Isolated facial nerve palsy resulting from temporal bone metastasis is rare and has been sparsely reported in the literature to be the initial presentation of cancer. The most commonly reported sites of origin of such metastases include the breast, lung, kidney, gastrointestinal tract, larynx, prostate, and thyroid, to name a few. Here, we discuss a patient initially presenting with isolated lower motor neuron facial nerve palsy. The diagnosis was revised to that of breast cancer with metastasis to the temporal bone resulting in facial nerve paralysis following the subsequent clinical presentation. Isolated facial nerve palsy, clinically represented as Bell’s palsy and commonly highlighted as an acute peripheral facial palsy of unknown clinical etiology, accounts for 72% of facial palsies . Bell’s While isolated occurrences are quite common, multiple recurrent episodes of unilateral facial paralysis are rare. Meanwhile, omission of congenital abnormalities, systemic inflammation or infection, or trauma as a prime cause of facial palsy should raise a suspicion of metastatic tumors to the temporal bone, as well as primary malignancies. Temporal bone metastasis is a rare clinical event and imposes a diagnostic challenge due to its asymptomatic feature in most cases . The mouA 54-year-old Malay lady presented to the emergency department with the inability to close her left eye, with an associated hemifacial droop of one week's duration. Examination findings were keeping in with idiopathic facial nerve palsy with lower motor neuron lesion. The rest of her general and neurological examination was unremarkable. A presumptive diagnosis of Bell’s palsy was made and she was discharged on a course of oral prednisone.She presented three months later with generalized weakness, bony pain, and unexplained weight loss and breast mass. She was hemodynamically stable on presentation. Facial examination revealed persistence of left facial nerve paralysis. Neurological examination was otherwise unremarkable. She was noted to have a 5 cm fungating right breast mass with an ulcerated surface, which was a new finding.Histopathology of the breast lesion confirmed invasive ductal carcinoma, with positive immunostaining for estrogen and progesterone receptors, as well as negativity for Cerb-B2. Further workup revealed extensive systemic metastases including deposits at bilateral temporal petrous apices consistent with bony metastasis Figure . The patThe prevalent clinical cause of unilateral facial paralysis is Bell’s palsy, Ramsay Hunt syndrome, Lyme disease, and complications of chronic otitis . HoweverThe most common symptoms reported in patients with temporal bone cancer are hearing loss, facial paralysis, peri-auricular swelling, otorrhea, otalgia, vertigo, aural mass, and tinnitus ,13. HoweIn a study conducted by Maddox, the incidence of facial paralysis in the presence of temporal bone metastases has been reported to be 34%, compared to 50% and 14.9% in the studies of Schuknecht et al. and Gloria-Cruz et al., respectively -13. ThusFurthermore, inflammatory diseases, systemic infections, or some benign tumors should be considered in the differential diagnoses. A case reported by Suryanarayanan et al. displayed a secondary deposit in the temporal bone mimicking facial nerve schwannoma and stressed the importance of having a suspicion for metastatic tumors in a patient with a previous history of malignancy -16.In conclusion, metastatic tumors to the temporal bone are rare, highlighting the need for the physician to consider them in the differential diagnoses of patients presenting with facial nerve palsy. The treatment of metastatic temporal bone lesions may involve systemic chemotherapy with local radiotherapy to the affected temporal region. In the present case, the patient was treated with letrozole, and reevaluation after six weeks revealed improvement in energy levels and pain scores, along with an interval improvement of the facial palsy.


The current study evaluates a rare case of parietal bone osteoid osteoma in pediatrics and review the differential diagnosis of button sequestrum sign in the literature. A 12‐year‐old girl expressed localized pain in the right parietal bone. MRI represented enhancing nodule with button sequestrum sign appearance. Due to the rarity of condition in children, osteoid osteoma should be considered as a differential diagnosis of button sequestrum sign in magnetic resonance imaging in children. She estimated the pain as level seven out of 10, worsening by combing and relatively constant with no radiation. No history of previous head trauma was identified. Physical examination merely revealed moderate tenderness on the right parietal bone with no visible or palpable lesion or lump on her head or face. As well, no other significant pathologic finding including visual, sensory, or neurologic disturbances was identified. She had an appendectomy 1 month before the current presentation due to acute appendicitis, which was consequently followed by a generalized seizure the next day after surgery. Her Electroencephalography (EEG) depicted considerable abnormalities that necessitated treatment with Carbamazepine 200 mg twice a day (B.I.D) for the following next 9 months. Of note, no relevant family history of similar headache, seizure, or epilepsy was identified, and the seizure did not occur again. The lesion was intended to be removed entirely by performing a craniotomy. Bone cement was then used to accomplish a cranioplasty; a surgical intervention used to repair cranial defects for both cosmetic and functional purposes. She recovered without incident and was pain‐free. The histology findings supported the osteoid osteoma diagnosis.2The Brain CT Scan and MRI , demonstrated an enhancing nodule, 7 mm in diameter located in the right parietal bone Figures , 2, 3, 43Osteoid osteoma is a benign bone‐forming tumor in children and adolescents, often presenting as a small round radiolucent nidus with a sclerotic margin in radiographic images. It is the cause of approximately 12% of benign bone tumors, usually occurring in the 2nd decade of life in the lower extremities, particularly in the proximal femur.4Due to the rarity of this condition in children, osteoid osteoma should be considered as a differential diagnosis of button sequestrum sign in magnetic resonance imaging. (1).Ali Mehri involved in writing the manuscript and submitting. Farrokh SeilanianToosi involved in describing the MRI and CT scan Images. Fariborz Samini involved in performing the surgery. Javad Akhondian and Narges Hashemi involved in patient care and manuscript proofing. Alireza Khooei involved in describing the histopathological samples.The authors declare that they have no conflict of interest.Written informed consent was obtained from the patient's parent to publish this report in accordance with the journal's patient consent policy.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.


The odontogenic keratocyst (OKC) is among the most aggressive odontogenic cysts because of its high recurrent rate. This study’s objective was to describe a 7-year radiological and clinical follow-up of an OKC with two recurrences and a combination of surgical treatments. The cyst contents were drained at the biopsy to allow decompression, and then marsupialization (Partsch I) was carried out with good results. In the following two years, the endodontic and radiological evaluation of the neighboring teeth indicated endodontic avoidance. The remaining OKC enucleation (Partsch II) with chemical curettage and suture was performed two years after the first presentation, and the multifocal recurrences developed were also treated by enucleation and chemical curettage. At the last radiological follow-up, no recurrence was evident. Based on our experience, we concluded that patients diagnosed with a OKC should be radiologically evaluated at least once a year for at least 7 years, the endodontic diagnosis and treatment of neighboring teeth should be performed as early as possible, and the combination of surgical treatment and the long follow-up period is challenging.  The odontogenic keratocyst (OKC) is particularly interesting because of its aggressive nature, very high recurrence rate, and disputed treatment modalities. The term “odontogenic keratocyst” was first reported by Phillipsen  as a sepThe OKC usually appears in the second and third decades of life with male predilection and grows within the medullary bone without significant cortical modifications. It can be located anywhere in the jaw, rarely in the maxillary sinus, but it is commonly seen in the posterior part of the mandible. In many cases, patients are free from symptoms until the cyst reaches a large size and involves the entire ascendant ramus and the mandibular body ,5. Radiographically, it presents as a unilocular or a multilocular radiolucency and may be confused with ameloblastoma or dentigerous cyst . To estaThe choice of treatment is based on the size of the cyst, the recurrence status risk, and the radiographic evidence of cortical perforation. Therapeutic interventions of OKCs include marsupialization, decompression, enucleation, enucleation with chemical curettage (Carnoy’s solution), enucleation with liquid nitrogen (cryodestruction), peripheral ostectomy, and jaw resection ,8. 3) dissolved in 6 mL of pure alcohol, 3 mL of chloroform, and 1 mL of glacial acetic acid. According to certain studies, Carnoy’s solution contributed to favorable results in terms of morbidity and lesion recurrence rate [Enucleation with chemical curettage using Carnoy’s solution (CS) application has been proposed as a therapeutic strategy with a lower recurrence rate than enucleation alone. Described in 1933, Carnoy’s solution (CS) contained 1 g of ferric chloride  to reduce the cyst’s volume, and than a radical surgery enucleation and chemical curettage (Partsch II technique).After the patient was informed of the diagnosis, the prognosis with and without treatments, the therapeutic steps, benefits, procedure risks, and probable adverse reactions, informed consent was signed.The extraction of tooth #48 under local anesthesia was performed, and the aspirate of cystic content carried out through the alveolus had a creamy appearance. The biopsy was harvested after the margins of the cortical bone were removed. The cavity was irrigated through the empty socket with abundant normal saline and a 5% povidone-iodine solution. For decompression, a plastic drain tube was introduced into the cystic cavity and sutured at the socket margins, which permits the irrigation of the cystic cavity.The histopathological diagnosis revealed a cystic structure lined on the inner part via squamous epithelium with focal parakeratinization, the basal layer with palisade-arranged hyperchromic nuclei, and the rectilinear relief. Inside the cyst, we noticed orthokeratotic keratin lamellae. Subepithelial connective tissue had fibrosis and edema with a tendency to form cleavage spaces without signs of inflammation a,b.A general clinical examination including Dermatological Department visit, blood tests, and chest and skull X-rays ruled out in the Gorlin–Goltz syndrome (GGS). To establish a diagnosis of GGS, at least two major and one minor or one major and three minor clinical and radiological criteria must be present, and none were present in our patient. For one year, the patient irrigated the cystic cavity with normal saline every day. Every month, as we decided at the initial management, we verified the permeability and, if necessary, resutured the tube at the neighboring teeth . At each follow-up, the first tube was replaced