retina and optic nerve in the group of participants consuming tadalafil for more than 6 months compared with the matched control group, and they theorize that this medication could damage retinal capillaries [The current study reported a case that achieved a Naranjo score of at least 4, which is the highest compared with previous reports and drew attention to possible serious adverse effects of sildenafil. However, the exact mechanism of this event is not clear. One hypothesis is that, in the setting of decreased systemic blood pressure, PDE5 inhibitors induces retinal vein engorgement, and since they share a common adventitia with central retinal artery at the level of lamina cribrosa, they compress central retinal artery that eventually leads to retinal ischemia, endothelial injury, vasospasm, and even central retinal artery occlusion . Second illaries . The vasillaries . We postCertainly, this study has inherent limitations that should be addressed in future investigations, specifically small sample size and a dearth of established treatment criteria for evaluating adverse drug reactions. Various algorithms for this purpose do not have a uniform scoring system, and weighting the parameters is not based on clinical possibility. Furthermore, some of these items are not applicable in all conditions. Some authors believe that these drawbacks make these algorithms less valid . On the We express concern regarding the possible association between sildenafil use and retinal vascular accidents, particularly among young, otherwise healthy individuals.


Coronavirus disease 2019 (COVID-19) is a disease characterized by predominantly respiratory symptoms, which can progress to respiratory failure. Due to the novelty of the vaccines, it is difficult to assess if there are any associated long-term side effects. Here, we present a case of an elderly female who received the Moderna COVID-19 vaccine and developed a high-grade sarcoma at the site of the injection. A 73-year-old female with a past medical history of hypertension, hyperlipidemia, and renal angiomyolipoma status post resection in 2019 presented with worsening right upper arm swelling for the past two weeks. She noticed the swelling two to four days after receiving her second dose of the Moderna vaccine within 1 cm from the prior injection site. Physical examination was remarkable for a 6 cm, circular, mobile, soft mass present in the right upper arm. MRI with and without contrast revealed a 5.2 cm soft tissue mass overlying the triceps region with irregular features concerning for malignancy. Fine needle aspiration revealed pathologic characteristics indicative of high-grade sarcoma. The patient ultimately had resection of the mass four months after the initial visit and was diagnosed as having grade 3, stage IIIA undifferentiated, pleomorphic high-grade sarcoma. Herein, we present a case demonstrating the development of high-grade sarcoma at the injection site in an elderly female patient within days of receiving the second dose of the Moderna COVID-19 vaccine. Currently, it is unclear whether there is a true association between the vaccines and malignancy or inflammatory response exacerbating underlying malignancy. This case highlights the necessity to investigate and be aware of such rare, adverse complications that may be associated with the novel COVID-19 vaccinations to guide physicians in their differential diagnosis. Coronavirus disease 2019 (COVID-19) is a disease characterized by predominantly respiratory symptoms that can progress to respiratory failure. Given the mortality and morbidity associated with COVID-19, novel COVID-19 vaccines were invented in response. Due to the novelty of the vaccines and the innovative mRNA technology the vaccinations utilize, it is difficult to assess if there are any long-term side effects associated with vaccination. Current literature reports headache, fever, fatigue, chills, and tenderness at the injection site as the most common short-term side effects . Based oA 73-year-old female with a past medical history of hypertension, hyperlipidemia, and renal angiomyolipoma status post resection in 2019 presented with worsening right upper arm swelling for the past two weeks. She noticed the initial swelling two to four days after receiving her second dose of the Moderna vaccine within 1 cm from the prior injection site. This was initially attributed to phlebitis. She reported mild, non-radiating pain on palpation. Vitals were unremarkable and the patient was afebrile. Physical examination was remarkable for a 6 cm, circular, mobile, soft mass present in the right upper arm without fluctuance, erythema, or warmth. She had no neurological deficits and radial pulses were present.Complete blood count (CBC) was unremarkable with no leukocytosis, leukocytopenia, or anemia. The comprehensive metabolic panel (CMP) was unremarkable. The patient was advised to obtain an MRI with and without contrast, which revealed a 5.2 cm soft tissue mass within the subcutaneous fat, overlying the triceps region, with irregular features concerning for malignancy Figure . A subseFNA revealed a hypercellular specimen consisting of bland spindle cells mixed with anaplastic cells, with multinucleation, pleomorphism, hyperchromatic, eccentrically located nuclei with eosinophilic cytoplasm, and brisk atypical mitotic features. Based on the examination, imaging, and FNA results, the patient underwent surgical resection with surgical oncology four months after the initial visit. At that point in time, the patient’s sarcoma was estimated to be 20 x 15 cm on physical examination. The patient was scheduled with surgical oncology for resection of the mass and had removal with clean margins. No spread to regional lymph nodes was evident. Grossly, the mass was 8.8 x 7.3 x 5.0 cm, tan-pink, well-defined, and soft, with a centrally located, bulging area measuring 2.3 cm from the nearest margin. The cut surface of the mass was tan-white to pale-pink, somewhat fleshy, lobulated, focally hemorrhagic, and rubbery-to-firm in consistency Figure . MicroscUndifferentiated pleomorphic sarcoma (UPS) was first described in the literature back in the early 60s as a form of undifferentiated fibroblasts formed in the background of histiocytic growth . Later, Based on an extensive search, we describe the first case of rapidly progressive, high-grade undifferentiated sarcoma that seems to have a strong association with the Moderna vaccination. Although the literature well documents the efficacy of the vaccines in immunocompromised patients such as those with malignancy , there aIt is well established that other vaccines may lead to the formation of subcutaneous nodules, particularly in children after the administration of the pertussis vaccine . A revieAlthough a non-malignant growth has been reported as a rare complication of the Moderna COVID-19 vaccine, it is important to consider other unique complications that may be implicated. It is well-documented in the literature for over 20 years that high-grade sarcomas have been linked to vaccine administration in felines . AlthougOur patient has a previous history of renal angiomyolipoma. Renal angiomyolipoma is often associated with tuberous sclerosis complex (TSC), which often presents with a variety of complications such as subependymal nodules, subependymal giant cell astrocytomas, skin lesions such as facial angiofibroma, renal cell carcinoma, cardiac rhabdomyoma, and so forth . AlthougInterestingly, another recent case report has reported spontaneous tumor regression following the administration of the Moderna COVID-19 vaccine . In thisHerein, we present a case demonstrating the development of undifferentiated, pleomorphic high-grade sarcoma at the injection site in an elderly female patient within days of receiving the second dose of the Moderna COVID-19 vaccine. Currently, it is unclear whether there is a true association between novel vaccinations and the development of malignancy. A review of the literature does not show any other case reports demonstrating malignancy after receiving the Moderna vaccine. This should be further investigated to see if there is an association and, if so, the mechanism thereof. Despite the uncertainty, it is necessary to note such cases of potentially rare, adverse complications as clinicians should consider the side effects of vaccinations in their differential diagnosis to guide patients in shared decision-making. Early recognition of the disease, as in this patient, proved to be vital for her quality of life and prevented further progression.


