Choledochal cyst are rare congenital disease of the biliary tree. It presenting as cystic dilatations of the biliary tree can involve the extrahepatic biliary radicles, the intrahepatic biliary radicles or both. They are typically a surgical problem of infancy and childhood, but less than a quarter of the patients the diagnosis is delayed until adulthood as it presenting with vague and nonspecific signs and symptoms. In a case with biliary symptoms, abdominal ultrasound scan is the initial imaging modality of choice. Precise and accurate delineation of the biliary system mandates cholangiography with the advantage of non-invasive magnetic resonance cholangiopancreatography (MRCP) over endoscopic retrograde cholangiopancreatography. A case report of 11-year-old Tanzania girl with abdominal swelling and jaundice presented to a health facility for evaluation. She complained of abdominal swelling that her mother noticed when she was 3 years old, located above the umbilicus and since then it was not changed its size until 8 years later when it rapidly increased in size associated with non-specific dull pain. Abdominal computed tomography (CT) scan was done showed A well-defined hypo-attenuated non-enhancing retro-gastric cyst. Percutaneous transhepatic cholangiopancreatography (PTC) was performed due to inconclusive findings from CT-scan showed extrahepatic huge cystic dilatation, dilated central right hepatic ducts, left intrahepatic ducts failed to be visualize. Diagnosis of choledochal cyst type isovaleric acidaemia (IVA) was made. Explorative laparotomy was done, huge cystic mass occupying common bile duct was seen below the liver with distended gallbladder covered with visceral peritoneum. Second part of duodenum, pancreases and transverse colon was adhered to the inferior surface of the mass that further make difficult for cyst excision and reconstruction. Cyst was decongested and cholangioenterostomy with Roue-en-Y reconstruction was made. Cholecystectomy was done, hemostasis archived abdomen closed and patient sent to Intensive care units (ICU). Despite of advanced diagnostic modalities, delayed diagnosis of choledochal cyst can be a challenge due to its vague and nonspecific signs and symptoms. Excision of the cyst and reconstruction by hepatojejunostomy as the standard therapy could be difficult due to its biliary complications such as adhesion and infection and hence cyst-enterostomy drainage procedure can be done as option for relief of patient discomfort and prevent further complications. Choledochal cysts are rare congenital bile duct anomalies, presenting as cystic dilatations of the biliary tree can involve the extrahepatic biliary radicles, the intrahepatic biliary radicles, or both. They may occur as a single cyst or in multiples within the biliary tree. They are typically a surgical problem of infancy and childhood, but less than a quarter of the patients the diagnosis is delayed until adulthood. The presentation is often vague and nonspecific. The so-called classic triad of intermittent jaundice, abdominal mass, and pain is found in a minority of cases according to most case series . The mosPatient information: an 11-year-old Tanzania girl with abdominal swelling and jaundice presented to a health facility for evaluation. She complained of abdominal swelling that her mother noticed when she was 3 years old, located above the umbilicus and since then it was not changed its size until 8 years later when it rapidly increased in size associated with non-specific dull pain. During rapid increase in size of the swelling she started presenting with intermittent yellowish discoloration of eyes. During the course of the illness, she had no history of vomiting, difficult in passing stool or passing bloody stool or black tarry stool. She reported history of passing deep colored urine during the period of yellowish discoloration with no history skin itching or passing clay colored stool. The patient had received treatment from multiple primary health facilities consisting of antibiotics and analgesics due to the complaints without relief until when she was referred to Tertiary Hospital.Clinical findings: the child presented in hospital fully conscious and ambulant, some pallor on her conjunctiva and tinge jaundice. She had no enlarged periphery lympnodes, no lower limb oedema and vital signs were normal. Her body mass index (BMI) was 17kg/m3 indicated that she was underweight. Abdomen was symmetrically distended with girth of 74cm at its largest point of circumference, moves with respiration with visible superficial veins. Mass was palpable on right hypochondriac region extending to involve right lumbar, right iliac, umbilical regions with smooth margin and edges, firm, tender on deep palpation, unable to go above it but its lower margin and edges were felt. Its lower edge was measured to be 18cm from right subcostal margin. Liver span was 20cm. It was generalized dull percussion note and normal bowel sound heard.3 with no septations. It has a thin wall, no calcification, no solid component. There is a dilatation of intra and extra biliary tree. The liver is normal in size, shape and density. Spleen and Vascular structure appeared normal , elevated markers for the biliary obstruction gamma-glutamyltransferase and alanine aminotransferase (GGT and ALT), serum bilirubin level was elevated with more than half being conjugated bilirubin level. She had mild elevated bleeding indices and microcytic hypochromic anemia. The patient stayed in the ward for one month while the investigations were being done and received vitamin K injection, transfused two units of blood and she was given analgesics for the abdominal pain. Abdominal CT scan was done showed A well-defined hypo-attenuated non-enhancing retro-gastric cyst is noted measuring approximately (17.5*15.8*16.3) cmd normal .Percutaneous transhepatic cholangiopancreatography (PTC) was performed because the computer tomography was considered inconclusive for definitive surgical management as revealed retro-gastric cyst with differential diagnosis of choledochal cyst and pancreatic cyst. During PTC with assistance with ultrasonography, contrast injected into Gall bladder, it was seen flowing through the cystic duct and emptying into the extrahepatic huge cystic dilatation. Right intrahepatic duct was assessed, contrast was injected showed cystically dilated central right hepatic ducts, left intrahepatic ducts failed to be visualize. Diagnosis of choledochal cyst type IVA was made. Panel discussion was made with pediatric surgeons and surgical gastroenterologist, the image findings were reviewed during the discussion and conclusion for explorative laparotomy with possible cyst-enteric drainage procedure was made. Laparotomy was done, through upper abdominal transverse incision abdomen was opened in layers with aseptic techniques, huge cystic mass was seen, arising from common bile duct below the liver .Pancreas, duodenum and transverse colon adhered below the mass. Liver was hard and nodulated with rough surface. The mobilization of transverse colon from the cyst was done. Duodenum and pancreas were not mobilized due to the risk of damage to pancreatic duct. Cholecystectomy was done. Cyst was decongested and drained 2.4 liters of bile and sent for biochemistry analysis. Retro-colic cholangioenterostomy with Roue-en-Y reconstruction was made. Biopsy was taken from liver, cyst wall and gall bladder for histopathology. Abdominal lavage with warm normal saline was done, and abdomen was closed in layers. During the operation the patient received two units of blood and single unit of fresh frozen plasma, the she was sent to ICU. The patient was full recovered from ICU then sent back to the ward and discharged after two weeks continue to be followed up in the clinic.Informed consent: written informed consent was obtained from the patient's parents for publication of this case report and any accompanying images. A copy of the written consent is available for review.Choledochal cyst are rare congenital disease of the biliary tree. It presenting with vague and nonspecific features