with a shorter one. In the same time, an endodontic evaluation with positive vitality response was performed twice at the dentist, and the decision to avoid endodontic treatment was chosen.A year after the decompression, the patient returned a–c for tPatient consent was taken, and local anesthesia and extended incision from the second molar up to the anterior part of the ramus were performed. The bony margins forming the opening for the cystic cavity, along with its attached overlying mucosa, were removed. After the cavity irrigation, the cystic lining  was folded, and the edges were sutured with a mucosal flap using a 6.0 Vicryl. The 5% povidone-iodine-soaked gauze was packed in the cystic cavity. For one year, the gauze was replaced every two weeks, taking care to ensure that it did not come into the mouth. At each follow-up appointment, we gradually reduced the size of the gauze. In our case, no antibioterapy was prescribed; oral hygiene was enhanced with 2% chlorhexidine for the first four weeks after surgery, and the diet was normal.The decision to forego endodontic treatment for molar #47 was made by the endodontic specialist based on CBCT image  analysisA year from the marsupialization (and two years after the first presentation), the radiological aspect showed complete healing in the posterior part of a former lesion at the level of the ascendent ramus. However, it remained the same in the anterior part, at the level of the mandibular body, under the first and second mandibular molars roots a,b. The The surgical treatment proposed for the remaining 2 cm OKC at the apical level of teeth #46 and #47 was enucleation and chemical curettage (Partsch II technique) associated with the second molar extraction c. ChemicThe endodontist decided against performing endodontic therapy on molar #46 based on radiological images and a positive vitality test.The histological assessment confirmed parakeratinized squamous epithelium with a prominent palisade-arranged basal layer; the finding confirmed the presence of an OKC.At the clinical and radiological follow-up, nine months after the enucleation and chemical curettage, two unilocular radiolucencies (one at the ascendent ramus and another under the first molar) were visible on the orthopantomography . The patient returned very late because of personal motivations in the COVID pan-demic period (after another 15 months from the diagnosis of the recurrence) and the radi-ological images demonstrated two separated radiolucent unilocular lesions a,b at boThe multifocal recurrent surgical treatment was decided, and informed consent was obtained. Due to the failure of molar #46 endodontic treatments, the decision to extract it was taken, and an apicectomy was not taken into account because of the high aggressivity of this type of cyst to prevent a recurrence. The surgery was completed under local anesthesia. It consisted of enucleation of both cystic lesions and chemical curettage in the same procedure associated with tooth #46 extraction. Biopsy revealed parakeratinized odontogenic keratocyst in both lesions.One year after the enucleation of multifocal OKC recurrence, at the clinical and radiological control, the aspect of the bone demonstrated a process of normal healing .A year later, at the subsequent follow-up, a new OKC recurrence (the second one) appeared at the radiologic control , situateAt the last follow-up, two years after enucleation, orthopantomography a, confirThe patient did not develop any recurrence despite the presence of focal parakeratinized area and cleavage spaces histologically. Seven years after the first presentation, the patient had no facial asymmetry and no IAN paresthesia and was proposed for dental implants at the right mandibular ramus. Despite extensive research, there is no consensus regarding the aggressive behavior of the OKC and the cause of recurrences. The recurrences might be explained by different causes, such as the incomplete removal of the highly active basal layer of the epithelial cyst lining, the growth of a small intramedullary satellite cyst left behind by conservative treatment, and the development of lesions localized in the adjacent region of the jaws ,21. For To some authors, marsupialization has a high success rate, and the OKC treatment protocol based on decompression offers a conservative and effective option with low morbidity . We chosOKC has been subject to a lot of debates and controversies because of its diverse nature and high recurrence rates .Blanas et al., in a systematic review, found a recurrence rate of approximately 17% to 56% when the OKC was treated by simple enucleation, which explains the addition of Carnoy’s in the cystic cavity for 3 min after enucleation, thus reducing the recurrence rate to 1.6%, i.e., a rate comparable to the resection one but without associated morbidity . According to Dashow et al. , the simThe young patient had a sizeable multiloculated cyst and a period of the volume-reducing lesion by decompression and marsupialization of two years with well-demonstrated advantages. Marsupialization permits a more gradual decompression of the cyst cavity, which, in our perspective, after the first year, remains too large for ambulatory surgery.Despite a rigorous enucleation treatment and chemical curettage, two recurrences appeared in the same place of the mandibular body. The recurrences could appear as a result of the posterior location of the lesion with a problematic approach, extensive and multilocular lesions that could not be resected easily, the presence of daughter cysts, and because the tumor could have a cleavage space between the epithelium and connective tissue that made the cyst to be fragile and friable  ,26. DespSome histopathological aspects are risk factors for recurrence, such as parakeratinization, budding, daughter cysts , and theAfter an extended (7-year) follow-up, with two recurrences, a multifocal one and a new OKC recurrence, the final result was acceptable, with an appropriate intraosseous bone structure at surgical sites and no aesthetic compromise. The patient is still in the follow-up period.In addition, our case exemplified the value of multidisciplinary patient management, including dermatology, endodontics, radiology, maxillofacial surgery, and pathology.Based on our case, both conservative procedures worked synergistically and, instead of a two-year decompression, we opted to undergo the marsupialization and to avoid a extensive surgery. The decompression and marsupialization decreased the lesion volume and allowed for a second stage of surgery to reduce morbidity and improve the patient’s postoperative quality of life. Histopathological aspects such as parakeratinization, cleavage space, and daughter cysts are essential in diagnosis and prognosis risk evaluation. The endodontic diagnosis based on radiological images must be properly performed and as soon as possible.Conservative surgical therapy to limit morbidity, the endodontic treatment of adjacent teeth to minimize the risk of recurrence, and the long-term follow-ups with periodic radiographic examinations are essential.Protocols for the early diagnosis of OKCs should be established to prevent radical treatment with a large loss of substance.


Gallstone ileus is one of the rare and insidious causes of small bowel obstruction, which should always be kept in mind by clinicians, especially when encountering older people with a history of gallstones disease. The high mortality and morbidity rate associated with the condition can be mostly attributed to delayed or misdiagnosis. Imaging modalities, particularly CT scans, play an important role in correct and timely diagnosis. We present the case of a 65-year-old man with a two-year history of colicky biliary pain, who had severe abdominal pain with obstructive symptoms for five days before admission. The diagnosis of gallstone ileus is made using CT scan findings. Enterolithotomy alone was performed three days later. A second, smaller migrated gallstone was also found, which excreted spontaneously. After 20 days, the patient achieved full recovery and was discharged. Gallstone ileus is defined as small bowel mechanical obstruction induced by an impacted ectopic gallstone. Terminal ileum and ileocecal valve are the most common sites of gallstone impaction. Generally, gallstones of less than 2-2.5 cm have a high probability of spontaneous transit . GallstoInflammatory changes, pressure erosion, and ischemia of the gallbladder wall could lead to biliary-enteric fistula in patients with a long-standing history of gallstone disease. The most common fistula is cholecystoduodenal fistula 32.5-96.5%) followed by stomach, small bowel, and colon involvement, respectively [2.5-96.5%Strong clinical suspicion is necessary for the early diagnosis of this condition. Nonspecific and intermittent symptoms, misdiagnosis, or delayed diagnosis are factors that cause high morbidity 20-57%) and mortality (7-18%) rates in this condition compared to other causes of small bowel obstruction . The med7% and moRadiological imaging is the cornerstone of the early diagnosis of gallstone ileus. The Rigler’s triad including pneumobilia, small bowel obstruction, and ectopic gallstone represent the pathognomic findings . The treA 65-year-old male presented to the emergency department with a five-day history of constipation, nausea, mild abdominal pain, and two-year history of multiple episodes of colicky biliary pain controlled by painkillers and opium. He had experienced acute severe right upper quadrant pain seven days before admission. The pain had not responded immediately to painkillers; it had subsequently improved but he had developed intestinal subocclusion symptoms. He had a history of diabetes, hypertension, and opium addiction.The patient's vital signs were stable; a non-tender abdomen and mild generalized distention were evident on physical examination. Lab data revealed leukocytosis (WBC=13000) and elevated creatinine level (Cr=2.1).Upright abdominal X-ray showed mildly dilated small bowel loops Figure . An abdoDue to poor patient compliance and surgery refusal, as well as partial obstructive symptoms, conservative management was chosen at first, inevitably. But his symptoms and signs worsened during the first three days of admission, including bilious vomiting, obstipation, increasing abdominal pain, distention, and leukocytosis in favor of complete obstruction. Finally, the decision was made to perform an urgent laparotomy after getting informed consent.The intraoperative examination showed dilated duodenal and jeujonal non-ischemic loops, impacted jejunal stone at the transitional point, and adhesions over the gallbladder and duodenal wall. An enterotomy was done using a longitudinal incision. An oval-shaped stone measuring 28 x 25 mm and biliary material were extracted, and then the double layer closure of enterotomy was performed. The remainder of the gastrointestinal tract was assessed carefully to find other ectopic stones, but none were found. The area of the cholecystoduodenal fistula was not manipulated due to the patient's condition and potential risk of injury due to extensive adhesions.On the fifth postoperative day, after beginning an oral diet, mild colicky abdominal pain and bilious vomiting were revealed. A second abdominopelvic CT scan with oral contrast was performed, which demonstrated another duodenal ectopic non-obstructing gallbladder stone measuring 17 x 15 mm with a typical Mercedes-Benz sign in favor of recently migrated gall stone Figure . Since tDuring the next seven days, the patient did not experience any obstructive symptoms and signs and