Intracochlear fibrosis is a rare disorder that can lead to hearing loss and make cochlear implantation challenging. The etiology of intracochlear fibrosis is diverse, including infections, inflammation, and past surgical procedures. The condition causes ossification and scar tissue growth within the cochlea, leading to progressive obstruction of the cochlear turn. High-resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) are sensitive diagnostic modalities for fibrosis and ossification. There is a paucity of information in the literature regarding cochlear implantation during the fibrotic stage.This case report discussed the presentation, diagnosis, and surgical management of intracochlear fibrosis in a patient with a history of sudden and severe hearing loss. A 44-year-old female patient with a 20-year history of sudden profound sensorineural hearing loss (SNHL) in both ears was successfully treated with cochlear implantation. Thorough preoperative planning for cochlear implantation, including HRCT and MRI cochlear protocol, is crucial for identifying intracochlear fibrosis, which can be missed on routine audiometry. She underwent a surgery for right cochlear implantation using postauricular approach. Drilling was done to the round window niche, and we removed an abnormal, chalky white bone we encountered by continuing to drill this abnormal bone following the scale tympani until we identified the opening of the scala tympani, then we inserted the cochlear implant device. She was doing well on the subsequent post-operative follow-up. Intracochlear fibrosis treatment with cochlear implantation has proven successful in several studies. Audiologic outcomes vary with time to implantation, so an early attempt should be made for cochlear implantation. Follow-up is important to monitor auditory outcomes Intracochlear fibrosis is a rare disorder that can be linked to various events, including infections, inflammation, or past surgical procedures. This condition causes ossification and scar tissue growth within the cochlea, which can lead to hearing loss and make cochlear implantation complicated. Labyrinthitis ossificans may develop after meningitis, labyrinthitis, temporal bone damage, stapedectomy, labyrinthectomy, autoimmune sensorineural hearing loss (SNHL), and lateral skull base surgery, particularly the translabyrinthine approach ,2. Even A 44-year-old woman presented to the otology clinic with a history of 24 years of sudden hearing loss that started in her left ear and, after one year, moved to her right ear with a profound degree of SNHL with an unclear etiology. She wore hearing aids only for one week before taking them off, as she did not get any benefit out of them. She was asthmatic with no other medical issues. She had her hearing test done, which showed bilateral profound SNHL  Figure , and sheClinical data for preoperative planning for cochlear implantation include a high-resolution CT scan for both temporal bones with coronal reconstruction acquired without IV contrast, as well as an MRI cochlear protocol. On bilateral CT scan, the cochlea was found to have relatively confluent hyperdense plaque of mineralization in the basal and middle turns just proximal to the round window, which is consistent with the Symons and Fanning grading system for retrofenestral otosclerosis grade 2c  with full insertion was implanted. One, three, and six months postoperatively, she had her audio processor switched on with a marvelous auditory outcome within two consecutive sessions, and she is doing excellent Figure . This case report highlights the importance of thorough preoperative planning for cochlear implantation, which includes an HRCT scan for both temporal bones with coronal reconstruction acquired without IV contrast, as well as an MRI cochlear protocol. These imaging modalities can help identify intracochlear fibrosis, which can be missed on routine audiometry.There are numerous causes of intracochlear fibrosis; bacterial meningitis is the most prevalent cause of labyrinthitis ossificans and profound acquired bilateral SNHL in childhood, with the highest incidence of hearing loss occurring in meningitis due to Streptococcus pneumoniae 31%) . Another% . AnothOne interesting thing in our case comes from the fact that ossification previously was thought to contraindicate cochlear implantation because of possible mechanical obstruction and uncertainty about the level of function that could be achieved by stimulating an ossified cochlea . HistopaWhether fibrosis removal or dilation was performed, there is no difference in the pure tone average (PTA) (p = 0.76), according to a previous study. Those who waited the longest to get surgery had the worst outcomes: four months after meningitis and 24 months after idiopathic abrupt SNHL . In our Intracochlear fibrosis is a rare condition, in which the cochlea is found to be ossified and/or fibrotic. It can occur post-meningitis, or due to a systemic autoimmune disease causing inner ear disease. It can also result from a translabyrinthine approach in temporal bone surgery. If there is no clear etiology, it is called idiopathic intracochlear fibrosis causing sudden SNHL. It can be diagnosed using a temporal bone CT scan and MRI, which can reveal fibrosis and mineralization within the cochlear turns, and narrowing, respectively. A successful treatment option is cochlear implantation after the limited fibrosis is removed or dilated if it is too extensive to permit implantation. According to several studies, good cochlear implantation audiologic outcomes vary with time to implantation, so intervention should be made as early as possible. The main goal after cochlear implantation is strict follow-up with the patient. We need further studies on the intracochlear fibrosis subject in the future, in order to increase awareness among physicians and to provide early diagnosis and treatment as soon as possible.


Acute gastric dilatation is an uncommon surgical pathology, leading to gastric ischemia, necrosis, perforation, sepsis, and death if untreated. While rare, the development of abdominal compartment syndrome is also a devastating complication of this entity. We present a case of a 42-year-old male with a history of gastric volvulus, presenting with severe acute abdominal distension and multi-organ failure. A diagnosis of acute gastric dilatation was made, with suspicion of abdominal compartment syndrome. Emergency laparotomy was performed when nasogastric decompression failed. Total gastrectomy without anastomosis was performed due to the patient’s hemodynamic instability. However, he demised shortly after on the operating table. This case report demonstrates that even with rapid diagnosis and management, acute gastric dilatation continues to be associated with high mortality. First described in 1883, acute gastric dilatation is an uncommon disorder, with most documentation of its occurrence in the surgical literature being case reports .,4,5.2,4,2O) or when the stomach is distended with three or more liters of contents [The ischemia associated with acute gastric dilation is thought to be due to venous congestion and insufficiency. Even with a rich collateral blood supply, this may occur when intra-gastric pressures exceed gastric venous pressure  over 20 mmHg  associated with new organ dysfunction or failure . In our As postulated by Abdu et al, the catastrophic complication of rupture is caused by mucosal necrosis, followed by full-thickness ischemia of the gastric wall and perforation. Perforation occurs when intragastric pressures exceed 120mmHg or four liters of intragastric content . The pubCharacteristic clinical features include nausea and vomiting , poorly localized abdominal pain, and distention . In someEarly radiological imaging, particularly abdominal X-ray, demonstrates a massively distended stomach with a fluid level, as illustrated in the case presented here. An X-ray may also indicate a pneumoperitoneum when perforation occurs. However, when rapidly available, computed tomography (CT) can also help identify the pathological cause . In thisRecognition of acute gastric dilatation and immediate decompression via nasogastric tube is the mainstay of treatment. Even after decompression, however, delayed perforation or bleeding is possible and this should be considered in planning treatment for these patients . In our When it comes to the ideal surgical approach, the underlying principle of resection of any gangrenous part of the stomach must be followed. Total gastrectomy with esophagojejunostomy and feeding jejunostomy is considered the procedure of choice, but it requires a considerable amount of operating time and a hemodynamically stable patient ,2. OtherIn our case, the patient was unstable, requiring high inotropic and ventilatory support settings. Due to the large lesser curvature perforation, persistent bleeding from the gastric mucosa, and full-thickness ischemia, a total gastrectomy without anastomosis appeared to be the only option for a damage control approach to treatment. To the best of our knowledge, this report appears to be the first documentation of total gastrectomy without anastomosis in a severely unstable patient with acute gastric dilatation. Few published case reports describe the successful completion of anastomoses in unstable patients with gastric dilatation and perforation .According to Jung et al, despite a curtailed operative time with a damage control approach, acute gastric dilatation is still associated with high surgical mortality rates of 50-65% and up to 100% when not treated appropriately .Acute gastric dilation is a potentially lethal condition with a myriad of etiologies. While uncommon, it is necessary for the attending clinician to be able to recognize the features of acute gastric dilation and to initiate early and rapid resuscitation, which includes intravenous fluids and nasogastric decompression. In many cases, non-operative management is successful, but emergent surgery is undertaken when non-operative management fails or complications like necrosis and perforation occur. The documentation of this case report highlights the importance of a rapid diagnosis of acute gastric dilatation and the need for definitive management.