usually associated with a relatively late diagnosis. These presentations include upper abdominal pain and jaundice which are common in many other illnesses of the upper gastrointestinal tract. The classical triad of jaundice, right upper quadrant mass, and abdominal pain is present in only a minority of patients (0-17%) . SimilarAbdominal ultrasonography remains the initial imaging modality of choice in gastrointestinal tract disease presenting with biliary symptoms as it is sensitive in the detection of cystic structures but not specific in delineate the structural origin. Computed tomography (CT) is infrequently required in the situation where the distal common bile duct is not visualized due to bowel gas. Ultrasound and CT are excellent modalities for detecting cystic right upper abdominal lesions and for assessing their size and extent, but the biliary origin of the cyst may not be always reliably commented . In thisMagnetic resonance cholangiopancreatography is emerging as a highly sensitive, safe, and noninvasive diagnostic preoperative technique for the detection of choledochal cysts although has limited capacity to detect associated ductal anomalies or small choledochocele . In thisThe treatment of a choledochal cyst has changed. In the past, a cysto-jejunostomy was the standard procedure. Currently, excision of the cyst and reconstruction by hepatojejunostomy is the standard therapy . This thCholedochal cyst are rare congenital disease during childhood presenting with nonspecific sign and symptoms which associate with relative late diagnosis. Excision of the cyst and reconstruction by hepatojejunostomy as the standard therapy could be challenge due to its biliary or infection complication and hence cystenterostomy drainage procedure can be done as option for relief of patient discomfort and prevent further complications.


This study reports a case of the spontaneous avulsion of primary pterygium with anterior segment optical coherence tomography (AS-OCT) findings.A 72-year-old woman complained of acute pain of the left eye. Primary pterygia were noted in both eyes on the initial examination 4 months ago. Slit-lamp microscopy revealed a nasal corneal epithelial defect, and the rolled elevated lesion in the corneal limbus of the left eye. She was diagnosed with a spontaneous avulsion of the corneal pterygium head. Then the avulsed pterygium head slowly recurred. The pterygium head of the fellow eye had a yellow-whitish elevated lesion beneath the epithelium with poor vascularity. AS-OCT revealed hyper-reflective foci beneath the epithelium corresponding to the yellow-whitish elevated lesion.The present case revealed the spontaneous avulsion of the pterygium head leading to the corneal epithelial defects and ocular pain, while the pterygium head of the fellow eye showed subepithelial hyper-reflective foci suspicious of spheroidal degeneration on AS-OCT. In this case, the cause of spontaneous avulsion of the pterygium head might be potentially weak adhesion to the cornea due to spheroidal degeneration.This case is a primary pterygium leading to spontaneous avulsion, in which hyper-reflective foci were noted in OCT. The significant environmental risk factors of development of pterygium are long-term exposure to ultraviolet radiation from the sunlight, and to dry and dusty environments.Generally, it is unlikely that the pterygia spontaneously regress or avulse; therefore, surgical removal is required when it clinically worsens, such as visual impairment, significant discomfort, or esthetic concerns.,Previously, Cook and Dapling first presented a case of pterygium showing a spontaneous resolution by auto-avulsion in 1994.2A 72-year-old Japanese woman was referred to our department as a first visit because of a fundus examination for diabetic retinopathy. She had a medical history of diabetes, appendicitis, breast cancer, varix of the lower extremity, pancreas cancer, and bilateral leg cellulitis. The best-corrected visual acuity was 20/16 × Sph+0.50D: Cyl −1.00D Ax100° and 20/16 × Sph+0.25D: Cyl −1.25D Ax85° in the right eye OD and left eye OS, respectively. As shown, the refractive values were against-the-rule astigmatism OU. However, corneal astigmatism obtained by auto keratometer  were −0.62D Ax123°OD and −0.50D Ax60°OS, indicating oblique astigmatism OU. Intraocular pressure was normal OU. Slit-lamp examination demonstrated similar primary pterygia in the nasal corneal limbus OU. Fundus examination revealed no diabetic retinopathy OU.Four months later, she complained of acute ocular pain OS, and was referred to our department again because the pain didn't improve due to unknown causes. She had no history of prior ocular trauma. Slit-lamp microscopy revealed a round corneal epithelial defect at the nasal aspect of the cornea with positive fluorescein sodium dye staining, together with the rolled elevated lesion in the corneal limbus OS  a, b. Sh3,As shown in the present case, a spontaneous avulsion of the pterygium head seems to be a rare phenomenon in primary pterygia. To the best of our knowledge, this case is the third report of a spontaneous avulsion of the pterygium head which had no history of prior trauma or injury of the globe.Spheroidal degeneration is observed in the cornea and conjunctiva, which is especially common in people who live in areas with high ultraviolet radiation from the sunlight.The etiology of spontaneous avulsion of the pterygium is unknown. The fibrovascular pterygial tissues secrete transforming growth factor β (TGF-β), a multifunctional cytokine, leading to fibroblast activation and expression of several types of matrix metalloproteinases (MMPs). These pathologies result in dissolution of hemidesmosome attachments, Bowman's layer, and the basement membrane of the cornea.In this case, the total astigmatism had against-the-rule astigmatism rather than with-the-rule astigmatism before the spontaneous avulsion of the pterygium head. One of the reasons might have been presumably weak corneal astigmatism due to mild adhesion with the pterygial tissue and flattening of the cornea. In fact, the corneal astigmatism showed an astigmatic shift toward 90° after avulsion of the pterygium, and an astigmatic shift toward 180° in recurrence of the pterygium.In addition, the pterygium is often preceded or accompanied by a pinguecula, and a previous report has shown that spheroidal degeneration is not often associated with the pterygium, but is commonly associated with the pinguecula.Although rare, the present report has several limitations. First, the patient has received neoadjuvant chemotherapy with gemcitabine and nab-paclitaxel and postoperative adjuvant chemotherapy with S-1 because of her pancreatic head cancer. However, although these chemotherapeutic agents are broadly used worldwide, there is no previous report of pterygium avulsion caused by these agents. Therefore, it is unlikely that chemotherapy caused the pterygium avulsion. Second, we did not remove the avulsed pterygium. This is because corneal erosion was spontaneously healed after the pterygium avulsion, and the patient did not wish to receive additional treatments or any surgical interventions. For this reason, we couldn't examine histopathology to determine whether the avulsed pterygium head has a spheroidal degeneration in the present case.In conclusion, we reported a case of spontaneous avulsion of the pterygium head thought to have been complicated by spheroidal degeneration. And it is possible that the pterygium with spheroidal degeneration may be weakly adherent and easy to avulse.All listed authors meet the ICMJE criteria. We attest that all authors contributed significantly to the creation of this manuscript, each having fulfilled criteria as established by the ICMJE.The authors received no financial support for this research, authorship, and/or publication of this article.The following authors have no financial disclosure: TT, SK, and SI.Informed consent to publish an identifiable photograph was obtained from the study participant. Written consent to publish this case has been obtained.No conflict of interest exists.