tolerated the semi-liquid diet well, which was suggestive of spontaneous excretion of the stone, subsequently confirmed by abdominopelvic CT. The patient experienced a full recovery and was discharged 20 days after the admission.Gallstone ileus is defined as small bowel mechanical obstruction induced by an impacted ectopic gallstone . NowadayThe terminal ileum and the ileocecal valve are the most common anatomical sites for gallstone impaction because of their small caliber and less active movements. Ileum (60.5% of cases), jejunum (16.1%), stomach (14.2%), colon (4.1%), and duodenum (3.5%) can also get involved. It can also pass spontaneously in 1.3% of cases -12. In oInflammatory changes, pressure erosion, and ischemia of the gallbladder wall could lead to biliary-enteric fistula in patients with a long-standing history of gallstone disease. Cholecystoduodenal fistula is the most common manifestation (32.5-.96.5%) [The classic presentation of gallstone ileus entails a “tumbling obstruction” in an elderly woman, indicating periodic subacute obstruction. Episodic and transient gallstone impaction results in nonspecific, generalized abdominal pain and vomiting. In our patient, a smaller migrated gallstone was also found, which, under our careful observation, resembled a “tumbling obstruction” and spontaneously resolved. The minimum size of the ectopic stone that leads to the development of obstruction is 2.5 cm in diameter. In the study by Clavien et al., the size of obstructing gallstone ranged from 2 to 5 cm . MultiplNonspecific and intermittent signs and symptoms, the advanced age of the patients and their comorbidities, and misdiagnosis or delayed diagnosis are factors that cause high morbidity 20-57%) and mortality (7-18%) rates in this condition compared to other causes of small bowel obstruction . The med7% and moRadiological imaging is the cornerstone for the early diagnosis of gallstone ileus. The classic Rigler’s triad illustrates the radiographic features of gallstone ileus: small bowel obstruction, pneumobilia, and an ectopic gallstone that migrates in serial films through the GI tract; the presence of two of them is enough for gallstone ileus diagnosis. Only 10% of gallstones are radiopaque, and this along with the presence of fecal material renders plain abdominal radiography incapable of detecting gallstones and reduces the chance of an early diagnosis . UltrasoLassandro et al. found that the Rigler’s triad is observed in 14.8% of cases via plain abdominal radiography, 11.11% of cases on ultrasound examination, and 77.78% on abdominal CT scan .The definitive treatment involves the removal of the impacted stone and relieving intestinal obstruction subsequently. However, preoperative conditions such as the age of the patients, comorbidity, diagnostic delay, and the need for urgent surgery have a significant impact on choosing the best approach, which should be decided by a careful evaluation of the risk-to-benefit ratio .Three surgical methods are usually employed for gallstone ileus treatment: enterolithotomy alone, enterolithotomy with cholecystectomy and simultaneous fistula closure (one-stage procedure), enterolithotomy and delayed cholecystectomy, typically four to six weeks later (two-stage procedure) .Enterolithotomy alone is the most commonly used method. An enterotomy involves extracting the stone through a longitudinal incision. The rest of the bowel is then evaluated for other migrated stones. Eventually, the incision site is sutured in a transverse fashion ,15,16.The one-stage procedure is suitable for low-risk patients without comorbidity, those who are relatively younger, and those with less severe disease, adequate nutrition, and stable hemodynamics, owing to the longer duration of surgery, postoperative complications, and hospitalization ,15. The The two-stage method carries the risk of cholecystitis, recurrence, and gallbladder malignancy due to the continuation of the bilioenteric fistula This procedure is recommended in high-risk patients who suffer from persistent symptoms secondary to residual gallstones or biliary fistula. The risk of gallstone ileus recurrence with a two-stage approach is 5-9% and only 10% of them require another surgery . Chou etConservative management is also useful in some cases. Indeed, when gallstones are only up to 2.5 cm in size, they can be discharged spontaneously. However, conservative treatment cannot always be recommended, because it leads to a positive result only in cases of partial intestinal obstruction ,20.As mentioned before, our patient underwent enterolithotomy alone, and the main reasons for preferring this approach were severe inflammatory changes and many adhesions around the gallbladder, which increased the risk of iatrogenic injuries. Also, the patient's advanced age, presence of comorbidities, and poor compliance played a role in this decision. In the case of the second ectopic stone, which was detected in the follow-up CT scan, we preferred conservative treatment over re-enterolithotomy due to the stone size and absence of clear obstruction signs, and it passed through the GI tract, fortunately. CT scan had a significant role in our decision-making, as it enabled us to estimate the size and location of the stone and choose the non-surgical option for treatment.Nowadays, the advanced manifestations of gallstone disease have considerably declined due to early diagnosis and treatment. Gallstone ileus should be considered when encountering a case of small bowel obstruction, especially in elderly individuals with a positive history of cholelithiasis. The best surgical option for gallstone ileus is still a subject of controversy. Due to the low risk of complications, enterolithotomy alone is the most popular surgical procedure. In our case, the delay in surgery due to patient refusal, his general clinical condition, the patient's advanced age, as well as extensive adhesion and inflammatory changes of the gallbladder and the duodenal wall prompted us to opt for enterolithotomy alone. CT scan is a readily available, quick, and high-resolution diagnostic modality, which, due to its high diagnostic accuracy, plays a decisive role both in the diagnosis and in determining the treatment plans. In our case, the findings of the CT scan enabled us to avoid performing re-surgery.


Lymphedema is originated from the reduced lymphatic flow, causing a volumetric increase in the affected region and is physically and emotionally uncomfortable. Surgical intervention is considered the best treatment option as it brings both functional and aesthetic benefits. This is a report from the case of a previously healthy 45-year-old man who developed idiopathic penoscrotal giant lymphedema. A surgical approach was proposed. There was resection of scrotal lymphedema followed by a classic postectomy, suprapubic advancement flaps, and use of a partial skin graft from the right thigh. The results of the therapeutic approach were aesthetically and functionally satisfactory. The genital form is the 3rd most frequent.Malignancy and filariasis are the main causes. Other etiologies are lymphogranuloma, mycobacterium spp. and cellulitis. Benign tissue lymphoproliferation causes massive lymphoedema, mainly associated with obesity. The disease is physically and emotionally uncomfortable, being disabling because of pain, difficulty in hygiene, mobility, social and sexual impairment.Conservative treatment is decompressive and usually the first line. However, the results are temporary and recurrence is frequent. Therefore, surgical intervention is considered the best treatment option. The technical possibilities aim to increase drainage capacity or remove affected areas in cases of fibrosis or fatty infiltration and consists in excision of the affected tissue with primary closure, or reconstruction with grafts and flaps. This approach brings functional and aesthetic benefits and improving quality of life.2AEK, male, 49 years, was referred to the urology service of a Tertiary Hospital, complaining of a progressive and voluminous penoscrotal increase for 6 years. In 2012, he presented a small lesion in the scrotum. It evolved with subsequent recurrent drainage (2014) and fluctuating edema. He denied comorbidities or previous surgeries and was a smoker. Physical examination revealed giant penoscrotal lymphedema . ResonanThere was a functional repercussion, with urinary and hygiene difficulties, low sexual performance, psychological impact, due to impaired aesthetics, and no response to clinical treatment.The whole area of large lymphedema proportions has been surgically removed from the inguinal region, isolating the spermatic funiculus and testicles, and dissecting the penis in anterograde way, preserving dorsal vascularization . For theThe procedure was uneventful. The patient was discharged from the hospital on the sixth postoperative day. During follow-up, the bladder catheter and stitches were removed 8 and 23 days after surgery, respectively. On the 60th postoperative day , the rec3There is no cure for lymphedema, but the identification and adequate choice of a technique allow its management and better quality of life, with a long time free from remission. Usually, the first line is conservative decompression treatment. At first, it may appear to have good results, but are usually temporary. Surgery is more effective, especially in relapsing, refractory or giant lymphedemas, as described.Delpech first described the reconstruction with thigh flaps. Possibilities are closure by edges approximation, grafting in the penis and/or scrotum, flaps rotation for the scrotum, or even skin from the excised tissue itself as a graft - not generally used, because of the thickening. Excision is the most described surgical procedureThe systematic review by Aulia et al. identified that all studies demonstrated quality of life improvement after intervention, with better subjective perspective, mobility, urinary function, sexual activity and scrotal reduction. The reported case had all of these aspects upgraded until now.Scaglioni and Uyulmaz performed a lymphedema excision (debulking) followed by a partial skin graft (upper thigh) for the penis. There were no recurrences or complications within six months of follow-up. Similarly, in this case, there has not been recurrence so far.Some early complications highlighted by Torio-Padron et al. were hematoma (4%) and dehiscence (2%). In these patients, preoperative decongestive physiotherapy and reduction surgery were used.In the described report, grafts and flaps were also used and, equally, there were no complications. This suggests there seems to be less identification of recurrences and complications when surgical techniques with grafts and flaps are used. However, epidemiological studies with larger samples are needed, in addition to specific cases, to establish such conclusions.4Giant penoscrotal lymphedema is uncommon but substantially detrimental to the patient's quality of life. Usually associated to filariasis, but can be present idiopathically. Bibliography shows that clinical measures are ineffective and uncomfortable, specifically in genital region.Therefore, surgical management with the removal of the affected tissue and genital reconstruction is the method of choice for the best recovery of the physical and emotional capacities of affected patients - especially because of aesthetic and functional recovery, as demonstrated in this case report, as well as a low rate of complications - possible when an adequate surgical technique is performed, by a prepared team, using grafts and flaps, postoperative care and outpatient follow-up.