The presented case report describes an incredibly rare instance of an intramedullary bronchial cyst located in the thoracic spinal canal on the dorsal side of the spinal cord, which was observed in a patient with chronic myelogenous leukemia. A 29-year-old man presented with back pain for half a month, along with numbness and pain below the chest and ribs for 1 week. Hypersensitivity was present in the inferior plane of the long xiphoid process in the nervous system. Magnetic resonance imaging (MRI) showed intramedullary cystic lesions in the vertebral body plane of the third to the fourth thoracic vertebra. There was no recurrence during the 6-month postoperative follow-up period. The histopathological findings were consistent with bronchogenic cysts. Cystic lesions were eliminated through the posterior median approach. After the cyst ruptured during surgery, gel liquid was seen, and the majority of the cyst walls were removed. One week after the surgery, the hypersensitivity fully subsided. Six months following surgery, an updated MRI revealed no recurrence. Intramedullary bronchogenic cysts on the dorsal side of the thoracic spine are extremely uncommon. Diagnosis requires histopathological evidence, and it is challenging to diagnose before surgery. Prompt surgical resection is recommended in case of positive diagnosis. It is an endodermal translocation tumor that frequently develops in the external spinal membrane of the cervical and thoracic spinal cords. Most of the sites are located in the ventral spinal cord. It rarely occurs in the spinal canal, and accounts for 10–15% of mediastinal masses. The first instance of an intraspinal bronchogenic cyst was reported by Yamashita in 1973 . However22.1A 29-year-old male patient was admitted to the Jincheng People’s Hospital, Shanxi Province, with back pain for half a month and numbness and pain below the chests and ribs for 1 week. A thorough physical examination was conducted upon admission. The patient was able to move his limbs based on instructions and could maintain autonomous postures. He had no swelling of superficial lymph nodes in the whole body, showed hypersensitivity below the xiphoid plane, and there was a symmetrical degree of hypersensitivity on both sides. The muscle strength of both lower limbs was grade V on the Lovett scale, the muscle tension was normal, the muscles of both lower limbs were not atrophied, and the pathological symptoms of both lower limbs were not induced. Past medical history: the patient was diagnosed with chronic myelocytic leukemia in the chronic stage in the Department of Hematology, Jincheng People’s Hospital in July 2016. The patient was orally administered imatinib mesylate tablets 400 mg/per time per day. Regular blood tests detected no abnormalities in the white blood cells or platelets. There was an intraspinal cystic lesion with hemivertebra deformity in the region of the third to the fourth thoracic vertebra on plain and enhanced magnetic resonance imaging (MRI) scans of the thoracic vertebra.The plain thoracic vertebra MRI scan is displayed in The initial diagnosis was a space-occupying lesion in the spinal canal of the third to the fourth thoracic vertebra.Informed consent: Informed consent has been obtained from all individuals included in this study.Ethical approval: The research related to human use has been complied with all the relevant national regulations, institutional policies and in accordance with the tenets of the Helsinki Declaration, and has been approved by the Ethics Committee of Jin Cheng People’s Hospital (JCPH No. 20230629001).2.2Cyst lesions were surgically resected using a posterior midline approach. During the surgery, a grinding drill is used to remove the entire vertebral lamina and spinous process, and the dura mater is incised. The partial vertebral plate and spinous process were also resected during the procedure, which also revealed a cystic tumor in the dorsal side of the spinal cord of the third to the fourth thoracic vertebra. There was no spinal cord pulse after the dura mater spinalis was incised. A fine needle was inserted into the cyst, which produced a gel-like fluid. The tumor was entirely resected under a microscope and the cyst wall was thin, grayish white, and translucent . On the 2.3The patient’s hypersensitivity was eliminated 1 week after the operation, and there was no sensation or movement disorder in his limbs. Following a complete MRI, no evident recurrence was discovered 6 months after the operation .3Intraspinal bronchial cysts are a rare congenital developmental anomaly of the respiratory system that arise from the pre-embryonic intestine. They account for approximately 0.7–1.3% of all intramedullary spinal cord tumors. The surface of the cyst is covered by respiratory epithelium ,5. (2) PClinical manifestations and symptoms of intraspinal bronchial cysts are generally site-related and non-specific. Neck and back pain, as well as sensory and motor abnormalities in the four limbs are the most typical symptoms. In this case, back and chest pain were the main symptoms of the patient.On MRI, the majority of intraspinal bronchogenic cysts show hypointense T1 and hyperintense T2 signal, and gadoteric acid meglumine does not clearly augment these signals. As it can be challenging to discern one condition from another using an MRI alone, a CT scan is required. Spina bifida and other congenital spinal abnormalities can be seen on a CT scan .Pathologically, intraspinal bronchogenic cysts are mostly characterized by the single-layer or pseudostratified columnar epithelium of the cyst wall, and glands, cartilage, smooth muscles, and nerve fibers may be present on the cyst wall. The fluid within the cyst is composed of various substances, with most exhibiting a gel-like or yellowish appearance. However, some portions of the fluid may be clear and colorless.The primary form of treatment for an intraspinal bronchogenic cyst is surgery. During the procedure, the spinal cord and nerve root should be safeguarded, and the total cyst wall resection should not be forced. It is reported that the cyst wall may be washed with diluted iodophor or ignited by local bipolar electrocoagulation micro-current after the cyst wall has been incised to liberate the cyst fluid . AdditioThe patient in our study had a bronchogenic cyst of the spinal canal complicated with chronic myelogenous leukemia. The preoperative surgical plan fully estimated the possibility of intraoperative bleeding, the number of white blood cells, red blood cells, platelets, and the coagulation function of the patient should be paid attention to in preoperative detection. As we all know, patients with leukemia often have coagulation dysfunction, resulting in excessive intraoperative field bleeding, and continuous postoperative bleeding leads to epidural or subdural hematoma, resulting in lower limb dysfunction . Therefo4Intraspinal bronchogenic cysts, especially those affecting the dorsal thoracic region, are extremely rare, and the case described in this report falls within this uncommon category. To confirm the diagnosis of this condition, imaging and clinical pathology examinations are essential. Surgery is generally considered the preferred method of treatment.


Liver tumors in children are uncommon and show remarkable morphologic heterogeneity. Pediatric tumors may arise from either the epithelial or mesenchymal component of the liver and rarely may also show both lines of differentiation. Both benign and malignant liver tumors have been reported in children. The most common pediatric liver tumors by age are benign hepatic infantile hemangiomas in neonates and infants, malignant hepatoblastoma in infants and toddlers, and malignant hepatocellular carcinoma in teenagers. Here, we provide an up-to-date review of pediatric liver tumors. We discuss the clinical presentation, imaging findings, pathology, and relevant molecular features that can help in the correct identification of these tumors, which is important in managing these children. Liver tumors constitute the third most common solid abdominal tumors in children, after neuroblastoma and Wilm’s tumor . Among tHemangiomas in children may be congenital or infantile, with the infantile type being more common. Hepatic congenital hemangioma (HCH) is a benign vascular tumor that develops in utero and is fully grown at birth, with no post-natal increase in size ,5,6. It HCH occurs most commonly as a solitary lesion in the right hepatic lobe and upon ultrasound showing a large mass with an extensive central infarction, cystic change, hemorrhage, calcifications, and sometimes an arterial feeding vessel that has direct shunts to hepatic veins. The use of magnetic resonance imaging (MRI) and computed tomography (CT) may show lesions with centripetal enhancements ,7,8. A dHistologically, HCH is composed of vague aggregates or lobules of variously sized capillary vessels lined by a single layer of round to oval benign endothelial cells with inconspicuous nucleoli and few pericytes  8]. The. The8]. Hepatic infantile hemangioma (HIH) is the most common benign liver tumor in infants and shows GLUT-1-immunopositivity., . Three sThere is no clarity regarding the natural history of HIH. Its clinical course is different to HCH, as it continues to proliferate until approximately 6 to 12 months old and then it gradually involutes until 3 to 9 years of life ,12. It iDiffuse or multifocal HIH shows homogenous or centripetal enhancement, adjacent flow voids, enlargement of hepatic arteries or veins, and arteriovenous shunts upon imaging . DiffuseMacroscopically, HIH shows white-tan nodules with occasionally degenerative changes in the centre. HIH is characterised by anastomozing, sinusoidal vascular channels  13]. Th. Th13]. Epithelioid hemangioendothelioma is an uncommon malignant tumor that is mostly reported in adults but can occasionally be seen in children . In chilHepatic epithelioid hemangioendothelioma demonstrates a distinctive growth pattern upon CT and MRI characterized by the peripheral distribution of neoplastic lesions, subcapsular retraction, and a “lollipop sign”, which consists of a hepatic or portal vein tapering and terminating at the periphery of the tumor .Firm, ill-defined, and sometimes confluent nodules with infiltrative borders are identified upon macroscopic examination ,20. HistHepatic angiosarcoma is a highly malignant tumor and is exceedingly rare in children. It may be present anytime from neonatal age up to adolescents, with a median age of 40 months. A female preponderance is seen .Neonates and infants may present with abdominal distension due to hepatomegaly or with abdominal compartment syndrome . In chilThe ultrasound results may show a mixture of hyperechoic and hypoechoic areas due to hemorrhage and necrosis. CT shows a hypoattenuating mass as compared to the surrounding liver parenchyma and may show hyperattenuating areas representing foci of hemorrhage . Using cA large single tumor or multiple tumor nodules, either separate or coalescing, sometimes with infiltrative borders replacing the hepatic parenchyma, are noted upon macroscopic examination. The cut surface can be variegated with areas of hemorrhage and necrosis. Cystic spaces and a fleshy appearance are also known to occur ,35. The Mesenchymal hamartoma (MH) is