We describe a case of abdominal pain in a male patient who performed dailyself-catheterization and developed a ruptured urinary bladder, which wasdiagnosed at bedside in the emergency department with point-of-careultrasound (POCUS).Ruptured urinary bladder is commonly associated with blunt abdominal trauma.It is a rare complication of Foley catheter insertion. These imagesdemonstrate that POCUS can be used as a screening tool to evaluate forbladder rupture when clinically suspected. An 83-year-old male with a history of metastatic prostate cancer requiring dailyself-catheterization presented to the emergency department (ED) complaining ofabdominal pain. His abdominal pain was mild and intermittent over the prior severaldays, without any aggravating or alleviating factors. The patient had beenself-catheterizing five times per day, without resistance or gross hematuria. Onarrival to the ED his vital signs were stable. Physical exam showed an elderlygentleman in pain with pale conjunctiva. His abdomen was soft, non-distended, andtender over the suprapubic area.To further evaluate him, a curvilinear probe was used to perform point-of-care renaland bladder ultrasound. Bladder images showed a loculated fluid collection in thesuperior and posterior aspect of the bladder and fluid outside the wall of thebladder, as well as hyperechoic dependent densities within the bladder lumen  and 2.The patient was found to be anemic with a hemoglobin of 5.5 grams per deciliter(g/dL) (reference range: 14.0–18.0 g/dL). His renal function was slightlyworse than his baseline with a blood urea nitrogen of 39 milligrams/dL(11–23 mg/dL), and creatinine of 1.50 mg/dL (0.7–1.5 mg/dL). Hisurinalysis was positive for leukocyte esterase, nitrites, and moderate red bloodcells. After placing two large-bore intravenous lines and initiating a bloodtransfusion, a computed tomography (CT) of the abdomen and pelvis was obtained,which showed fluid-filled loculation of the posterior aspect of the bladdermeasuring 9.5 centimeters, suspicious for a partially contained bladder rupture.Urology was consulted and obtained CT cystogram, which demonstrated a partiallycontained bladder rupture, without extension of bladder contents into the peritoneum. UrologyBladder rupture is commonly associated with blunt abdominal trauma or surgicalcomplication.What do we already know about this clinical entity?Bladder injuries are commonly associated with blunt abdominal injury.There are only few cases that reported bladder injuries withself-catheterization.What is the major impact of the image(s)?These images show that performing a point-of-care (POC) ultrasound caneasily detect bladder injuries in the right clinical settings.How might this improve emergency medicine practice?POC ultrasound can expedite the diagnosis of bladderrupture.Bladder rupture is recognized as an irregular-shaped bladder with free fluid adjacentto the bladder, or fluid in the right or left upper abdominal quadrants. The patientin the case had been self-catheterizing up to five times per day, increasing hisrisk of iatrogenic bladder injury. Clinicians should have a high yield of suspicionof bladder rupture in cases of self-catheterization. When used in the right contextPOCUS is a useful bedside tool to help clinicians diagnose this condition.


The liver is the organ most commonly injured in blunt abdominal trauma. Significant changes in the management of liver trauma have occurred over the last four decades with non-operative management being the first-line of treatment. Although hepatic resection for trauma is an accepted and established option for definitive treatment, it is rarely performed because of the associated morbidity and mortality, at least historically. Herein we describe a case of a 24-year old male who had blunt abdominal injury for which a right hepatectomy was eventually performed after an initial attempt at damage control surgery. We would like to highlight that early decision by a dedicated team of surgeons coupled with the necessary support from ancillary services as well as coordination between trauma surgeons led to a successful outcome in this case. This case presents an opportunity to revisit the role of hepatic resection in the management of complex liver injuries. •Liver is a frequently injured organ in blunt or penetrating injury.•Non-operative management can be successful in majority of cases.•Nonanatomic or anatomic hepatic resection can be a treatment option in select cases.•Timely decision is paramount in the success of operative management for blunt trauma. Hepatic resection for trauma is rare and has historically been associated with a high mortality and has thus consigned this procedure, for the most part, into the recycling bin of surgical techniques. Herein we describe a case managed at a high-volume academic trauma center for which a selective operative approach was undertaken exemplifying that hepatic resection may be reserved for those instances where bleeding cannot be arrested by packing or damage control surgery. The case presents an opportunity to revisit the role of hepatic resection in the management of complex liver injuries. This report has been written in line with the recent SCARE criteria for case reports .2A 24-year-old male with no known comorbidities figured in a motorcycle crash in an island resort 1400 km away. He was initially managed at a local hospital wherein they performed damage control surgery  before being airlifted to our institution. Initial FAST (Focused Assessment with Sonography in Trauma) was positive for fluid at the hepatorenal space while an abdominal CT scan done prior revealed liver parenchymal laceration in segments V, VI and VIII (AAST (American Association for the Surgery of Trauma) Grade IV) and right renal laceration (AAST Grade III) with hemoperitoneum A and B. 3The management of blunt liver injuries have evolved over the last four decades. Early experience with hepatic resection for trauma yielded high mortality rates (>50%) which led to the procedure being deprecated. The reemergence of perihepatic packing and influx of modern diagnostic and therapeutic tools ushered in a new option of non-operative management (NOM) which can be successfully applied in >80% of cases . The imp–V liver injuries, NOM should be considered only in selected settings provided there are available trained surgeons, operating room, intensive care unit (ICU), access to angiography, blood products, and in locations where a system exists to quickly transfer such patients to higher level of care facilities. Our patient was airlifted from an island as the previous institution had limited angiogram and ICU facility, lacked a complement of specialty surgeons as well as ancillary services. Coordination between the two hospitals and airlift availability led also to the delay in transport which, when taken all together, may have contributed to suboptimal