Pulmonary actinomycosis is an uncommon kind of bacterial illness caused by actinomycetes, involving the chest wall in extraordinarily rare cases. Due to non-specific clinical signs and perplexing radiological characteristics, this kind of pulmonary actinomycosis is frequently misinterpreted as a malignant tumor or lung abscess.An 11-year-old child presented with a palpable lump on his left chest and periodic chest discomfort. An irregular soft-tissue mass in the left upper zone with bony destruction was first identified as a malignant small round cell tumor (MSRCT) known as an Askin tumor on post-contrast CT. However, pathological biopsy of the pulmonary lesion through the chest wall revealed actinomycosis.Pulmonary actinomycosis is an uncommon bacterial illness that has a variety of clinical manifestations, particularly in young patients. A chest lump with nearby “lace-like” rib bone destruction was the distinguishing characteristic of our case. For appropriate treatment and diagnosis, infection with actinomycosis should be considered when observing a similar chest lump. Pathological biopsy, as a valuable diagnostic tool, can help to distinguish between infectious diseases and thoracic tumors. The pathological manifestations of actinomycosis are characterized by inflammatory lesions that range from purulent to granuloma-like inflammatory processes, and second-generation sequencing of alveolar lavage fluid can help to confirm pathogens. Actinomycosis is a rare and chronic suppurative granulomatous infection mainly caused by Actinomyces israelii, first reported in 1857 . In termAn 11-year-old boy presented with occasional chest pain for 2 weeks. A palpable mass was initially found on his left chest. He denied having a cough, chest stuffiness, polypnea, or weight loss. Dental caries was observed. On physical examination, an 8 cm × 7 cm palpable mass was found on the left upper chest wall; there was tenderness of the mass but no swelling; and skin temperature was normal. Retraction to a slight extent and decreased breath sound were observed over the left lung field.Laboratory tests showed neutrophilic leukocytosis of 13.27 × 109/L, hemoglobin of 105 g/L, platelets of 524 × 109/L, C-reactive protein level of 44.59 mg/L, 70.9% lymphocytes, and 0.2% eosinophils, and a Mantoux skin test was negative. The patient underwent a detailed series of radiological examinations to visualize the entire landscape of the lesion. 3D-VR imaging and MinIP showed the mass and its internal structures .via the chest wall suggested purulent inflammation with scattered bacterial clusters, which morphologically suggested Actinomyces species  or tuberculosis (low fever and night sweats). Obvious weight loss, feeling unwell, and high fever could warn of the possible spread of pulmonary actinomycosis . LaboratPulmonary actinomycosis is associated with diverse radiology findings at different periods. In the early stage, irregular peripheral pulmonary nodules near the pleura with a hazy border similar to a halo sign may be found on CT. Some radiologists consider the halo sign to be associated with local invasion or pulmonary hemorrhage . In contObtaining stable positive pathological specimens is critical to confirm the diagnosis of pulmonary actinomycosis. Because conventional alveolar lavage fluid is easily exposed to air, the strictly anaerobic nature of actinomycetes makes it difficult to cultivate actinomycete clusters. The pathological biopsy is the gold standard for identification of pulmonary actinomycosis without typical clinical characteristics. Bronchoscopy biopsy or CT-guided percutaneous aspiration pathology biopsy is the most commonly used test and has high efficiency . Sulfur β-lactam antibiotics, penicillin G, and amoxicillin. A long course of parenteral penicillin in large doses is a recognized recommendation in the field of therapy for actinomycosis, and ampicillin and ceftriaxone can be used as alternatives (Although there is an extensive literature on actinomycosis therapy, most of the information is obtained from clinical case reports, and few publications focus on pediatric actinomycosis therapy. Antibacterial drugs for actinomycosis were first applied in 1938, in a case in which sulfonamides successfully cured an 11-year-old boy of his abdominal actinomycosis . So far,rnatives . The crirnatives . At presrnatives .Pediatric pulmonary actinomycosis is an extremely rare bacterial infection caused by a group of bacteria known as actinomycetes, usually misdiagnosed as pulmonary tuberculosis, lung abscess, or malignant tumor due to its lack of specific clinical symptoms or imaging features. Pulmonary actinomycosis is a granulomatous inflammation characterized by multiple abscesses, pus containing sulfurous granules, sinuses, and fistulas. Through production of lytic enzymes such as proteases, this form of actinomycosis is prone to spreading and infecting surrounding tissues, and hence appears to have a poorer prognosis than cervicofacial or abdominal actinomycosis. Suspended air bubbles, distribution across fissures, and lacy periosteal reaction may hold particular value in CT diagnosis of pulmonary actinomycosis, but go largely unacknowledged owing to the rarity of the disease.Bronchoscopy and CT-guided percutaneous aspiration pathology biopsy can help to differentiate the diagnosis from tuberculosis, intratubular tumors, and foreign bodies. Overall, accurate diagnosis in the early stages and appropriate treatment can lead to a good prognosis.


First branchial cleft cyst (FBCC) is a rare entity of congenital anomalies in the head and neck area. Dealing with FBCC is a clinical challenge as the condition is frequently forgotten in the differential diagnosis of lateral neck swelling. We report a rare case of unilateral type I FBCC in an 11-year-old boy who presented with a painless and slow-growing preauricular mass masquerading as a benign cystic lesion of the parotid. The lesion was completely removed via surgical excision. Histopathology report confirmed the findings of squamous epithelium‑lined cyst wall, which was a characteristic of a branchial cleft cyst. The combination of good clinical acumen, with the help of radiological correlation, along with a strong degree of suspicion for the condition, facilitates the diagnosis of this condition and hence proper management. Among the congenital neck masses in the pediatric population, branchial cleft anomalies are the second most common following thyroglossal duct cysts. Within the diagnosis of branchial anomalies, the second branchial cleft is the most affected, with a reported incidence of up to 95% of cases . The firWork has classified first branchial cleft cyst (FBCC) into two types . Type I,The diagnosis of FBCC, prior to surgery, is mostly based on clinical signs and symptoms. Due to their rarity and unfamiliar clinical features, even with the aid of radiological images, they can be easily misdiagnosed, leading to improper treatment. This case report highlights how an FBCC presenting as a benign parotid tumor. Besides, the challenges we faced in its diagnosis and surgery are also described.An 11-year-old boy with no known medical illness was referred to our otorhinolaryngology clinic. He presented with a painless swelling in the left neck since he was two years old. It was slowly increasing in size over the last two years, which prompted him to seek medical attention. There was no history of recurrent ear discharge. Upon physical examination, there was a well-defined mass in the parotid region of the left neck measuring 5x5cm  was performed for further evaluation, which demonstrated a well-defined lobulated intra-glandular cystic lesion within the left parotid gland measuring 4x4cm Figure . A fine With a provisional diagnosis of a benign cystic lesion of the parotid, he underwent a superficial parotidectomy. Intra-operatively, the cystic lesion was located within the superficial lobe of the left parotid gland superficial and lateral to the facial nerve. Difficulties were encountered as we noticed that the cyst can be traced superiorly to and adhered firmly to the cartilaginous part of the EAC. Believed to be a duplicate of EAC, this raised our suspicion of an FBCC. The lesion was carefully explored, and the cyst was removed together with a small section of the EAC cartilage. Remaining of the EAC cartilage was primarily closed, all the facial nerve branches were identified and preserved, and the lifted superficial lobe was replaced. The histopathology reported a cystic lesion measuring 50mm in diameter on sectioning with residual glandular tissue Figure . The cysNeck masses in pediatric medicine are commonly encountered and can position the clinician involved in a diagnostic dilemma. The common location for neck swelling indicating an FBCC includes the cervical (41%), parotid 35%), and preauricular regions (24%) %, and pr. This exClinically, it is challenging to diagnose FBCC. Their rarity and similar presentation with other conditions such as chronic otitis externa, abscess, parotid tumor, or tail of parotid cyst often lead to misdiagnosis, resulting in inappropriate treatment. In our case, it mimicked a parotid tumor. While parotid swelling will be present in both cases, FBCC has a various spectrum of manifestations , which may differentiate them from the parotid tumor . The masDiagnostic modalities may help provide substantial information in a patient with parotid mass, but their accuracy for diagnosis is sometimes questionable, especially in differentiating rare disease. Ultrasonography can be of first assistance, which could ascertain the cystic nature of the lesion. An ultrasound Doppler would be useful if a vascular lesion is suspected to assess flow. MRI will be able to help in providing valuable information about the lesion and anatomical relation, suggesting the possible condition, and guiding the surgical approach owing to its high resolution in soft tissue differentiation. There is no specific radiological feature for the diagnosis of FBCC as opposed to other benign cysts . HoweverThe treatment modality of choice for FBCC is surgical excision. Ideally, the access is by parotidectomy approach using modified Blair incision with adequate exposure of the facial nerve trunk . In someFBCC is a rare entity. It demands a great attention to the presenting history and physical examination together with thorough pre-operative radiological work-up in considering the condition. The presence of a parotid cyst in a pediatric age group with radiological correlation of being adjacent to the EAC should raise the level of suspicion. The complexity and close relationship of FBCC with facial nerve and EAC must not be underestimated by the treating surgeons.