the second most common benign liver tumor in children, which usually presents before 2 years of age, with 15–20% patients presenting in the neonatal period. It has a slight male preponderance ,38,39,40MH may be detected prenatally using ultrasound examination or may present with prenatal complications such as pre-term labor, maternal toxemia, fetal hypdrops, and fetal demise. MH has a prominent cystic component and fluid accumulation within the cyst may lead to rapid enlargement of the mass, leading to massive abdominal distension, diaphragmatic compression, and respiratory distress ,39,42. IThe mass may be predominantly cystic with thick or thin septae or it may be solid with few cystic spaces. Thus, the imaging features may vary depending on the percentage of solid and cystic components. Using ultrasound, cystic MH is anechoic with thin or thick echogenic septa. Low-level echoes may be seen within the fluid, reflecting the gelatinous contents. Round hyperechoic parietal nodules within the cystic spaces are specific for MH. The solid component is isoechoic to the surrounding liver and shows minimal vascularity. The mixed solid cystic tumor shows a sieve-like appearance upon ultrasound with multiple anechoic cystic areas and intervening isoechoic to hyperechoic solid tissue ,44,45,46MH may show great variations in size ranging from a few centimetres up to greater than 30 cm . TypicalHematoxylin and eosin staining alone is sufficient to provide a diagnosis of MH. The biliary epithelium and entrapped hepatocytes show immunoreactivity for pancytokeratin stains, while the pseudocysts and mesenchymal cells show vimentin expression. Immunoreactivity to smooth muscle actin, α-1-antitrypsin, and desmin is also reported in the mesenchymal component . RecurreInflammatory myofibroblastic tumors (IMFTs) of the liver are uncommon mesenchymal neoplasms, mostly reported as case reports or small series in the literature ,62. IMFTThe patient can present with fever, abdominal pain, weight loss, abdominal mass, vomiting, or diarrhea. Jaundice caused by either the presence of a porta hepatis tumor or involvement of the hepatic hilum has also been reported ,65,66. TThe imaging features are non-specific and pose difficulties in providing a correct diagnosis. Using ultrasound, IMFT appears as an isoechoic or hypoechoic lesion . The masIMFTs may be solitary or multiple, being well-circumscribed, firm, and white-tan, with a solid or whorled cut surface. Focal necrosis may occur. The IMFT is composed of sheets and loose fascicles of spindle cells admixed with inflammatory cells, embedded in a variably myxoid to collagenous stroma . The spiThe kidneys are the most common site for malignant rhabdoid tumor (MRT), with primary liver MRT being rare. It is a malignant tumor, with most patients presenting before 1 year of age. MRT of the liver is highly aggressive and approximately 60% of patients show metastasis at presentation and 90% of patients die of the disease ,74.MRT of the liver presents with fever, abdominal distension, abdominal pain, anorexia, or vomiting. Upon examination, hepatomegaly or a right upper quadrant mass may be identified ,77,78,79The use of ultrasound shows a heterogeneous solid mass that may have cystic changes and calcification ,77,79,82Macroscopically hepatic MRT presents as a large lobulated mass often located in the right lobe with frequent areas of necrosis and hemorrhage. MRT shows sheets of asymmetrical, discohesive, undifferentiated, polygonal tumor cells with eccentrically placed vesicular nuclei, prominent nucleoli, and an abundant, brightly eosinophilic cytoplasm . The claEmbryonal sarcoma of the liver (ESL) is an aggressive mesenchymal tumor accounting for 9–15% of all pediatric liver tumors and is the third most common childhood hepatic malignancy after hepatoblastoma and hepatocellular carcinoma ,91,92,93The patient presents with abdominal pain, an abdominal mass or distension, weight loss, and vomiting ,90,91,92ESL is a solitary lesion that is more common in the right lobe of the liver . Using uThe lesion is a large and well-circumscribed expansive mass with a fibrous pseudocapsule. The cut surface is solid and yellow-tan with cystic and myxoid or gelatinous areas. Foci of hemorrhage and necrosis may be seen ,92. HistHepatobiliary rhabdomyosarcoma (RMS) is a malignant tumor of myogenic origin that may present as an intraluminal biliary mass or cluster of grapelike masses ,106. HepThe tumor occurs mainly in children at a median age of 3 years, with a clear preponderance of boys. Hepatobiliary RMS typically presents with jaundice and obstruction of the bile duct, and occasionally cholangitis.Radiological studies usually demonstrate a hypoechoic intraductal or periductal solid cystic lesion with dilation of an obstructed biliary tract.Embryonal RMS is variably cellular, ranging from stellate cells in an abundant myxoid stroma to sheets of poorly differentiated, densely packed rounded cells with round to slightly angulated hyperchromatic nuclei  107]. E. E107]. Children with absent or severely impaired T-cell function, such as from primary immunodeficiency (PID), post-organ transplantation immunosuppression, or acquired immunodeficiency syndrome (AIDS), commonly develop Epstein–Barr virus (EBV)-induced lesions owing to uncontrolled proliferation of the infected cells ,109,110.The clinical signs of EBV-SMT are non-specific and mainly depend on the location, size, and organ displacement or disruption . EBV-SMTThere are no pathognomonic imaging features for EBV-SMT and a biopsy is essential for a formal diagnosis .Histologically, EBV-SMT is primarily composed of spindle to elongated neoplastic cells with an eosinophilic cytoplasm and indistinct cytoplasmic borders predominately arranged in interlacing fascicles resembling leiomyomas or leiomyosarcomas  108,109109,110. Hepatocellular adenomas (HCAs) are rare pediatric liver tumors. HCA is present in adolescents with a mean age of 14 years and comprises less than 5% of all pediatric liver tumors ,114. TheHNF1A mutations, and maturity-onset diabetes of the young type 3, among others [HCA is discovered incidentally upon imaging but patients may present with abdominal pain. Approximately 25% of patients across all age groups present with tumor rupture and intratumoral or intraperitoneal hemorrhage . This isg others ,114,128.The appearance of HCA on imaging is homogeneous and similar to the surrounding liver, but those with hemorrhage and fat show varied features . Using uMacroscopically, HA appears as a well-demarcated lesion with thin or no fibrous capsule . HistoloFocal nodular hyperplasia (FNH) comprises 2–4% of all pediatric liver tumors, with an incidence rate of 0.02% in children ,137,138.Most FNH cases are detected incidentally during imaging and the patients are usually asymptomatic . Around Using ultrasound, FNH appears as a homogeneous, well-circumscribed mass with variable echogenicity, central or eccentric vascular supply, and a central stellate hyperechoic scar ,147. In Grossly, FNH is a well-circumscribed lesion with a central scar in 70% of cases . CentralHepatoblastoma (HB) is the most common primary malignant liver tumor in children . The incChildren with HB present with abdominal pain, distension, and a palpable abdominal mass, along with other non-specific complaints such as anorexia, fatigue, and weight loss . The preUsing ultrasound, HB appears as a lobular, well-circumscribed hypoechoic or heterogeneous lesion. Calcification, necrosis, and vascular invasion may be seen. Congenital HB may be detected using prenatal ultrasound, and such pregnancies may be complicated by polyhydramnios secondary to gastrointestinal compression by the mass, resulting in fetal hydrops . Using CA macroscopic examination is important in hepatoblastoma. Hepatoblastomas usually involve the right lobe and present as a solitary tumor but can be multifocal. The cut surface is usually lobulated and may be whitish , yellow,Hepatoblastoma is classified broadly into epithelial and mixed epithelial and mesenchymal subtypes, and their histopathological features are summarized in Crowded fetal HB shows closely packed cells, and the light and dark areas seen with the well-differentiated histology are not evident. The increased nucleocytoplasmic ratio gives this subtype a more crowded appearance . NuclearThe small-cell undifferentiated (SCUD) subtype accounts for ~5% of all HB cases . HistoriThe mixed epithelial and mesenchymal type is subdivided into two categories based on the presence or absence of teratoid features. The most common mesenchymal element is osteoids  followedHepatocellular carcinoma (HCC) is the second most common primary liver cancer in children after HB . PediatrHCC presents as an abdominal mass and pain. In the late stages, there may be jaundice and cachexia . PatientUsing ultrasound, the lesion appears as a heterogeneous hyperechoic mass with increased vascularity . Using cSeveral histological subtypes of HCC have been reported in adults . In pediFibrolamellar HCC accounts for 1% of all HCC and is more common in children and adolescents as compared to adults ,207. GroHepatoblastoma with carcinoma features (HBC) is a newer entity that displays histological and molecular characteristics that are a combination of HB and HCC. HBC is a genetically unstable tumor with high mutation rates and a poor outcome . In 2014Biphasic HCN NOS, which shows distinct areas with HB- and HCC-like features;Equivocal HCN NOS, with features intermediate between HB and HCC;Hepatoblastoma with a focal macrotrabecular pattern, pleomorphism, and anaplasia (HBFPA).HBC includes 3 histologic categories of tumors:The second category, i.e., an equivocal hepatocellular neoplasm, resembles HB but shows a macrotrabecular pattern, acinar pattern, high N/C ratio, pleomorphism, anaplasia, a high mitotic count, and has giant tumor cells. HBFPA may focally show pleomorphic cells with large irregular hyperchromatic nuclei, intranuclear inclusions, prominent nucleoli, and atypical mitosis. Sumazin et al. accomplished the molecular profiling of theses tumors and found Wnt signaling pathway gene alterations  in all patients with HBC. Further, 57% of patients showed alterations in pathways associated with stem cell pluripotency, including PI3K-AKT or mTOR signaling, as also seen in HCC. Mutations in genes associated with aggressive cancers, such as TERT, FGFR4, and KMT2C, were seen in 51%, 13%, and 10% of patients, respectively.Benign and malignant pediatric liver tumors show a wide variety of morphologic features and pose a significant challenge to a surgical pathologist. There is a continuous evolution in the pathologic classification of pediatric liver tumors as we update our understanding via molecular characterization and the correlation with its morphology. The proper utilization of immunohistochemistry markers helps in the accurate characterization of these tumors in daily practice. The use of international, multicenter, collaborative efforts and consensus workshops holds promise for furthering our understanding of these uncommon tumors. We hope that the pathological description and use of ancillary techniques described in this review will help pathology trainees, fellows, and practicing pathologists navigate through this group of diagnostically challenging tumors.