surgical management. Within 48 hours upon arrival at our institution, while in the ICU, hemodynamic instability led to the shift of approach to operative management. An anatomic resection (right hepatectomy) was performed by a team of trauma, hepatobiliary and liver transplant surgeons. There were significant necrotic tissues in the involved liver segments and most bleeding came from its parenchyma.Recent guidelines recommend NOM as the treatment of choice for all hemodynamically stable liver trauma patients regardless of AAST grade in the absence of other internal injuries requiring surgery . In patiOver the years, with the improvement of our understanding on liver anatomy and physiology, advances in critical care and the advent of liver transplant teams, hepatic resections have become safe in many centers worldwide. Although operative mortalities for high-grade liver injuries remain high in the literature, several reports have concluded otherwise especially in centers who have more experienced liver and transplant surgeons. Strong et al. reported mortality rates after hepatic resection of 11.1% with liver-related morbidity of 19% and emphasized the use of simple methods to successfully treat injuries while resection, if needed, be performed by a dedicated liver team . TsugawaSeveral risk factors have been associated with non-survival with the grade of liver injury being a strong predictor of mortality. Doklestic et al. studied 121 hepatic trauma patients with AAST Grade III-IV injuries and showed that non-survivors had higher grade of injury, higher liver enzyme level, significant hypotension, higher Injury Severity Score (ISS) score and lower Glasgow Coma Scale (GCS) on arrival . They al. However, if persistent bleeding is encountered, a timely decision must be made to proceed with hepatic resection. As demonstrated in the case, a right hepatectomy was the most effective way of controlling bleeding. The initial management, albeit suboptimal, age and minimal transfusion requirements may have led the patient to endure such major hepatic resection. The resuscitative efforts in the ICU perioperatively and the assistance of the anesthesiologists were also crucial in avoiding the triad of massive blood loss, hypothermia and acidosis leading to irreversible coagulopathy.The general approach to hepatic injury at our center is to attempt non-operative management for blunt injuries regardless of grade of injury. We utilize FAST to evaluate presence of intraabdominal fluid and proceed with diagnostic peritoneal lavage (DPL) assessing for hollow viscus injury . Hemodynamic instability, presence of peritoneal signs or a positive DPL would prompt a shift in management to operative intervention. Recently, we have expanded our armamentarium and involved interventional radiologists in selected cases of complex injuries. If the patient requires laparotomy for liver injury, packing is initially attempted and if it controls bleeding and the patient is still unstable, a damage control approach is takenIn conclusion, an anatomic or non-anatomic hepatic resection is a viable and safe option for traumatic and complex liver injuries. Early decision by a dedicated team of surgeons coupled with the necessary support from ancillary services is essential for an optimal outcome in these cases.Not commissioned, externally peer-reviewed.Yes, ethics approval was acquired thru the University of the Philippines Manila Research Grants Administration Office with reference number RGAO-2021-1409.None.Yes. Consent was obtained from the patient.SP – writing the paper, final editing.EA – data collection, study design, manuscript editing.AC – data collection, review of literature, writing the paper.JM – data collection, review of literature, final draft.OO – writing the paper, final draft.1. Name of the registry: N/A.2. Unique Identifying number or registration ID: N/A.3. Hyperlink to your specific registration (must be publicly accessible and will be checked): N/A.Siegfredo R. Paloyo, MD, MPH.None.


Introduction: Non-Hodgkin lymphomas (NHL) comprise 85% of the total lymphomas diagnosed, with the histological type of diffuse large B-cell lymphomas (DLBCL) being the most prevalent in adults. In about 40% of cases, the location is extranodal. Uterine cervix lymphomas of this type are extremely rare (0.5–1.5%) and represent a diagnostic challenge. A case of DLBCL of the cervix is presented here along with a review of the literature. Materials and methods: A 75-year-old patient was referred with a bleeding vegetant tumour occupying her entire vagina. The histological and pathological investigations performed following the tumour biopsy indicated a malignant, diffuse, vaguely nodular lymphoid tumour proliferation. The immunohistochemistry results were in favour of a diffuse B-cell non-Hodgkin lymphoma (DLBCL). CHOP , Oncovin (vincristine), Prednisone or Prednisolone) polychemotherapy and radiotherapy were effective and resulted in tumour regression . Conclusions: The uterine cervix lymphoma prognosis is more conservative than that for a nodal lymphoma, mainly due to a later diagnosis determined via immunohistochemistry. Chemotherapy is the main treatment. Diffuse large B-cell lymphomas (DLBCL) are the lymphoid neoplasms most frequently diagnosed in adults (about 40%) every year .Approximately 40% of diffuse large B-cell lymphomas are extranodal; the gastrointestinal tract (15%), bone, and central nervous system are the most common sites involved ,3. UteriThe clinical symptoms of uterine cervix lymphoma are nonspecific to the histological type and may include vaginal bleeding, vaginal tumour, dyspareunia, and pelvic pain ,3.We report a case of primary cervix DLBCL in an elderly female, discuss the related diagnostic work up, and compare our results with those reported in the literature.A 75-year-old patient was referred to our clinic in 2018 with significant vaginal bleeding and a diagnosis of cervical tumour. The gynaecological exam described the presence of a bleeding vegetant tumour occupying the entire vagina up to the introitus; it was hard to delineate the uterus, which had infiltrated perimeters, with the left one next to the bone wall. Anaemia (haemoglobin level of 9.47 g/dL) and leucocytosis  were also present. A cervical biopsy was performed, and diffuse malignant lymphoid tumour proliferation was detected: vaguely nodular, with large–medium polymorphous cells. An immunohistochemistry (IHC) study was afterwards performed, and a histological diagnosis of non-Hodgkin large B-cell malignant lymphoma (DLBCL) NOS (Not Otherwise Specified), non-GC  , CD10 negative, BCL6 positive, Mum1 positive, p16 positive, CD5 negative, Ki67: 50% in atypical large cells. The patient was subsequently referred to the haematologist and a bone marrow biopsy was performed; no evidence of marrow involvement was found after IHC studies. All procedures performed were in