Acrometastases are rare lesions that originate most commonly from the primary lung cancer. They can mislead the diagnosis and the treatment, since they often appear as an osteomyelitis of the affected area. The presence of these metastases is a sign of poor prognosis, with a life expectancy of few months. We report a case of a 78-year-old male with an acrometastasis to the distal phalanx of the right fourth toe. It was the first sign that his previously diagnosed large cell lung carcinoma had reached a metastatic stage. Amputation of the toe was considered in this case where the intense pain of his acrometastasis could not be managed even with strong analgesics. Metastases to the bone are a common site of secondary localization of certain types of cancer. However, acrometastases, defined as metastases below the elbow or the knee, occur in only 0.1% . These aA 78-year-old Caucasian male, with a history of 40 pack-year cigarette smoking and percutaneous transluminal coronary angioplasty (PTCA) done 15 years ago, was referred to our orthopedic department for an acrometastasis of his distal phalanx of the fourth toe of the right foot. His report showed that he presented to the emergency department, four months ago, with shortness of breath. He was diagnosed, based on a chest radiograph, with bilateral pulmonary edema and fluid overload. Echocardiography showed a very low ejection fraction of 25%. He was treated symptomatically with furosemide and discharged few days later on furosemide, Entresto, spironolactone and Procoralan. Two weeks after, he was rushed again to the emergency department for hypotension and dyspnea. This time, a chest computed tomography (CT) scan was performed, and a mass was detected in the left posterior-basilar lung along with fibrotic changes in the lung parenchyma  scan showed a single tumor of the left lower lobe without adenopathies, or extrathoracic disease, with changes suggestive of pulmonary fibrosis. The brain MRI did not show any particular changes. His blood test results were insignificant.His case was discussed in a multidisciplinary meeting between the oncology team, anesthesiologists, and cardio-thoracic surgeons. Due to the cardiac insufficiency and the pulmonary fibrosis, it was decided that the patient would not tolerate chemotherapy or a lobectomy. Instead, he was referred for radiation to the chest after his consent. The patient underwent five sessions of radiotherapy at a dose of 4500 cGy in 11 days. After the completion of the radiation sessions, the patient had a WHO performance score of 0 and was asked to perform a total body CT scan for follow-up after few weeks. A month later, the patient started feeling a burning type of pain and noticed some swelling in the fourth toe of his right foot, without a history of trauma or diabetes. His doctor primarily suspected a soft tissue infection, and he prescribed an amoxicillin/clavulanate regimen for seven days. After completion of the antibiotic treatment, the swelling, erythema and pain were still increasing Figures , 3.An MRI of his right foot was performed to rule out an osteomyelitis. A soft-tissue mass was seen on the MRI in the fourth toe measuring 20 x 17 mm, hyposignal with T1 and weak hypersignal with T2 short tau inversion recovery (STIR), leading to a total osteolysis of the distal phalanx Figures -7.The aspect of the mass was highly suspicious of a secondary localization of the large cell carcinoma, given the patient history. No other anomaly of the toe or the foot was seen. On the light of this discovery, he underwent a total body CT scan, which had been already planned but not completed a month earlier. The chest CT, compared to the imaging done before radiotherapy, showed 30% regression of the size of the pulmonary tumor, the appearance of multiple bilateral mediastinal and hilar adenopathies, as well as fibrotic changes of the left posterior-basilar lung, most probably due to radiation Figure . On the After realizing that his disease was advanced, the patient decided to seek palliative care only. A palliative radiation therapy for the distal phalange was not advisable because of the complete bone loss. Instead, he was referred to our orthopedic team, for a surgical intervention, aiming to amputate the affected toe. This procedure was done as a palliative intent, given the unbearable pain the patient was suffering. It was poorly controlled by opioids. The intervention was done under loco-regional anesthesia with no complications Figures , 10.The patient was rapidly relieved from the pain a few days after his discharge from the hospital. The pathology report confirmed that the amputated toe was indeed a poorly differentiated carcinoma of pulmonary origin Figures , 12.A metastatic lesion to the foot is a rare entity, especially when it comes to the digits. It is known that an acrometastasis, which is a metastasis that appears below the elbow or the knee, is a very rare entity that accounts for 0.1% of bone metastases . AcrometThe most common localization of the acrometastasis to the foot is the hindfoot  followed by the midfoot . The presence of metastasis to the forefoot  is the rarest . The cliNormal radiographs are the most common imaging technique used to diagnose acrometastasis . Their pThe main concern in the treatment of acrometastasis is the relief of pain or other symptoms, and if possible, the regain of some functional ability of the affected area . In factThe case we introduced here highlights the fact that an acrometastasis is to be suspected in patients with an active large cell carcinoma of the lung, even if the tumor was initially localized to the lung parenchyma. The rapid evolution from a localized tumor to a metastatic state made the diagnosis of the acrometastasis quite challenging, as it was the first manifestation of the evolution of the cancer. The option of palliative surgery should be integrated in the therapeutic arsenal of acrometastasis, especially when the localization of the metastasis is distal and in the digits. In this case, the amputation of the toe gave the patient great pain relief, compared to the multiple side effects of the opioids or other strong analgesics needed to relieve the pain.


Inadvertent laceration of the jugular bulb is a potentially serious complication of the retrosigmoid transmeatal approach to vestibular schwannomas. Here, the authors present the case of a 51-year-old woman with a right Hannover T4a vestibular schwannoma and bilateral high-riding jugular bulb, which was opened during drilling of the internal auditory canal (IAC). They highlight the immediate management of this complication, technical nuances for closing the defect without occluding the jugular bulb, and modifications of the standard technique needed to continue surgical resection.https://stream.cadmore.media/r10.3171/2021.7.FOCVID2155The video can be found here:  The patient was a 51-year-old woman with history of right-sided deafness first noticed 3 years prior to consult. Audiogram confirmed nonserviceable hearing on the right side, and MRI showed a cerebellopontine angle mass compatible with a Hannover T4a vestibular schwannoma extending into the most lateral part of the internal auditory canal. Bilateral high-riding jugular bulbs were also noted, reaching up to the level of the inferior limit of the IAC, with the right side being dominant. However, it did not appear as it would be exposed during drilling of the IAC.We performed a right retrosigmoid craniotomy in dorsal decubitus in standard fashion. After opening the lateral cerebellomedullary cistern and exposing the tumor, we raised a dural flap to expose the posterior wall of the IAC before beginning tumor resection.Drilling began using a large cutting drill bit to outline the IAC, then using a smaller bit in the lateral portion. At approximately 8 mm from the internal auditory meatus, brisk bleeding was encountered from the jugular bulb while using a large diamond bit. The head was immediately lowered at the level of the heart, and we let blood fill the surgical site, not suctioning directly over the jugular bulb, to prevent air embolism. A small cotton pledget was placed over the defect to aspirate the pooled blood safely; then a small crushed muscle patch was used to seal the defect, carefully positioning it below the cotton pledget. This graft is always obtained during the approach to reconstruct the IAC after resection of the tumor, which is why we prefer it over other synthetic materials such as bone wax or gelatin sponge. When positioning the graft, extreme care must be taken to avoid occluding the jugular bulb, so the patch must be gently placed to cover the laceration of the bulb without tamponading the lumen, nor entering the bulb and potentially become embolic material. It may take a while to obtain adequate closure and still allow proper visualization to continue the surgery. After bleeding stopped, fibrin glue was applied to seal the defect.Using cutting and coarse diamond drill bits of decreasing size, drilling was continued with care to avoid catching the muscle with the drill. Since visualization to the most lateral and anterior part of the IAC was still partially limited by the graft, we needed to expand the opening of the superior wall of the canal to improve the angle of attack. Here, we also needed to trim the muscle graft to improve visualization. Then, we continued gradually drilling around the muscle patch from medial to lateral, and from superior to inferior, palpating with a blunt dissector the limits of the canal, until we achieved sufficient exposition, which meant opening around 100° of it, far more than it is usually needed.The dura of the IAC was incised parallel to the path of the nerves, and then internal debulking began at the level of the meatus. Using blunt and sharp dissection, the tumor was gradually reduced and separated from the brainstem, following the arachnoid plane. Inside the IAC, the tumor was gently dissected from the nerves. In this case, the superior vestibular nerve was first identified and cut. At this point, it was noted that residual tumor remained in the most lateral part of the inferior vestibular nerve, which was the nerve of origin, so additional drilling was needed to expose the remaining lesion. By carefully drilling below the patch, the tumor was removed completely from the IAC.Lastly, the cisternal portion of the tumor was resected, first identifying the cochlear nerve in the anteroinferior quadrant of the tumor, and the facial nerve in the medial part of it. By gently dissecting the tumor from the facial nerve, gross-total resection was achieved. The facial nerve could be preserved both anatomically and functionally, whereas the cochlear nerve had to be sacrificed. Finally, the IAC was reconstructed with a large muscle patch and fibrin glue.Postoperatively, the patient developed House-Brackmann grade III facial palsy and moderate headache, that spontaneously resolved on postoperative day 5. MRI confirmed gross-total resection of the lesion, as well as a small thrombus on the dome of the jugular bulb, without occlusion of venous blood flow. We do not routinely anticoagulate such cases.1–3 In case of laceration during the transmeatal stage of resection, all actions should be focused on preventing air embolism. The defect may be effectively closed with a crushed muscle patch, and extreme care must be taken to position the graft without occluding blood flow. It is also prudent to evaluate stopping surgery in case complete occlusion is suspected, particularly in dominant bulbs, as severe cerebellar edema may rapidly set in and further complicate resection. In nondominant bulbs, occlusion may produce engorgement of cerebellar veins, or an increase in cerebellar venous bleeding. Finally, anticoagulation is not usually indicated, and few reports have shown safety in close monitoring of these cases.4–6In conclusion, a high-riding jugular bulb must be actively looked on preoperative images, as it may be present in around 9% of patients undergoing surgery for vestibular schwannoma.The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this publication.Primary surgeon: Ramina. Assistant surgeon: Constanzo, Coelho Neto. Editing and drafting the video and abstract: Constanzo, Teixeira. Critically revising the work: Teixeira, Coelho Neto, Ramina. Reviewed submitted version of the work: all authors. Approved the final version of the work on behalf of all authors: Constanzo. Selecting and editing neuroimages: Teixeira.