Epidermoid cyst in the oral cavity is uncommon. It is even more rare to see an epidermoid cyst in the sublingual region. We report the case of a 30-year-old male presenting with a swelling in the floor of the mouth extending into the submental and submandibular regions. The midline swelling was painless, soft, and dome-shaped. CT scan contrast revealed the site and extent of swelling. The complete surgical excision of the lesion was performed via a transcervical approach. Histopathology revealed cystic fibrocollagenous tissue covered by squamous epithelium containing some keratin flakes. Epidermoid cysts are benign lesions, arising from the growth of epidermal cells in a confined area of the dermis . They maEpidermoid, dermoid, and teratoid are the three types of squamous epithelium-lined cystic malformations, according to Meyers classification, with dermoid cyst often used as a broader term encompassing all of these. Etiologically, they may develop due to trauma and dysodontogenesis and from thyroglossal cyst .In the floor of the mouth, they may occur above or below the mylohyoid muscle presenting as an asymptomatic painless midline swelling with slow but progressive growth . When thA 30-year-old male came to our department with the complaint of a painless swelling in the floor of the mouth, bulging into the submandibular region Figure . Over maWhen the oral cavity was examined, a reddish dome-shaped enlargement was seen covering the entire gingivo-lingual sulcus and extending up to the anterior faucial pillars. It was soft, fluctuant, and compressible but non-reducible. Moreover, it was non-tender and non-pulsating.Upon the examination of the neck, there was an inverted dome-shaped 13 × 11 cm swelling in the submental and submandibular regions extending from the angle of the mandible to the hyoid bone inferiorly. There was no discoloration of the overlying skin. Transillumination was absent. There was no enlarged cervical lymph node palpated.A CT scan of the head and neck with contrast was performed. It showed a large mass of 10 × 7 × 5 cm with a fat-fluid level, extending from the soft palate to the floor of the mouth and submental region Figure . It was The surgical removal of the lesion was planned. General anesthesia was given following intranasal fiber-optic intubation . The lesion was approached via the neck Figure  through The excised specimen was sent for histopathological evaluation, which revealed cystic fibrocollagenous tissue focally covered by squamous epithelium. The cyst showed a denuded lining and some keratin flakes Figure . The tisEpidermoid cysts are nonmalignant inclusion cysts lined by the ectoderm alone . EtiologHistologically, according to Meyers classification, these squamous epithelium-lined cystic malformations are classified as epidermoid, dermoid, and teratoid. They all contain keratin and sebaceous fluid. However, epidermoid cysts are lined by only epidermal-type squamous epithelium, while dermoid cysts in addition include skin appendages, and teratoid cysts have their lining derived from all three germinal layers . AnatomiClinically, epidermoid cysts are usually painless and asymptomatic. However, larger ones may push the tongue backward interfering with mastication, articulation, swallowing, and sometimes respiration. Moreover, they are well-encapsulated, slow-growing cysts and soft to doughy in consistency and usually present without lymph node involvement -7.The differential diagnoses of epidermoid cyst can broadly be classified as infective , neoplastic , developmental , and anatomic abnormalities  and mucous extravasation (ranula). The absence of inflammatory signs helped us exclude infectious lesions. While the benign appearance and the lack of lymph node involvement reduced the likelihood of malignancy -11. SincImaging methods are useful in establishing the diagnosis alongside clinical examination. Despite the fact that MRI, CT, and US are of immense utility in diagnosing the lesion and surgical planning, we can only shortlist differentials on the basis of imaging. Fine-needle aspiration cytology (FNAC) also has a low sensitivity and specificity for the diagnosis of these lesions. Hence, histopathology is the only effective method for accurately diagnosing the disease .The complete surgical excision of the cyst is the recommended treatment in these cases to validate the diagnosis and relieve the patient from difficulty in mastication, articulation, and swallowing. Any obstruction to the laryngeal airway due to the posterior bulge of the tongue may lead to grievous consequences; hence, surgical excision is necessary. Alternatively, marsupialization with intralesional packing can be done . The exaAlthough intraoral cystic lesions in the floor of the mouth are rare, epidermoid inclusion cyst should be considered in the differential diagnosis. The treatment of epidermoid cysts in the floor of the mouth is surgical and can be intraoral or extraoral according to the localization and the size of the lesion.


A 13-year-old girl presented with an apparent classic osteochondroma. Because she was skeletally immature, the decision was made to observe the lesion. She returned to the clinic at age 17 for unrelated reasons and was noted to no longer have the palpable mass. Magnetic resonance imaging confirmed resolution of the osteochondroma. The age range of this case fits with reported cases of childhood osteochondromas. The mechanism of resolution has been theorized to be incorporation of the lesion back into the bone during remodeling, fractures, or pseudoaneurysms. An initial period of observation is thus recommended with new patients. Osteochondromas are very common skeletal lesions resulting from a herniation of part of the growth plate; they are generally not considered to be a neoplastic process. Osteochondromas grow away from the plate and have a characteristic appearance on X-ray. Solitary lesions are the norm, but cases exist of multiple osteochondromas as hereditary conditions. No standardized treatment guidelines exist, as treatment varies according to the symptoms associated with the condition. Asymptomatic cases are generally observed. Surgery is reserved for lesions that result in pain or decreased function because of their location. Thirty-one published cases of spontaneous resolution have been described , and theInformed consent was obtained from the patient for the publication of this case report and accompanying images. A 13-year-old girl presented to the orthopedic clinic complaining of a noticeable mass on her right knee for the past six months. She was experiencing intermittent knee pain and a “popping” feeling associated with bending the knee joint. She reported no pain on palpation. X-ray imaging revealed a bony exocytosis projecting superiorly from the right medial femoral condyle with features of an osteochondroma . Repeat X-rays were taken of the right distal femur Figure , which sMagnetic resonance imaging (MRI) was performed for diagnostic purposes of the mechanical problems Figure  and confThis case demonstrated spontaneous resolution of an osteochondroma in a skeletally immature patient who presented to the clinic with mild symptoms in her right knee. An osteochondroma at the medial epicondyle of the femur was noted on X-ray. Symptoms resolved after a course of physical therapy, despite continued presence of the osteochondroma. Her last follow-up was at age 17, where the resolution was observed via X-ray and MRI. The exact mechanism for her resolution is uncertain.Osteochondromas generally arise during childhood and account for approximately 30% of benign bone tumors. They consist of a cartilaginous cap and protrude from underlying bone, most commonly at the metaphysis ,3. OsteoSpontaneous resolution of an osteochondroma is an extraordinarily rare event, sparingly reported in the literature. Durán-Serrano et al.  discoverThe mechanism for resolution is under debate. Paling  first dePaling’s theory appears to be the most applicable to our case. Our patient was 13 years old at the time of presentation, and the amount of radial growth in her bones may have been limited at the time. She reported no traumatic injuries, excluding trauma, and no pseudoaneurysm was noted at the initial or follow-up clinic visits.Age at presentation may be the single most important factor in the potential for resolution, as the bones of younger patients will experience the most radial growth and remodeling. According to Durán-Serrano et al., 29 of the 31 reported resolutions were diagnosed before the age of 14 -14, withIn conclusion, while spontaneous resolution of osteochondromas may be considered a rarity, the event may occur much more frequently than appreciated. Future research, increased awareness, and alertness in daily clinical practice is needed, as an unknown number of tumors may be undiagnosed and thus unreported. While not unique, this additional patient brings the published case series total to 32 for a lesion that may occur in close to 5% of children.We recommend periodic follow-up and nonoperative management of osteochondromas in skeletally immature children who are asymptomatic or present with mild symptoms. Spontaneous resolution is a modest, yet possible, outcome in patients who have yet to reach skeletal maturity.