accordance with the ethical standards of the 1964 Helsinki Declaration and its later amendments.Whole-body imaging with CT (Computed Tomography) scan detected no secondary lesions in the solid organs or in the lymph nodes except for inguinal bilateral adenopathies less than 1 centimetre in diameter. The primary tumour was represented by an intensely iodophilic, dense vaginal mass of 3.4/2.9 cm with no changes of the uterus or the uterine adnexa. The MRI (Magnetic resonance imaging) scan confirmed the primary cervical tumour .2 at day 1, vincristine 2 mg/m2 at day 1, cyclophosphamide 600 mg/m2 at day 1, and dexamethasone 16 mg/m2 from day 1 to day 5. Treatment was given every 21 days [2) [The diagnosis of stage IE DLBCL of the cervix was established and the patient was referred to the haematology department, where the decision was made to treat her with the standard dose of CHOP  every 3 weeks for six cycles. CHOP chemotherapy treatment was decided via a multidisciplinary consultation: doxorubicin 50 mg/mTwo contrast-enhanced chest/abdomen/pelvis CT scans were performed in 2020 (at 12 and 18 months after radiotherapy): the cervix showed an apparently homogenous structure with no regional adenopathies and again no secondary lesions were detected . The vagUterine cervix lymphomas account for 0.008% of malignant cervical tumours . CervicaThe case presented herein is an Ann Arbor stage IE ,7. StudiA PUBMED literature search for papers published within the past 5 years 2016–2020) identified 14 more cases of primary uterine cervix lymphomas . The pat6–2020 idThe immunohistochemistry markers subclassified DLBCL into two subtypes: germinal centre cell lymphoma (GC-B), with CD5-; CD10+; BCL6+ or CD5-; CD10-; BCL6+, IRF4/MUM1-; and the non-GC-B subtype: CD5-; CD10-; BCL6+, IRF4/MUM1+ (1).These studies also highlight the importance of immunohistochemistry (IHC) as the most important diagnostic tool in the diagnosis and differential diagnosis of primary malignant cervical lymphomas. Currently, there is no IHC marker standard for this diagnosis. LCA, vimentin, CD20, CD30, Bcl6, and Bcl2 can be used, as reported in ,9. The dAs this is a rare disease, there is no consensus regarding the best treatment. Lymphomas are considered chemotherapy- and radiotherapy-sensitive  and, thePET-CT is credited as a highly positive and specific method for lymphoma stage determination . From anExtranodal genital and cervical MNHLs are very rare, and often associated with late diagnosis. Combining histological and pathological investigation and immunohistochemistry leads to a precise cervical MNHL diagnosis and contributes to a differential diagnosis against other genital tumours. Imaging scans are important in diagnosing and monitoring this pathology .The main treatment is chemotherapy, with radiotherapy as an adjuvant. If found early and treated, the prognosis for this disease is very good, with an 80% 5-year survival rate.


Fibro osseous lesions of the craniofacial skeleton are a benign condition in which the normal architecture of the bone is replaced by fibrous connective tissue with varying degrees of mineralization. JOF forms a special entity among the fibro osseous lesions because of its age of occurrence and its aggressive nature thereby mimicking a malignancy. The Juvenile Ossifying Fibromas were further subdivided into Psammomatoid and Trabecular variant based on their histopathological characteristics. They tend to differ in their mineralized portion with the trabecular variant showing woven bone while the psammamotoid shows lamellated and spherical ossicles in various shapes in a myxoid stroma intermingled with bone cyst like areas. The reported cases of JPSOF are few, hence histopathological examination is a valuable tool in the diagnosis of this rare lesion and JOF should always be considered in the differential diagnosis of the lesions of the craniofacial skeleton. Early diagnosis and a complete surgical excision with adequate margins and a long term follow up is mandatory for a good prognosis of this highly recurrent and aggressive lesion.The diagnosis of JOF requires a careful correlation of clinical, radiological and histopathological features. The purpose of this paper is to report a case of JPOF of the orbit to stress the need for consideration of JOF in the differential diagnosis of the aggressive lesions of the Cranio facial skeleton. Key words:Juvenile psammomatoid ossifying fibroma, orbit, ossifying fibroma. Fibro osseous lesions of the craniofacial skeleton are a benign condition in which the normal architecture of the bone is replaced by fibrous connective tissue with varying degrees of mineralization. They are broadly classified as cemento-osseous dysplasia,Ossifying fibroma and Fibrous Dysplasia. Ossifying fibromas are in turn further divided into Conventional(Cemento-Ossifying fibroma) and Juvenile Ossifying Fibroma( JOF) .The term Juvenile active Ossifying fibroma was first coined by Golg  in 1949.The Juvenile Ossifying Fibromas were further subdivided into Psammomatoid and Trabecular variant based on their histopathological characteristics according to the WHO classification of odontogenic tumors 2005 . Histoloet al.  of size 2×2cm involving the infraorbital rim not involving the maxillary sinus or the nasal septum Fig. c-e. A prThe surgical procedure was done under general anaesthesia using naso endotracheal intubation. A infraorbital incision was given, periosteum was incised and the lesion was identified and excised and peripheral ostectomy was done Fig. a,b. The Juvenile aggressive ossifying fibroma is a benign fibro osseous lesions of jaws seen occurring predominantly in children and adolscents. According to WHO it is describred as a lesion affecting individuals below the age of fifteen . JuvenilEl- Mofty  reportedet al.  presents a major diagnostic challenge in cases of JPSOF in paranasal sinuses because the clinical and radiographic features are very similar . They caThe management of JOF ranges from complete surgical resection ,enucleation and curettage and enucleation with peripheral ostectomy . SmallerThe recurrence rate of JPOF is reported to be 30% to 56% . The comJuvenile Ossifying fibroma is a benign fibro osseous lesion with a high recurrence rate and aggressive behavior clinically mimicking a malignancy. Hence a proper diagnosis based on a complete clinical,radiological and histopathological examination is warranted to avoid a misdiagnosis and for executing the correct treatment modality. The reported cases of JPSOF are few, hence histopathological examination is a valuable tool in the diagnosis of this rare lesion and JOF should always be considered in the differential diagnosis of the lesions of the craniofacial skeleton. Early diagnosis and a complete surgical excision with adequate margins and a long term follow up is mandatory for a good prognosis of this highly recurrent and aggressive lesion.