Lymphangiomas are benign vascular tumors arising from the lymphatic system. They commonly affect the head and neck regions. Pancreatic involvement is extremely rare. Even though they are rare, it should be considered as a differential diagnosis for patients who present with pancreatic mass.We report the case of a 6-year-old African male patient who presented with abdominal mass and dull aching pain of 6 months duration. He was examined and underwent excision, with biopsy showing pancreatic lymphangioma.These tumors are benign and slow growing and have potential to regress spontaneously. Thus, most literature agrees that surgical interventions should be reserved for symptomatic cases. Lymphangiomas are benign congenital tumors of lymphatic vascular origin. They commonly affect head and neck regions. Pancreatic involvement is extremely rare. These tumors are usually congenital but can arise as a result of blockage of lymphatic flow from trauma, fibrosis, inflammation, or radiotherapy. Patients are usually asymptomatic but can present with abdominal pain and mass. We report the case of pancreatic lymphangioma in a 6-year-old male patient presenting with abdominal pain and mass.A 6-year-old African male child presented with abdominal mass and dull aching type of abdominal pain of 6 months duration that worsened 1 month before presentation. He had no past medical illness or surgical interventions. He also had no family history of similar illness. Upon our evaluation, the patient was stable. There was an ill-defined, smooth-surface, nontender, fixed, intra-abdominal mass at the center of the abdomen extending to the left upper quadrant measuring about 8 cm × 10 cm. Liver function test, renal function test, pancreatic enzymes, and tumor markers were all in normal range. Abdominal computed tomography (CT) scan showed multiple cystic mass adjacent to each other with no communication between the masses; the largest cyst measured about 8 cm × 7.5 cm × 9.5 cm. The mass was found adjacent to the head of the pancreas with no clear plane between the mass and pancreas (Fig. The patient was operated, with an intraoperative finding of well-capsulated multiloculated cystic mass that was wrapped with pancreatic tissue and no sign of local invasion Fig. . The masLymphangiomas are benign vascular tumors arising from the lymphatic system . They acPancreatic lymphangioma is a very rare tumor accounting for less than 1% of abdominal lymphangioma and less than 0.5% of all cystic pancreatic lesion . These tThese tumor are benign in nature, slowly growing, and usually asymptomatic . Some paThere are no associated laboratory findings specific to this abnormality. Abdominal imaging such as ultrasound and CT scan assist in the diagnosis and evaluation of such lesions; however, imaging by itself is not 100% specific to differentiate lymphangiomas from other cystic pancreatic lesions such as pseudocysts, cystadenomas, congenital cysts, and ductal carcinomas. Thus, this pathology can be differentiated from other cystic pancreatic lesions by histopathologic examination , 9, 10. Pathologically, lymphangioma can be classified as macrocystic, microcystic, or mixed-type lymphangioma. Macrocystic lymphangiomas are greater than 1 cm in diameter and occur in areas with loose connective tissue such as in the abdomen, neck, and axilla, while microcystic lymphangiomas are less than 1 cm in diameter and arise in areas with dense connective tissue such as tongue and lip. The natural history of lymphangioma depends on its histology type. Macrocystic and mixed types can spontaneously regress, while microcystic lymphangioma does not regress. Therefore macrocystic and mixed types can be serially followed in asymptomatic patients with stable tumor , 10.Surgical interventions are reserved for patients with symptomatic pancreatic lymphangioma and for those with diagnostic dilemmas. Excision of the mass is considered to be curative. As there is a high chance of recurrence, the mass should be removed in its entirety , 3, 5. WPancreatic lymphangioma, even though it is a rare tumor, should be considered as a differential diagnosis in patients who present with cystic pancreatic mass. This tumor is benign and slow growing and has the potential to regress. Thus, surgical interventions should be reserved for symptomatic cases.


Epipericardial fat necrosis (EPFN) is a rare, benign cause of acute chest pain imitating symptoms of life-threatening diseases, such as acute coronary syndrome. Here We report a 37-year-old, healthy male presented to the emergency department (ED) with sudden-onset pleuritic chest pain after an isometric physical training. Initial cardiac workup included ECG, echocardiography was unremarkable, but diagnosis of an inflammatory process that involved the epipericardial fat tissue surrounding the heart was made by showing encapsulated fatty lesion, enhanced adjacent parietal pericardium using of contrast‐enhanced magnetic resonance imaging (MRI). Magnetic resonance imaging would help physicians to differentiate EPFN from severe and life-treating conditions. Fat necrosis can occur at multiple sites in the body: in the breast and subcutaneous fat, in the abdomen, and in the mediastinum. The etiology of fat necrosis is various as trauma, torsion, and inflammation.Epipericardial fat necrosis is an inflammatory process that involves pericardial and adjacent mediastinal fat and leads to acute chest pain CT scan has been suggested as a modality of choice to detect EPFN A 37-year-old man presented to the emergency department (ED) with a sudden-onset pleuritic pain in his right anterior lower chest radiating to the upper abdomen. The symptoms started a day before to presentation, shortly after a powerlifting training session. The pain was graded 6 of 10 on a 1-0 scale by patient. There were no associated signs or symptoms such as fever, chills, palpitations, syncopes, cough, or shortness of breath. His physical exam was unremarkable, with a normal resting blood pressure, normal heart sound without murmurs or friction rub, and normal breath sounds with no wheezing or crackles. The patient did not report any significant past medical history and cardiovascular risk factors. An electrocardiogram (ECG) showed normal sinus rhythm with no ST, T wave changes. The laboratory tests, including the D-dimer: 100 ng/L (Reference range <500 ng/L), troponin I: <0.01 (Reference range < 0.016 ng/L), CRP: 2.0 mg/mL (Reference range < 8.0 mg/l) were normal. A transthoracic echocardiography (TTE) reportedly showed normal global systolic left ventricular function without regional wall motion abnormality. There was no evidence of significant pericardial effusion.A broad range of causes from life-threatening aetiologies to self-limited ones should be considered in the differential diagnosis of acute chest pain. It was including myocardial ischemia, pulmonary embolism, myocarditis, pericarditis, pneumonia, musculoskeletal etiologies, and upper abdominal pathologies such as acute cholecystitis and hepatitis With the suspicion of muscle strain, the emergency physician prescribed analgesic medication for the patient. Due to the poor response to treatment and the radiating nature of the chest pain to the upper abdomen, he was referred for a contrast-enhanced MRI. A 1.5 T MRI revealed an encapsulated, oval-shaped, and fat-containing nodular lesion (18 × 11 mm) with a surrounding thin, low signal intensity rim, that was located in the right cardiophrenic angle  and 2. ABased on the patient history, laboratory test results, clinical and imaging findings, diagnosis of EPFN was made. The course of disease was benign and self-limited. On follow-up evaluations at 1, 3, and 6 months: the patient's pain was completely disappeared.Radiologic follow-up was not performed.EPFN is a rare and self-limited cause of acute chest pain, mimicking life-threatening diseases like myocardial infarction and pulmonary embolism EPFN affects both genders equally, with an average age of 50 EPFN frequently involves the left side The absence of specific signs and symptoms on history and physical exam besides normal laboratory and ECG findings necessitates the utility of imaging modalities to confirm EPFN.CT scan has been suggested as a modality of choice to detect EPFN MRI with fat-suppression techniques is an accurate method to characterize adipose tissue with a high soft-tissue contrast Pineda et al reported using a combination of clinical and CT scan-based criteria for diagnosis of EPFN -To review the clinical and imaging features of EPFN as a rare and underdiagnosed cause of acute chest pain.-To point out the ability of MRI in the differentiation of EPFN from fat-containing tumors, which reduces the need for complementary invasive and non–invasive diagnostic techniques.-To highlight the advantages of MRI compared with CT scan in the diagnostic workup of patients with acute chest pain presentation.The authors of this article confirm that informed consent for publication of this case report was obtained from the patients.


Olfactory neuroblastoma (ONB) is a rare malignant neuroectodermal tumor of the nasal cavity. Olfactory neuroblastoma centered in the posterior right orbit with prominent orbital protrusion is even rare. Grading ONB is extremely important as individualized treatment plans must be formulated according to tumor grade. We report the case of a 67-year-old female who presented with the chief complaints of persistent nasal congestion with intermittent epistaxis and unilateral proptosis over the past five years. Radiological imaging was suggestive of a large heterogeneous mass in the right superior nasal cavity with extensions into the right medial orbit, nasopharynx, the right maxillary sinus, the anterior cranial fossa, right ethmoidal, frontal and bilateral sphenoidal sinuses, as well as into the right frontal lobe. Assessment of the radiologic features revealed the diagnostic possibility of olfactory neuroblastoma. A nasopharyngeal biopsy confirmed an olfactory neuroblastoma. Frontal osteoplastic craniotomy and excision of the intracranial part of the tumor from above and transnasal endoscopic removal of the mass in the nasal cavities, paranasal sinuses and right medial orbit from below was done. Evaluation of histopathological characteristics and immunohistochemical findings revealed a diagnosis of WHO grade IV olfactory neuroblastoma. Because of poor economic condition, the patient did not take adjuvant radiotherapy and chemoradiation and post-operative examination. We report a huge ONB centered in the posterior right orbit with prominent orbital protrusion. Magnetic resonance image and computed tomography are helpful for evaluating the appearance and the extent of ONB, as well as grading this tumor, which may aid therapeutic decisions and improve survival. Olfactory neuroblastoma (ONB), also called as esthesioneuroblastoma, is a rare malignant neoplasm of the nasal cavity arising from the olfactory neuroepithelium. It accounts for 3–5% of all nasal and sinonasal malignancies with no race or gender predilection . ONB hasA 67-year-old female was presented to the Otolaryngology department with unilateral prominent orbital protrusion and a mass in the right nasal cavity, which caused persistent nasal congestion with intermittent epistaxis over 5 years. Three years earlier she had been treated for suspected chronic rhinitis without resolution of symptoms. She came for further treatment with progressive enlargement of the mass and worsening of symptoms over the last 2 years.A nasal exam was performed using rigid endoscopy, which revealed a large mass, medial to the middle turbinate in the right nasal cavity. The mass extended to the choana, but did not appear to extend into the nasopharynx clinically. The lesion was pink and lobulated with a rubbery, non-friable texture. No lesions were identified in the left nasal cavity. Examination of visual acuity revealed no visual impairment. She had no history of headache, dizziness, or diplopia. Her blood profile for biochemistry and hematology was within normal limits. Tests for human immunodeficiency virus (HIV), hepatitis B surface antigen (HBsAg), and hepatitis C virus were negative.Magnetic resonance image (MRI) studies revealed a heterogeneous mass with isointense on T1-weighted imaging  scans demonstrated a heterogeneously enhancing lobular soft tissue mass that filled the right nasal cavity and orbit Fig. g. ContraFrontal osteoplastic craniotomy and excision of the intracranial part of the tumor from above and transnasal endoscopic removal of the mass in the nasal cavities, paranasal sinuses and right medial orbit from below was done. On histopathological examination, evaluation of the hematoxylin and eosin stained biopsy specimen revealed interconnecting tumor lobules embedded within a richly vascularized fibromyxoid stroma Fig. i. The tuOlfactory neuroblastoma (ONB) is a rare malignant tumor of neural crest origin, arising from the olfactory epithelium of the nasal vault . It is aThe most common presenting symptoms are unilateral nasal obstruction (70%), and epistaxis (50%). Other symptoms include anosmia, headache, pain, excessive lacrimation, and rhinorrhea. In particular, patients with periorbital extension may present with proptosis, periorbital edema and decrease visual acuity, just like the rare case we reported. Uncommonly, ONB may be associated with syndrome of inappropriate antidiuretic hormone secretion (SIADH) with dilutional hyponatremia or ectopic adrenocorticotropic hormone (ACTH) production leading to Cushing syndrome .ONB may histologically mimic a number of types of tumor within the sinonasal tract, making it much difficult to diagnosis . Multi-mHistologically, ONB typically have a high nucleus/cytoplasm ratio, with scant, poorly defined cytoplasm. High-grade tumors characteristically present with nuclear pleomorphism, mitotic figures (> 2 per high-power microscope field), and necrosis . Homer We values in ONB [The diagnosis of ONB is extremely challenging as several sinonasal neoplasms must be excluded. The differential diagnosis is widely broad including rhabdomyosarcoma, lymphoma, undifferentiated carcinoma, neuroendocrine carcinoma, melanoma and Ewing’ s sarcoma . The difs in ONB . In parts in ONB . The dias in ONB .Kadish et al. and Dulguerov et al. proposed ONB classifications based on primary tumor extension and clinicoradiographic data, respectively. Kadish et al. were the first to propose a staging classification for ONB . Morita The management of ONB usually requires craniofacial surgical approach, trephination procedure, which is technically challenging and achieving good results are difficult. Recently, authors have come to an agreement that a multimodal approach is necessary to treat patients with ONB. Treatment modalities for ONB are enbloc resection, extra cranial resection or surgery combined with radiotherapy and/or chemotherapy. But unfortunately, the patient did not take adjuvant radiotherapy and chemoradiation and post-operative examination because of poor economic condition.We report a huge ONB uncommonly centered in the posterior right orbit with prominent orbital protrusion. From an analysis of the findings in the olfactory neuroblastomas, the diagnosis of ONB should be considered when radiographic imagings reveal a dumbbell-shaped mass in the nasal cavity with peritumoral cyst, and multi-modality imaging are helpful for evaluating the appearance and the extent of ONB, as well as grading this tumor, which may aid therapeutic decisions and improve survival.