Burkitt lymphoma (BL) is an aggressive, high-grade B-cell lymphoma common in children and young adults. Despite being frequently discovered in extranodal sites, BL rarely occurs in the pancreas. We present a case of a patient with BL presenting as obstructive jaundice. Burkitt lymphoma (BL) is an aggressive type of non-Hodgkin lymphoma characterized by its rapid growth, propensity for extranodal involvement, and association with Epstein-Barr virus (EBV). BL typically presents with widespread lymphadenopathy, constitutional symptoms, and involvement of extranodal sites including the jaw, gastrointestinal tract, and bone marrow. However, in rare instances, BL can manifest with unusual clinical features, including obstructive jaundice, obscuring the diagnosis for physicians. Obstructive jaundice can result from various etiologies impeding the bile ducts. The occurrence of obstructive jaundice as the primary presenting symptom of Burkitt lymphoma is exceedingly rare, making it a diagnostic challenge for physicians. Atypical presentations, like obstructive jaundice, delay diagnosis and appropriate management, impacting patient outcomes. We present a case of a patient with BL presenting as obstructive jaundice and a literature review of non-Hodgkin lymphoma cases presenting similarly.A 23-year-old male with no past medical history presented to the emergency department (ED) multiple times with left arm pain, suspected to be due to a muscular injury, and was treated with tramadol and meloxicam. On his first ED visit, a computed tomography (CT) scan of the abdomen and pelvis with contrast was obtained due to elevated transaminases and showed no acute intra-abdominal pathology. During subsequent ED evaluations, the elevated transaminases persisted. He again presented to the ED, this time with a scleral icterus. An abdominal ultrasound showed a small amount of sludge in the gallbladder with mild diffuse gallbladder wall thickening. He was admitted for further evaluation of his worsening transaminases and hyperbilirubinemia. Magnetic resonance cholangiopancreatography showed a mild dilatation of the intrahepatic bile ducts, which was thought to be secondary to a lesion in the head of the pancreas. A second, larger lesion was also seen in the uncinate process of the pancreas Figure .Endoscopic retrograde cholangiopancreatography was subsequently performed, and two stents were placed, which resulted in an improvement in the jaundice. An endoscopic ultrasound-guided fine needle aspiration of the mass in the pancreatic uncinate process was performed. Examination of the direct smears and cell block preparations shows sheets of monotonous intermediate-sized cells with a starry sky appearance Figures , 3, 4.Numerous tingible body macrophages are noted and account for the starry sky morphology. The neoplastic cells demonstrate round nuclei with finely clumped chromatin and several paracentral nucleoli Figure .The mitotic rate is high with single-cell necrosis and increased apoptotic activity. Ancillary immunohistochemical staining was performed on the cell block, showing the neoplastic cells to express CD20, CD10, Bcl-6, and EBV Figures , 8.The Ki-67 proliferation index was >99%  analysis showed abnormal results with evidence of MYC (8q24) gene rearrangement and t in 87% and 88% of cells examined, respectively. FISH analysis was negative for BCL2 (18q21) and BCL6 (3q27) gene rearrangements, which helped exclude other high-grade B-cell lymphomas. There was evidence of right cervical lymph node involvement in addition to the two extranodal pancreatic masses consistent with stage IV BL. The patient was started on hyper-cyclophosphamide, vincristine, doxorubicin (Adriamycin), dexamethasone (CVAD), and rituximab, as well as intrathecal methotrexate and cytarabine. Three months after starting therapy, repeat positron emission tomography/computed tomography (PET/CT) showed resolution of lymph node disease and pancreatic masses.BL is an aggressive, high-grade B-cell lymphoma most commonly found in children. It only accounts for <5% of lymphomas in adults . DiffereEarly tumor classification is essential for patients to receive prompt and appropriate treatment. For instance, surgical resection is the only potential curative option for early pancreatic adenocarcinoma, which typically presents in a 60- to 80-year-old patient population. Whereas primary pancreatic lymphoma (PPL) is usually diagnosed at a mean age of 53 years old . As tumoA literature review was conducted to identify similar cases of NHL presenting with obstructive jaundice. Six of the ten cases were biopsy-confirmed BL, with the remaining four cases identified as other B-cell lymphomas. Anatomically, five of the ten cases were peripancreatic lesions; two involved the common bile duct; one had a hepatic etiology of disease; and two had duodenal disease causing obstruction. The reviewed cases have been summarized in Table In conclusion, primary pancreatic BL is a rare and aggressive neoplasm that should be considered in the differential diagnosis of young patients presenting with obstructive jaundice. Early recognition is pivotal as it facilitates the timely implementation of tailored treatment approaches, which in turn leads to enhanced clinical outcomes and an improved overall prognosis for affected individuals. Medical professionals must maintain a high index of suspicion, utilize advanced diagnostic modalities, and collaborate across disciplines to ensure optimal care for patients with primary pancreatic BL.


Malignant melanomas occurring in the central nervous system are mostly metastatic. Primary intracranial malignant melanomas are a rare entity, accounting for 0.07% of all brain tumors. In the central nervous system, melanocytes originate from the neural crest cells that are found in the leptomeninges. Only a few cases of malignant melanoma primarily arising from the brain have been reported in the literature to date.We report a rare case of primary intracerebral malignant melanoma in a 39-year-old female. Through this case report, our aim is to highlight the role of imaging in the early diagnosis and management of malignant melanoma. Most malignant melanomas of the central nervous system are metastatic, constituting 12-17% of all intracranial malignancies. Primary intracranial malignant melanomas are rare entities. These aggressive primary melanomas account for 0.07% of all brain tumors [Malignant melanomas arise from the melanocytic cells that are mostly present in the uvea, cerebral parenchyma, leptomeninges, mucous membranes, and skin . In the Prognosis of primary intracranial malignant melanoma is variable depending on the tumor site, the accuracy of the diagnosis, and the extent of tumor growth. The presenting symptoms of patients with primary intracranial malignant melanoma include raised intracranial pressure 43%), a neurological deficit (35%), or subarachnoid hemorrhage (16%) [3%, a neuIn our case report, we will discuss the clinical presentation and radiological findings of primary intracerebral malignant melanoma arising from the left insular cortex.A 39-year-old female presented to our hospital with complaints of headaches for the last two years that had worsened in the last two months. The headache was associated with numbness on the left side and epistaxis for two days. There was no history of prior surgery. On thorough clinical examination, no visible cutaneous lesion was identified. The patient then underwent a contrast-enhanced brain MRI that showed a 4.1 x 4.2 x 3.8 cm mass in the left insular region, demonstrating a peripheral rind of thick nodular contrast enhancement along with vasogenic edema and a midline shift of 15 mm. The mass was encasing the left middle cerebral artery. Fluid attenuated inversion recovery (FLAIR) hyperintense signals were also seen involving the left anterior temporal lobe, left basal ganglia, left insular region, and left frontal lobe  for the EWSR1 gene was done and turned out to be negative. Human melanoma black-45 (HMB-45), Melan A, and SRY-box transcription factor 10 (SOX 10) stains were positive. So the final diagnosis of primary intracranial malignant melanoma was made.Fluorodeoxyglucose (FDG)-positron emission tomography/computerized tomography (PET/CT) was performed after a craniotomy and biopsy that demonstrated hypermetabolic left frontal-parietal region mass. The rest of the scan was negative for abnormal FDG uptake. No bowel-related or cutaneous mass was noted Figures -4.A limitation of our case report is not excluding a bowel melanoma lesion by endoscopy. However, positron emission tomography (PET) is a sensitive examination, and no abnormal activity was noted.The post-craniotomy CT scan of the brain also demonstrated heterogeneously enhanced mass with surrounding vasogenic edema involving the left frontal-parietal region and persistent midline shift Figure .During the course of her admission, our patient developed diffuse cerebral edema and raised intracranial pressure. Mannitol infusions were given but the Glasgow Coma Scale (GCS) kept on lowering. Three fractions of 60 Grey' whole-brain radiotherapy were given along with intravenous dexamethasone (4 mg) during the hospital stay; a 0.5 mg oral dose and four tablets twice a day were prescribed upon discharge. Despite treatment with radiotherapy and steroids, our patient could not survive and passed away after five months due to cerebral edema and raised intracranial pressure before any systemic treatment could be given.Melanoma originates from the melanocytes and can occur possibly anywhere in the human body. Melanocytes are predominantly found in the skin, mucous membranes, leptomeninges, cerebral parenchyma, and uvea . PrimaryPrimary cerebral melanomas pose difficulty in preoperative diagnosis. The imaging features are also variable. On a CT scan, typical primary cerebral melanomas are hyperdense with intense post-contrast enhancement, like in our case. Intratumoral hemorrhage and melanin are the usual causes of increased attenuation on a precontrast CT scan . HoweverThe mainstay of treatment for primary cerebral melanomas is complete surgical resection followed by postoperative radiotherapy -12. DacaPrimary malignant melanomas arising from the central nervous system are rare entities. They pose difficulty with a definitive diagnosis owing to their nonspecific presentation and imaging findings. The correlation between immunohistochemical staining and imaging findings provides the most definitive method to diagnose primary intracranial malignant melanomas and differentiate them from other tumors. The mainstay of treatment includes surgery and combination therapy with radiotherapy, chemotherapy, and immunotherapy. However, there is still room for improvements in targeted therapies to provide more efficient treatments for primary cerebral melanomas.