Volume restoration is often required after parotidectomy due to the resultant facial contour deformity. Common procedures include local pedicled flaps, such as the sternocleidomastoid muscle flap, fat grafting, and even autologous free flaps, for more extensive defects. Local pedicled flaps have the advantage of a single surgical site, which spares the patient the added morbidity of a separate fat graft donor site, while simultaneously reducing the operative time. We report two cases of a novel reconstructive option using pedicled level I and II cervical lymphoadipose tissue for volume restoration after superficial parotidectomy. This reconstruction would be useful for patients with benign parotid lesions and inferior parotid defects. In addition, with maintained blood supply to this tissue, it would likely provide sustained bulk over time. The most common site for salivary gland tumors is the parotid gland, with surgical resection being the standard option in treatment . A majorIt is well known that grafts exhibit volume loss over time, with both muscular and fatty tissue atrophy , 4, but An 80-year-old Caucasian male presented to our clinic for evaluation and management of a right parotid tail mass that had been present for several years and had significantly increased in size over the past two months. Computed tomography (CT) revealed a 3.4 × 2.7 cm mass, and subsequent ultrasound guided core biopsy was consistent with a low-grade oncocytic neoplasm. On surgical evaluation, the tumor was found to be superficial to the facial nerve branches, so a superficial parotidectomy was performed with dissection and preservation of the facial nerve. Intraoperative frozen section analysis was performed confirming benign pathology, and final pathology would confirm an oncocytoma, measuring 4.8 × 3.7 by 2.3 cm.After resection, there was a considerable mass defect that required volume reconstruction. Once frozen section pathology confirmed a benign tumor, the mass defect was reconstructed using a level I and II lymphofatty packet of tissue mobilized off the posterior aspect of the submandibular gland and SCM, advanced posteriorly and superiorly, and secured to the parotid fascia.A 58-year-old woman with a five-year history of a right-sided mass at the angle of the mandible sought evaluation after noting a recent increase in size with discoloration of the overlying skin. CT scan of the neck showed a mass measuring 3.2 × 2.9 cm involving the superficial lobe of the right parotid gland. Fine needle aspiration findings were consistent with a cystic lesion. On surgical evaluation, the tumor was found to be partially superficial and deep to the facial nerve branches. These were identified, dissected, and preserved while resecting the mass. Frozen section analysis demonstrated a papillary cystadenoma lymphomatosum tumor with cystic degeneration. Final pathology would confirm the frozen section diagnosis. The mass defect was reconstructed using a level I and II lymphofatty packet of tissue mobilized off the posterior aspect of the submandibular gland and SCM, advanced posteriorly and superiorly, and secured to the parotid fascia.Surgical technique for volume reconstruction was performed similarly for both cases . The lymAfter superficial or total parotidectomy, there is often a significant defect that requires volume reconstruction. There are several common locoregional options for relatively small defects including acellular dermis, sternocleidomastoid (SCM) muscle flap, and local fascia flaps . Other rThe use of pedicled cervical lymphoadipose tissues added minimal operative time at a single operative site and can be performed with no additional incisions. Furthermore, it has limited associated morbidity compared to SCM muscle flaps which can require harvesting of a considerable portion muscle. Skeletal muscle flaps also tend to atrophy over time which may lead to variability in results . This rePotential limitations include variations in the amount of cervical lymphoadipose tissue and the location and size of the defect. The volume that can be replaced by this reconstructive method is dictated by patient anatomy and amount of tissue that is available. This limitation is mitigated by the advantage of this being a vascularized flap undergoing less atrophy in comparison with nonvascularized grafts as discussed above. Liang et al. found that vascular anatomy of the SMG flap prevented the coverage of the superoposterior aspect of the defect in some cases, which would also likely be the case when using pedicled lymphoadipose tissue. As in our cases, pedicled lymphoadipose tissue would be useful to cover inferior defects and could be used in conjunction with another reconstructive modality to provide optimal volume reconstruction. Again, this reconstructive technique should only be considered for benign parotid lesions, and we recommend frozen section analysis of the parotid tumor and any suspicious lymph nodes prior to reconstruction.Continued utilization of this novel reconstructive option will allow for delineation comparison of long-term outcomes, complications, and patient satisfaction to more established reconstructive methods.


Gastrointestinal bleeding caused by gastrointestinal tract involvement in patients with Klippel–Trenaunay syndrome (KTS) is extremely rare and often overlooked.Case presentationA 9-year-old girl who presented with chronic gastrointestinal bleeding was admitted to our hospital. Laboratory examinations revealed microcytic hypochromic anemia and a positive fecal occult blood test. Computed tomography (CT) of the lower limbs combined with physical examination confirmed KTS. The pelvic CT showed concentric thickening of the sigmoid colon and rectum, with progressive enhancement after the administration of a contrast agent. Colonoscopy demonstrated vascular malformations of the sigmoid colon and rectum. The patient was finally diagnosed with KTS with gastrointestinal tract involvement. The patient improved after receiving conservative treatment.A suspicion of gastrointestinal tract involvement as an etiology for gastrointestinal bleeding should not be overlooked in patients with KTS. Endoscopy and imaging modalities can synergistically help diagnose this condition. Klippel–Trenaunay syndrome (KTS) is a congenital and rare vascular malformation disorder that mainly involves the lower limbs. It is characterized by capillary, lymphatic and/or venous malformations and overgrowth of soft tissue and/or bone . GastroiA 9-year-old girl with an 8-year history of chronic gastrointestinal bleeding was admitted to our hospital. The patient had no corresponding family history. Sclerotherapy in the varicose veins of her right lower limb was previously performed in another hospital. Physical examination showed a hypertrophic and longer right lower limb with vascular nevus. Blood was not observed during the digital rectal examination. Laboratory examinations revealed microcytic hypochromic anemia with a hemoglobin level of 7.3 g/dL  and a positive fecal occult blood test.Pelvic CT showed concentric thickening of the sigmoid colon and rectum, with a thickness of 7.8 mm Fig. A. The thColonoscopy revealed patchy, branch-like and erythematous almost submucosal appearing lesions affecting the distal sigmoid colon and rectum Fig. E–G, and KTS is a disorder combining vascular malformations and an affected overgrowth of a limb. It is believed to be caused by somatic mosaicism in the phosphoinositide 3-kinase/PIK3CA signaling pathway . Common Gastrointestinal tract involvement accounts for approximately 20% of KTS cases . The sigIn conclusion, gastrointestinal tract involvement should be considered an alternative etiology for unexplained gastrointestinal bleeding in patients with KTS. Imaging provides a non-invasive modality to identify lesion extent, especially if these lesions are located in the small intestine. Critically, using CT and contrast agents, marked thickening of the affected bowel and progressive enhancement can be observed. Endoscopy facilitates direct observation of the mucosa, helps determine the nature of the lesion and identifies the bleeding site. Thus, the synergistic combination of imaging examinations and endoscopy can help diagnose this condition. Treatment modalities depend on the extent of lesions, and patient symptoms.