Anal canal duplication is a rare gastrointestinal malformation characterized by extra anal orifices at 6 o'clock in the lithotomy position. To date, there have been only 110 reported cases. The purpose of this study is to contribute two infant cases, one of which is associated with anorectal stenosis, which has never been described. Anal canal duplication (ACD) is the rarest malformation of the distal alimentary tract, usually defined as “cases with an additional blind anal orifice, sagittally positioned, exhibiting the same histological features as normal anal canal” . The patWe describe two additional infant cases: case 1 presented with refractory constipation, and case 2 was asymptomatic. A barium enema of case 1 revealed anorectal stenosis and mega-rectosigmoid. Two patients underwent surgery, and the pathology confirmed the diagnosis.An 8-month-old baby was referred for refractory constipation. She was born full-term and fully breast-fed since birth, without other medical issues. As she began complementary food, she developed progressive constipation. Treatment such as laxatives and enema was given to aid in defecation, but the condition aggravated. A posterior anal orifice was identified on physical examination . Barium Removal of the entire ACD and anoplasty were performed in a prone position. After anesthesia, we used a urethral catheter to confirm the depths of ACD and the normal anus. We could put a maximum 10Fr catheter (about 3.3 mm in diameter) through the normal anus, while only a 6Fr (about 2 mm in diameter) catheter could be introduced into the ACD tube with a depth of 3 mm. The existence of ACD led to rectum stenosis. The operation could be divided into two parts: (1) ACD dissection and (2) treatment of rectal stenosis and anoplasty.We started with a dissection of ACD ventral mucosa and found a 20-mm-long blind tubular structure that started in the rectum and ended near the dentate line, sharing a mucosal wall with the normal anus and rectum but without communication . The bliHistology confirmed the diagnosis of ACD and showed the specimen covered with squamous epithelium, fibers, blood vessels, and smooth muscle tissue . Hegar’sAn 11-month-old girl was found to have a posterior anal orifice since birth . She wasA simple mucosectomy was performed in the prone position. The duplication was completely resected and sent for pathological examination, with the posterior rectal wall left completely intact . HistoloAnal canal duplication is a rare malformation with poorly understood etiology and less defined clinical management criteria. Patients are always asymptomatic or just with local symptoms around the perianal region. Differential diagnosis should be carefully evaluated between ACD with perianal fistula and perianal abscess. Comorbidities were found in 25% of the patients, of which presacral masses were the most common, accounting for 50%, and anorectal malformations (ARMs) account for 5% , 5. We rEarly diagnosis is crucial but easy to miss in neonates . Our patThe etiology and the reason for the female incidence dominance remain poorly understood due to the complexity of embryonic development. Interestingly, male patients make up a larger proportion of patients with an imperforate anus . PreviouThe prognosis for surgery is favorable, but the necessity is controversial . Akova eThe clinical features of ACD, the anatomic relationship with the anal sphincter, and its associated malformations are important determinants of the surgical approach . For casACD is a rare malformation with a lack of well-defined clinical care guidelines and a poorly understood etiology. Early clinical suspicion of ACD and association elimination are crucial. Contrast enema and MRI are useful auxiliary examinations for ruling out presacral masses and ARMs. For ACD associated with anorectal stenosis, a transanal pull-through procedure of the rectum with full thickness is recommended with good results.


Urachal cancer is very rare, accounting for only 0.5–2% of bladder-associated malignancies and 0.01% of all cancers in adults. It has an insidious appearance, an aggressive behavior and a poor prognosis. The most common symptoms are hematuria and the presence of a palpable hypogastric mass. The scarcity of cases and the low number of studies carried out explains the lack of an evidence-based management strategy, but it seems that surgical treatment  represents the gold standard, while neoadjuvant and adjuvant chemotherapy or radiotherapy has a limited impact on overall survival. Since mucinous cystadenocarcinoma of urachal origin is a very uncommon pathological condition the differential diagnosis may be difficult and pathological investigations have to elucidate this disorder. It is worth mentioning the psychological impact on the patient in addition to the medical aspects. A rare condition is associated with heightened risk for mental health and psychosocial difficulties and this must be taken into account in the subsequent follow-up of the patient. In order to increase awareness of this rare entity we report a case of a 40-year-old male with a urachal adenocarcinoma who was treated surgically, with a favorable outcome. We also perform a brief literature review about this type of tumor. Urachus is an embryological remnant that connects the umbilicus and anterior wall of the bladder . During Frequently, patients present with microscopic or gross hematuria, occasionally with abdominal pain, dysuria or mucosuria. Rarely, some unusual urinary symptoms, such as pollakiuria, pyuria, recurrent urinary tract infection or umbilical discharge may occur. Systemic manifestations are often found in the elderly population, such us fever, weight loss, and nausea .The diagnostic pattern may include imaging. Ultrasonography can expose an inhomogeneous mass located in the midline of the abdomen, sometimes associated with calcifications that are pathognomonic. Computed tomography (CT) scan or magnetic resonance imaging (MRI) are used in evaluating the tumor  ,14. A neThe stage of the disease decides the management of the treatment and oncological outcomes. Surgery is the gold standard for localized tumoral mass. Excision of the urachus and the umbilicus, and partial/radical cystectomy, with extended bilateral pelvic lymphadenectomy (PLND), are routinely performed. The target in surgical management of UrC is to obtain negative surgical margins, particularly after partial cystectomy ,13. ReguPerioperative chemotherapy has a limited role, especially in advanced stages when the patient has a poor prognosis. Neoadjuvant chemotherapy could be recommended in unresectable tumors, but only if the response may lead to surgical treatment with negative margin resection . RadiothRegarding urachal tumors, they are associated with a poor prognosis and with a five-year survival rate of between 9 and 50 % ,20,21. TMucinous cystadenocarcinoma of urachal origin is a very uncommon pathological condition, so the differential diagnosis may be difficult and pathological investigations have to elucidate this disorder. Due to its rarity, there is some disagreement in the literature about the diagnostic criteria, the staging system and the best treatment options. Previous studies about UrC are insufficient, and the optimal therapeutic management is still a matter of debate. In order to increase awareness of this rare entity we report a case of a 40-year-old male with only gross hematuria as a symptom and with a large nonspecific intra-abdominal tumoral cystic mass: UrC (23 × 9 × 8 cm), adherent to the anterior abdominal wall and to the urinary bladder. We also perform a brief literature review about this type of tumor.A 40-year-old male patient presented to our hospital with a one-week history of lower urinary symptoms (gross hematuria) and lower abdominal pain. He denied nausea, vomiting, constipation, diarrhea, or any other associated symptoms. The patient was otherwise healthy with no prior surgical or medical interventions and no history of tobacco, alcohol or drugs use. Suprapubic ultrasonography performed in another hospital showed an enlarged cystic intra-abdominal mass in his lower abdomen. The mass, which was measured as 80/90/120 mm, possessed a well-defined boundary, regular shape and several separations nearing the bladder. The patient was admitted to the General Surgery department of the Military Emergency Hospital, Iași, for diagnosis and treatment.Physical examination revealed a large, mobile mass in the hypogastric region. Laboratory studies ), tumor markers (carcinoembryonic antigen (CEA) and cancer antigen (CA 19-9)) were unremarkable.A contrast computerized tomography (CT) scan of the chest, abdomen and pelvis revealed parietal thickening of the antero-superior bladder wall (40 mm), with enhanced contrast. A heterogeneous, hypodense mass was located on the topography of the communication with a voluminous anterior bladder diverticulum (9 cm). The tumor had developed partly in the bladder, partly in the diverticulum and infiltration of surrounding tissue of the bladder near to the tumoral mass was identified.No abnormal enhancement was identified. Small calcifications were seen in the lower part of the cystic mass. There was no retroperitoneal lymphadenopathy, ascites, or intestinal mechanical obstruction A–C.Cystoscopy detected mucosal erosion and excluded a potential invasion of the bladder. Furthermore, it revealed no bladder diverticulum. Pelvic Magnetic Resonance Imaging (MRI) showed a large cystic mass confined to the pelvis measuring 75/91/127 mm (AP/TR/CC). The complex cystic lesion extended from the umbilicus to the antero-superior dome of the bladder, on the trajectory of the medial umbilical ligament. The tumoral mass extended anteriorly to the plane of the rectus abdominal muscles, and the postero-lateral came into contact