Glioblastoma (formerly named glioblastoma multiforme) is the most common primary central nervous system tumor, representing 45% of all cases and 15% of all intracranial neoplasms [1]. Its typical radiologic findings and localization make it often a lesion easy to diagnose. In MRI it usually appears as an irregularly shaped cystic lesion with ring contrast enhancement in T1-weighted images, localized in subcortical white matter and deep gray matter nuclei of the cerebral hemispheres. It involves more frequently the frontotemporal region followed by parietal lobes [1]. Few articles in literature described cases of intraventricular glioblastomas, defining those as secondary ventricular tumors because of their probable origin primarily from cerebral tissue with consequent transependymal development . Atypical presentations of these tumors make it more difficult to clearly differentiate them from other lesions more commonly located in the ventricular system. We describe a case with a unique radiological presentation: an intraventricular glioblastoma lying entirely within the ventricular walls, involving all the ventricular system, without mass effect or nodular parenchymal lesions. A 56-year-old woman with history of ankylosing spondylitis presented with a 2-month drug-resistant headache and short-term memory loss. Brain MRI showed an enhancing multinodular lesion with dissemination along the walls of all the ventricular system Fig. . Total bThe integrated histological diagnosis with molecular analysis was compatible with glioblastoma IDH-wildtype, grade 4 according to CNS WHO 2021 criteria  is the most common primary central nervous system tumor representing 45% of all cases and 15% of all intracranial neoplasms [The peculiar distribution following only the ependymal lining instead of white matter tracts is anecdotic, but it may support the hypothesis that the glioblastomas’ cell of origin (COO) is a neural precursor cell located in the subventricular zone (SVZ) along the lateral ventricular walls. Lee and Manzano stated that possible alternative origins of intraventricular glioblastomas could be the septum pellucidum or the fornix, both components of the limbic system : the invWe here present this case because of its uniqueness: to our knowledge no such cases with subependymal distribution in all ventricular walls, without nodular appearance or mass effect on brain parenchyma, has been described in literature.


Hepatic hemangioma is a common benign vascular hepatic lesion with typical imaging features. However, hepatic hemangiomas with atypical radiological characteristics can sometimes be diagnostically challenging. Here, we report a case of an elderly patient with colonic adenocarcinoma who was incidentally found to have an atypical hepatic hemangioma showing a progressive centrifugal enhancement pattern instead of a typical centripetal pattern on contrast-enhanced computed tomography mimicking a malignant liver lesion. Hepatic hemangioma, or hepatic venous malformation, is the most common benign vascular liver lesion with well-established imaging characteristics. A typical hepatic hemangioma is a well-defined hyperechoic lesion on ultrasound which is a hypoattenuating lesion on computed tomography (CT) showing discontinuous, nodular, peripheral enhancement on the arterial phase with progressive centripetal fill-in on the portal venous, venous, and delayed phases . HoweverA 65-year-old male who presented with decreased appetite, lethargy, significant weight loss, and hematochezia was found to have iron deficiency anemia and was advised to undergo a contrast-enhanced CT scan of the abdomen. The blood hemoglobin level of the patient was 8.6 g/dL at the time of presentation. However, he was hemodynamically stable. His liver function tests had normal findings, and his tests for viral markers (hepatitis B and C) were negative. After analyzing the pre-contrast images, the radiology team advised a triphasic contrast study of the abdomen after giving neutral oral and rectal contrast. On post-contrast images, irregular mural thickening and luminal narrowing involving the rectosigmoid junction Figure , associaThere was active contrast extravasation into the bowel lumen at the same site from a prominent branch of the superior rectal artery associated with the progressive pooling of contrast material within the lumen of the sigmoid colon Figure . HoweverA well-defined, hypodense lesion measuring 4.8x4.6cm and showing central hyper-enhancing areas on arterial phase images with a progressive, centrifugal, or inside-out pattern of enhancement on portal venous, venous, and delayed phase images was noted in segment VI of the liver (Figure A peripheral, unenhanced portion was still visible on five-minute delayed images. There was no evidence of dilatation of the common bile duct or intra-hepatic biliary radicles. No other differentially enhanced lesions were found in the liver. Although the imaging findings were atypical, considering the age and clinical features of the patient, hepatic metastasis from a malignant colonic neoplasm was given as the most likely diagnosis. However, an alternate possibility of hepatic hemangioma with an atypical enhancing pattern was also suggested.The interventional radiology team took the patient for digital subtraction angiography. Endovascular intervention was preferred over endoscopic examination since the patient had active hemorrhage from a prominent and tortuous branch of the superior rectal artery and had not undergone bowel preparation. Selective catheterization and endovascular coil embolization of the actively bleeding branch of the superior rectal artery were performed Figure  by the iOn colonoscopic biopsy of the rectosigmoid lesion, the lesion was histopathologically proven to be an adenocarcinoma colon. The treating team also decided to go ahead with an ultrasound-guided biopsy of the liver lesion after taking high-risk consent from the patient, in view of atypical imaging features, in order to obtain a definitive diagnosis. The patient tolerated the procedure well. However, the histopathological diagnosis was surprisingly suggestive of a cavernous hemangioma of the liver.The patient was treated by transabdominal resection of the colonic carcinoma, followed by adjuvant chemotherapy and radiotherapy. The patient tolerated the treatment well. On follow-up after one month after discharge from the hospital, the patient did not have any new complaints, and the blood hemoglobin level improved to a value of 10.9 g/dL.Large vascular spaces with thin intervening septa were observed in hepatic hemangiomas that had rapid arterial phase enhancement, as opposed to smaller vascular components and a significant amount of fibrous interstitial space in hemangiomas that had delayed enhancement. It is likely that puddling, poor blood flow, and partial thrombosis are the causes of prolonged retention of contrast in vast intravascular spaces, which leads to delayed contrast enhancement in hepatic hemangiomas .Fibrosis of hepatic hemangioma often begins in the lesion's center and spreads peripherally to varying degrees . HoweverA few authors have described atypical hepatic hemangiomas with a centrifugal or inside-out pattern of enhancement . HoweverMost hepatic hemangiomas have typical radiological features that are extremely reliable and well-studied. However, intra-tumoral anatomical and physiological variations can result in atypical imaging features. Confusing clinical histories and co-existing diseases can make imaging diagnoses even more challenging. Histopathological confirmation may be necessary in such rare scenarios to draw further conclusions.


Anterior Thoracic Myelomeningocele is a very rare condition. The diagnosis may be made before or after birth. Association with loss of physiologic curvature of the spine is common. We report the case of an 8-year-old boy with congenital dorsal sinistro-convex scoliosis, which was considered nonspecific and improved slightly after physical therapy. The onset of urinary and fecal incontinence at the age of 8 years led the parents to consult a urologist. MRI of the spinal cord revealed an anterior thoracic heterogeneous cystic lesion extending from the third to the eighth dorsal vertebrae. The heterogeneous cystic mass contained a solid spinal cord-like structure on all sequences. No abnormal enlargement, no torsion of the cauda equina or cerebellar amygdala. No malformation of the posterior fossa. CT scan showed aplasia of the right pedicle of the third thoracic vertebrae (T3) with thoracic scoliosis. The associated anterior thoracic myelomeningocele was the final diagnosis that motivated the transfer of the patient to a specialized neurosurgical center. Spinal dysraphism (SD) is a general term for congenital anomalies of the midline of the neural tube. The midline closure of bone, neural, or other mesenchymal tissues is defective ,2. TheseMost SD conditions are diagnosed during pregnancy or shortly after birth Approximately 90% of all reported myelomeningoceles are located in the lumbosacral region, followed by the thoracic and cervical spine The aim of this paper is to report a rare case of anterior thoracic myelomeningocele and to highlight the importance of medical imaging in the diagnosis of this condition.An 8-year-old boy with a long history of scoliosis and regular physiotherapy at a rehabilitation center was admitted to the urology unit of a university teaching hospital in Bukavu, eastern Democratic Republic of Congo (DRC), for new-onset urinary and fecal incontinence.The patient had undergone several spinal radiographs over the past 5 years, which showed only isolated scoliosis. The parents did not recall any possible exposure to mining or other ionizing sources before or during pregnancy. A spinal cord MRI was performed for further etiologic investigation of these new neurologic symptoms. A 0.45 Tesla machine from Hitachi  helped to perform multiplanar sequences such as T1 weighted image-Fast spin echo (T1WI FSE), T2 weighted image-Fast spin echo (T2WI FSE), T2 Short tau inversion recovery (STIR) and T1 fat sat postgadolinium.An anterior right cystic mass (approximately 60.7 × 48.4 × 42.1 mm or 61.84 mL) was visualized at the level of the third (T3) to eighth (T8) thoracic vertebrae. An isointense filiform structure could be seen within the mass, which proved to be the spinal cord. The mass showed an inhomogeneous signal on both T1- and T2-weighted images, globally hypointense on T1 , A and BFor completeness, 2 sequences centered at the craniocervical junction were subsequently performed to look for possible associated posterior fossa malformations. This study did not reveal any additional lesions , A and BOverall, the diagnosis of isolated anterior thoracic myelomeningocele with levoscoliosis was maintained. There was no obvious pulmonary complication at the time of diagnosis. The patient was referred to a tertiary neurosurgical center for multidisciplinary management. Of note, the findings of the preoperative chest CT scan  performed 2 months later revealed the T3 right pedicle defect , A–D.FigMyelomeningocele is the commonest anatomic subtype of SD, the most recurrent central nervous system malformation, but still compatible with life ,2,7. In Low-income countries do not have sufficient and adequate health facilities to screen and diagnose such congenital malformations. Ahuka et al. For anterior cervico-thoracic myelomeningocele, one should distinguish between true and false anterior cases, according to the localization of the defect. False cases, mostly associated with neuroforamen enlargement due to congenital mesenchymal pathology, are well described (more than 100 cases reported) Awareness and early detection of such malformations may prevent neurologic complications in a setting where surgical intervention is possible. In this case report, the presence of a mediastinal mass remained undetected for several years, as all radiographic studies showed only isolated scoliosis, until the onset of neurologic symptoms. This may be explained by either the small size of the mediastinal mass at the time of previous visits, the lack of training in imaging diagnosis, or most likely the under-equipped health centers.Prenatal screening also includes alpha-fetoprotein dosage and fetal ultrasound This case report highlights its place in the diagnosis of SDs. Apart from the final diagnosis, imaging modalities with 3D postprocessing allow to establish anatomical relationships in the preoperative evaluation It also helped us to rule out associated neurological malformations such as Chiari malformation and hydrocephalus, since the literature reports that spina bifida and its variants are frequently associated with these malformations, in about 85% of cases Anterior thoracic myelomeningocele is a very rare central nervous system malformation, particularly in sub-Saharan Africa. Healthcare professionals, especially those working in resource-limited settings, should be aware of this condition, as its early detection is crucial for appropriate management and may reduce the associated morbidity. Neuroimaging, especially MRI of the spinal cord, should be considered as a key tool in the diagnosis of this spinal malformation, especially in the closed and asymptomatic subtypes.The father of the child has given written informed consent for the publication of this report and images.


Benign fibro-osseous lesions with cystic degenerations have been scarcely reported in craniofacial bones and its unusual characteristics pose a diagnostic challenge. Here, we report a case of craniofacial fibrous dysplasia presenting a large cystic degeneration. A 55-year-old woman had a history of pain, slight asymmetry on the zygomatic region and ocular pressure. Computed tomography revealed on the right side, multiple craniofacial bones showing a ground glass aspect, associated with an extensive hypodense, unilocular, well circumscribed lesion in the maxilla, and smaller lesions in the sphenoid bone. After a surgical procedure performed in another service, there was a complete improvement in symptoms, and after 1 year, the patient remains stable, with no changes. In the literature review, thirty-three reported cases of the same association in the craniofacial region were found. The main symptoms were sudden increase in the lesion and pain, and the indication of intervention in cystic lesions was only indicated in symptomatic cases or functional deficit. The knowledge of the possibility of the association of benign fibro-osseous lesions and cystic degenerations in craniofacial bones is essential to perform a correct diagnosis and treatment for these patients, consequently avoiding unnecessary procedures. Key words:Craniofacial fibrous dysplasia, Fibrous dysplasia, Benign fibro-osseous lesions, Cystic degeneration. Benign fibro-osseous lesions constitute a group of diseases, in which healthy bone is replaced by fibrous tissue with foci of bone neoformation. The 3 most common lesions affecting craniofacial bones are fibrous dysplasia (FD), cemento-osseous dysplasia (COD), and ossifying fibroma (OF). Although, such diseases differ clinically and radiographically , they present similar histopathological features .FD is caused by a post-zygotic mutation in the GNAS 1 gene that is linked to changes in osteoprogenitor cells, leading to abnormal bone formation. Monostotic FD affects only one bone and polyostotic several bones and may be associated with syndromes, such as McCune-Albright. The term craniofacial FD is used for FD involving multiple skull bones . There aA 55-year-old woman was referred to the Stomatology Department for evaluation of an injury on the right maxilla. During anamnesis, the patient reported to be treated and followed for 5 years by either otorhinolaryngologist and maxillofacial surgeon. She also had a history of pain and eye pressure, on the right side of the face, that ameliorated after a surgical procedure. In fact, the surgeon informed us that he found an empty cavity during the exploratory surgery. Due to doubts in relation to diagnosis, he referred the patient for our evaluation. On clinical examination, it was observed a slight swelling on the right side of the face. Computed tomography (CT) performed 6 years ago showed an extensive and expansive lesion with a mixed aspect mainly “ground glass”. The lesion involved the maxilla, greater sphenoid wing, temporal, frontal, pterygoid process, floor and lateral wall of the orbit, all on in the right side. It was also observed an extensive hypodense, unilocular, well circumscribed lesion in the maxilla. Such lesion caused a remodeling of the lateral and inferior walls of the ipsilateral maxillary sinus, decreasing its dimensions. Moreover, a cortical thickening of orbital floor and anterior wall of the maxillary sinus was observed. It is worth mentioning that similar lesions, but smaller, were also observed in the sphenoid bone Fig. . In the The association between craniofacial FD and cystic formations is uncommon, with few cases reported in the literature. The exact pathogenesis of this association is unknown. El Deeb  and WojnWe found a total of 33 cases of FD presenting with cystic formations  in craniofacial bones in the English literature , 1 cont.et al. (Regarding treatment, it is suggested that symptomatic patients and/or presenting functional deficit, the cystic lesions should be promptly treated, otherwise the patient should be only followed up. Hong et al.  reportedIn conclusion, the present study described a rare case of craniofacial fibrous dysplasia with cystic degeneration. To our knowledge, this is the third case of such association in the maxilla. The knowledge of the association of benign fibro-osseous lesions and cystic degenerations in craniofacial bones is essential, considering that in most of the cases the treatment is expectant or performing only cystic decompression, and large resection must be avoided.