with some intestinal loops and a portion of the sigmoid colon. In the caudal portion the antero-superior wall of the bladder was imprinted by a parietal infiltrative nodular lesion (not exceeding the mucous layer), in hyposignal T1 and T2, with several areas in hypersignal T2 and STIR, without diffusion restriction and intense contrast and size of 27/31/29 mm (AP/TR/CC).The aspects might suggest an urachal cyst with milky transformation in the portion of the bladder dome. There were several nodes with a short infracentrimetric axis located external iliac and bilateral inguinal A–C.Radiologically, a diagnosis of urachal tumor with unknown malignant potential was put forth.The patient underwent a surgical radical resection through a suprapubic midline incision. Laparotomy revealed a large intra-abdominal cystic mass adherent to the anterior abdominal wall and to the urinary bladder A,B. We pMacroscopically, the mass weighed 583 g and measured 23 × 9 × 8 cm. It was covered by a thin membrane. The cut surface showed a cystic mass filled with yellow-greenish semisolid material.Microscopically, the fragment of the bladder wall was lined with urothelium without dysplastic changes. The wall identified cavity space lined with urothelium without significant changes, located in an area of fibrosis with chronic inflammatory infiltrate. Morphological aspects related to clinical data may correspond to the urachal orifice.The cystic space was characterized by fibrosis and a sclero-hyalinosis wall, chronic inflammatory infiltrate and calcifications, and lined with intestinal mucinous epithelium with low-grade dysplasia, but also with high-grade areas, frequently associated with mitosis. There were also places of intraparietal extracellular mucus, lined with mucinous epithelium with high-grade dysplasia. The cystic wall had adipose tissue or smooth muscle bundles in the periphery.The described intra-cystic area was represented by sclero-hyalinosis with important calcifications and bone metaplasia. It was noticed on a slope, in the areas of sclero-hyalionosis, that there was extracellular mucus in which nests of cells with important cytonuclear atypia and mitosis were identified, an aspect that could correspond to a minor component of mucinous adenocarcinoma.Morphological and immunohistochemistry (IHC) aspects correlated with imaging data argued in favour of a mucinous cystadenocarcinoma of urachal origin, possibly developed on a potentially malignant mucinous cystic tumor. Bladder intraparietal arrangement and excision margins on the examined sections located in normal adipose connective tissue corresponded to local evolution (Sheldon Stage IIIA). No tumoral involvement of the bladder was found on the examined tissue section.β-catenin. These IHC results supported the diagnosis of urachal origin.Immunohistochemical stains expressed CK20, was positive for CK7, SATB2 and CK34𝛽E12 and presented diffuse membrane staining for The patient’s urinary symptoms were relieved, abdominal drainage was removed on the sixth day and he was discharged on the 7th postoperative day after an uneventful stay. The urethral catheter was removed after 21 days and no complications were recorded after the patient’s release. The patient was subsequently referred to a medical oncologist who decided to perform periodic imaging evaluation without further adjuvant treatment. The patient was evaluated by thoraco-abdomino-pelvic computer tomography after 3 months from surgery. The scan did not identify any signs of local recurrence or pathological lymphadenopathies .Using the Medical Subject Headings MeSH term “urachal” and “urachal carcinoma”, we performed a PubMed literature search for randomized controlled trials (RCTs), systematic reviews, observational studies, series of cases studies and case reports from the earliest possible date to May 2022. Articles and their reference lists, published in English, were analyzed for other relevant articles. Searching PubMed for the term “urachal”, 1663 articles were found, but when the term “carcinoma” was added, only 365 articles returned. We reviewed the title and the abstract of the articles and the full text of 98 articles, to extract data on incidence, sex ratio, diagnosis, etiology, associated abnormalities or treatment. Ninety-two of all the articles found were quoted.Primary urachal adenocarcinoma is a rare entity, first described by Hue and Jacquin in 1863 and responsible for only 10% of primary bladder adenocarcinoma ,23. LessUrC is often perceived as a poor prognosis malignancy, but, corresponding to SEER and RKI records, the relative five-year CSS rate was 54.8% in Germany and 64.4% in the USA . While eAlmost 70% of urachal adenocarcinomas are mucin-producing tumors and, as happened in our case, display calcifications . AbdominA computer tomography detail of urachal carcinoma is a midline mass antero-superior to the dome of the bladder with low-attenuation components represented by mucin . In 50% Tumor markers have become the key support for the diagnosis of various neoplastic lesions, but not all have sensitive markers. Considering the low incidence of urachal tumors, the literature has not identified a sensitive tumor marker.The largest cohort registered was by Siefker-Radtke; they found elevated (>3 ng/mL) CEA serum levels in 59% of patients with urachal adenocarcinomas median: 36 ng/mL), which also decreased in response to chemotherapy, suggesting the potential utility of CEA testing in follow-up surveillance of the patients ,36. Elev ng/mL, wOther serum biomarkers described in low case numbers of urachal adenocarcinomas include lactate dehydrogenase (LDH) , cancer Amin et al. and Paner et al. have created a classification system to help maintain consistency in naming the epithelial neoplasms of the urachus and more specifically the mucinous cystic tumors  65,66].,66.65,66Location of the tumor in the bladder dome and/or anterior wallEpicenter of carcinoma in the bladder wallAbsence of widespread cystitis cystica and/or cystitis glandularis beyond the dome and anterior wallAbsence of a known primary tumor elsewhere.A set of criteria was published in the 2016 World Health Organization (WHO) for the diagnosis of urachal adenocarcinoma and they are :LocationThere are a few staging systems for urachal carcinomas . The universally accepted one, and the most accurate, is the staging proposed by Sheldon  10,32],,32,10,325.Differentiating between invasion/metastasis of colorectal adenocarcinomas and urachal adenocarcinomas6.Distinguishing urachal adenocarcinomas from those of primary bladder origin7.Identification of the origin of a mucinous adenocarcinoma of unknown primary sites is also important because urachal adenocarcinomas regularly metastasize to various organs, such as the bone, lung, and liver .Biomarkers for differential diagnostic purposes are required given the overlapping histopathological features of adenocarcinomas of urachal and primary bladder origin and from different sites  ,68,69,70β-catenin.The immunohistochemical markers most often engaged in the work up of adenocarcinomas of different sites usually include Cytokeratin 20 (CK20) and CK7. The urachal adenocarcinoma is generally positive for CK20 and CK7 in 60% of the cases, and for CK34𝛽E12 in 66% of the cases, but only very focally ; nuclearIn the study conducted by Henning Reis et al., they concluded that CK20, havingan overall positive rate of 97%, and CK7 displayed a pooled reactivity rate of 51%, compared to considerably lower rates in colorectal cancer (0–38%) .β-Catenin is a protein involved in cell-cell adhesion and gene transcription regulation and, as a biomarker, can be found in the majority of cases . NuclearUseful biomarkers of urachal adenocarcinomas, like alpha-methylacyl-CoA racemase AMACR, p504s), CD15 (Leu-M1), carcinoembryonic antigen (CEA), CK34βE12 (high-molecular weight cytokeratin), GATA binding protein 3 (GATA3), mucin 2 (MUC2), and mucin 5AC (MUC5AC), should be taken into consideration . Further4s, CD15 The differential diagnostic between the various tumors entities is enlightened by immunohistochemical staining of a panel of antibodies. This process also includes the use of further antibodies in addition to the core panel.The recommended treatment for nonmetastatic cases is surgery, but no standard surgical treatment has been recommended until now. In localized disease, primary bladder adenocarcinomas are usually treated with complete cystectomy, while urachal adenocarcinomas mostly require partial cystectomy with en bloc removal of the umbilical ligament and umbilicus  and have a significantly different impact on quality of life ,9. PartiThe role of neoadjuvant or adjuvant chemotherapy or radiotherapy (R1 excision) is still to be proven ,53. VariPsychological challenges are related to medical aspects of the disease. More precisely, the lack of knowledge of patients about the medical condition, uncertainty about the future, evolution, disease progression and treatment. Attitude of the medical multidisciplinary team involved should guide the patient in managing his or her medical and social situation .Urachal adenocarcinoma is a rare condition with a poor prognosis due to locally advanced or metastatic disease at the time of diagnosis, especially because most patients are asymptomatic. The most common symptoms are hematuria and the presence of a palpable hypogastric mass, such as in the case presented. Surgical treatment represents the gold standard, while adjuvant chemotherapy or radiotherapy has a limited impact on overall survival. Besides the rarity of the condition in the general population, another particularity of the case is that rare diseases lead to a negative psychological impact on the patient. People with rare diseases may face challenges different from those with frequent medical conditions.