Pseudomyxoma peritonei (PMP) is any mucin-producing tumor in the abdomen. If left untreated, it can compress vital abdominal organs. We describe a misleading presentation of disseminated peritoneal adenomucinosis (DPAM) of appendiceal origin. Treatment strategies for PMP vary from watchful waiting to cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) or early postoperative intraperitoneal chemotherapy (EPIC). The rarity of PMP has precluded any randomized studies, and few institutions see sufficient cases to report a series. The purpose of this case report is to contribute to reaching a common consensus on the guidelines of pseudomyxoma peritonei. Recent studies support that cytoreduction followed by HIPEC improves survival in patients with peritoneal carcinomatosis of colorectal origin. Our case report introduced a patient with low-grade appendiceal mucinous neoplasm with visceral peritoneal penetration who underwent surgical debulking with promising results that supports the practice. Our patient has also responded well to adjuvant systemic chemotherapy without hyperthermic intraperitoneal chemotherapy.  Pseudomyxoma peritonei (PMP) is an uncommon disease known for mucin secreting cells seeding the peritoneum, which if left untreated, can compress vital abdominal organs . The terA 62-year-old female patient with a past medical history significant for type 2 diabetes mellitus, obesity, essential hypertension, and a left breast multifocal high-grade ductal carcinoma in situ  status post simple left mastectomy and sentinel lymph node (LN) biopsy with no evidence of micro-invasions and free lymph nodes, followed by six cycles of 5-fluorouracil, doxorubicin, and cyclophosphamide. During her follow-up, she complained of abdominal enlargement and generalized tenderness. Abdominal ultrasonography reported minimal ascites, enlarged early cirrhotic liver, and enlarged spleen. An MRI of the abdomen was significant for nodular stranding of the omentum, nodular and sheet-like lesions with an abnormal signal intensity related to the posterior aspect of the anterior abdominal wall (maximum thickness of 4 cm), suggesting omental caking Figure . SerologPMP refers to a clinical syndrome of diffuse mucinous peritoneal involvement, often associated with a primary appendiceal lesion, but includes peritoneal dissemination of a wide variety of mucin-producing invasive adenocarcinomas of the appendix, large and small bowel, lung, breast, pancreas, stomach, bile ducts, gallbladder, and fallopian tubes/ovary. PMP is not a histologically based term and is not used for staging or histologic classification of appendiceal neoplasms -4. A defAppendiceal adenocarcinomas have three separate histologic types ,5. MucinPeritoneal mucinous carcinomatosis (PMCA), due to its rarity and often delayed presentation, is commonly misdiagnosed at first. There is no consensus regarding the proper management of aggressive cases. Low incidence of this disease has precluded the performance of randomized studies, and few institutions see sufficient numbers to report a series of homogeneously treated patients. Treatment recommendations and guidelines from expert groups remain wanting. A role for adjuvant chemotherapy for adenocarcinoma of the appendix has not been definitively established. Cytoreductive surgery in combination with hyperthermic intraperitoneal chemotherapy could be appropriate for aggressive PMCA.


Background: Brown tumor is a rare skeletal manifestation of secondary hyperparathyroidism. Although diagnosis of the disease is increasingly seen in early stages due to improved screening techniques, some patients still present in a progressed disease stage. The treatment depends on tumor mass and varies from a conservative approach with supportive parathyroidectomy to extensive surgical resection with subsequent reconstruction. Case presentation: We report a case of extensive mandibular brown tumor in a patient with a history of systemic lupus erythematosus, chronic kidney disease, and secondary hyperparathyroidism. Following radical resection of the affected bone, reconstruction could be successfully performed using a free flap. Conclusions: There were no signs of recurrence during five years of close follow-up. Increased awareness and multidisciplinary follow-ups could allow early diagnosis and prevent the need for radical therapeutical approaches. In rare cases, such a disturbance in bone turnover can result in skeletal manifestations of HPT. Brown tumor is one of the skeletal manifestations of HPT. The lesion is characterized by non-neoplastic reactive tissue, extensive osteoclastic bone resorption with osteoclast-like multinucleated giant cells, osseous microfractures, and hemorrhage as well as hemosiderin depositions. The term “pearance .Incidence of primary hyperparathyroidism (PHPT) varies and ranges between 0.4–21.6 cases per 100,000 person-years . AlthougIn recent years, thanks to improved screening techniques and more frequent early detection of hyperparathyroidism, brown tumors have become a rarity. The general prevalence of brown tumors is low (approximately 0.1%); however, it is increasingly found in PHPT and SHPT patients . The patHere, we report a rare case of an extensive brown tumor with manifestation in the mandible of a patient with secondary HPT, who also had systemic lupus erythematosus and a history of chronic renal failure. Further, we provide a review of the available relevant evidence, including other case reports published to date.A 41-year-old female presented to our hospital with suspicious swelling of the lower jaw. She reported that the swelling on the right side of her face had slowly increased in size over the past 4–6 weeks. An intraoral examination revealed a hard swelling that extended from the right premolars to the ascending ramus. The buccal mucosa and lingual mandibular plates were expanded, leading to a narrowed buccal sulcus. Tooth 45 was loose and showed attenuated pulp sensory responses upon thermal stimulation.A panoramic X-ray revealed an osteolytic lesion, partial loss of cortical bone, and early root resorption of teeth 45 and 46 .A CT scan of the head and neck region was performed to determine the anterior-posterior expansion of the lesion and carry out virtual planning of reconstruction. The lesion involved the body of the right mandible with caudal displacement of the mandibular canal. The resulting subtotal bony destruction involved both buccal and lingual cortical bone and measured approximately 38 × 17 × 26 mm . No lympThe patient had a history of systemic lupus erythematosus and suffered for approximately 10 years from chronic kidney disease and osteoporosis. She had no family history of hypocalcemia or endocrine disorders and received regular nephrology screening. Associated high blood pressure was controlled with an angiotensin-converting enzyme inhibitor to slow progression of renal failure. An elevated parathyroid hormone (PTH) level of 200.8 pmol/L was measured at the time of presentation . The biochemical preoperative investigations showed hypocalcemia , Vitamin D levels of 20 µg/L , creatinine at 3 mg/dL , and calcitonin levels of 11 pg/mL  .The initial clinical diagnosis was a giant cell lesion, based on the clinical examination and considering the localization in the right mandible, tumorous swelling, a lack of localized increased temperature, and normal coloration. Clinical differential diagnosis included ameloblastoma, SHPT brown tumor, and aneurysmal bone cyst. Thus, an incisional biopsy was taken. Microscopically, the lesion consisted of plump fibroblasts and numerous osteoclast-like giant cells surrounded by reactive bone formation . FurtherBecause of the extent of the tumor and the resulting functional problems for the patient, extensive surgical removal was planned. Notably, a limited amount of remaining intact bone was present in the mandibular region. Therapeutic options were discussed among the patient and nephrologist, with the conclusion that surgery was most appropriate, owing to the patient’s young age and her stable renal function.Radical resection with immediate reconstruction with a deep circumflex iliac artery (DCIA) flap was performed. Virtual 3D planning was carried out to better plan the resection and reconstruction and ensure a short operation time .The DCIA was harvested in standard fashion following the method originally described by Taylor et al. . HypotenAfter isolating the bone flap, the pedicle was prepared and divided after the recipient area was ready. A suction drain was placed in the donor area and wound closure was performed in multiple layers. Bupivacaine infiltration (AstraZeneca) was provided at the donor site to minimize postoperative pain. After wound closure, Steri-Strips  and Cosmopor E  were used to cover the wound and a. pressure bandage was applied. The postoperative status was satisfying. After 7–10 days, the nephrological situation improved to preoperative levels after initial temporary acute kidney injury due to tubular necrosis. The intraoral wound healed with no signs of infection. The donor site morbidity was examined preoperatively and during the perioperative period with a specific orthopedic IOWA Hip-Score . OverallClinical examination, including patient nephrological status, was carried out immediately post-operation, and five years of regular follow-ups revealed satisfying results with no evidence of recurrence. A post-operative cone-beam computed tomography scan with a 3D reconstruction of the patient showed no signs of tumor leftovers. The follow-up intervention one year postoperatively included a successful implant-prosthetic rehabilitation of the affected area .A review of the literature identified a total of 23 patients with secondary HPT resulting in brown tumors of the facial bones . OverallCountries reporting cases of SHPT were Serbia, Greece, Spain, Brazil, India, Bulgaria, Iran, USA, Turkey, Belgium, Indonesia, and China. Most reports of brown tumors associated with secondary hyperparathyroidism came from India. Of the 23 patients identified, 16 were females, and 7 were males (2.3:1). The mean age was 36.3 years, ranging from 12 to 70 years. Overall, 16/25 (64%) brown tumor lesions occurred in the mandible, whereas 9/25 (36%) were found in the maxilla. Reported tumor sizes ranged between 2 × 2 cm to 6 × 7 cm. The majority of patients were treated surgically. One case was successfully treated conservatively with 60,000 IU Vitamin D3 once a week and calcium (500 mg) twice daily . Follow-Secondary HPT is most often a result of chronic renal disease with decreased calcium levels and increased PTH secretion. Considering the increasing availability of hemodialysis and diagnostic capabilities, prevalence may increase in the future . PathogeOsteitis fibrosa cystica, often used as a synonym for brown tumors, is a rare skeletal manifestation and was first described by Recklinghausen in 1891 . As a reBrown tumors are reported to be more common in the mandibles of young female patients, with the highest incidence between the third and sixth decades ,23. ThisTreatment of brown tumors depends on the extent of the tumor mass and associated functional problems. Close control and adjustment of calcium, phosphate, and 1,25-dihydroxy Vitamin D blood levels in patients with chronic renal disease are crucial. The first steps in therapy include normalization of calcium and phosphate levels and thus a multimodal therapeutic approach. Administration of calcitriol or Vitamin D receptor activators (VDRAs) is often sufficient to control SHPT and attenuate tumor mass growth . UnrespoSurgical removal of the tumoral masses may be necessary for patients with inadequate response to conservative therapy and parathyroidectomy. This is often required due to esthetic complaints and functional problems affecting chewing and swallowing . FurtherDue to subtotal local destruction of the mandible and high risk of local complications, such as pathological fracture, radical surgical excision of the brown tumor was undertaken in our patient. In such cases requiring extensive resections, surgical reconstruction can be challenging due to possible complications associated with prolonged general anesthesia in patients with chronic renal diseases. These complications include an elevation of blood urea level, creatinine, decreased erythropoietin production, and reduction of acid, salt, and water excretion, promoting anemia, acidosis, hyperkalemia, hypertension, edema, and platelet dysfunction with resulting bleeding.Successful reconstructive strategies should aim to optimize functional and esthetic outcomes while reducing donor site morbidity. Furthermore, reconstruction of the jaw should allow future dental rehabilitation to maintain the full function of the masticatory system. The type of defect, along with the patient characteristics, are generally the main factors in making the choice of therapeutic approach. In contrast to historical strategies of mandibular reconstruction, focusing on a staged or delayed approached to account for the recurrence of disease, today’s strategies are increasingly aimed at one-stage therapy with immediate reconstruction . ImmediaVascularized bone grafts are required for large defects with soft tissue loss when healing independent of an available recipient bed is necessary. We aimed for a reliable and long-lasting reconstruction of large bone loss and face shape, creating a surface for dental restorations and providing a stable wound that would not result in an orocutaneous fistula. Mandibular reconstruction with vascularized flaps is associated with early bone union and less resorption. Further, it provides a generous amount of tissue compared to reconstruction with metal plates or non-vascularized bone grafts. Microvascular free flaps are technically challenging but are a reliable and stable therapeutic option with good outcomes even for large defects involving the anterior mandible. Vascularized free flaps include fibular free flaps, radial forearm free flaps, scapula free flaps, anterior thigh flap, pectoralis major myocutaneous flap, metatarsus osteocutaneous flap, metatarsus osteocutanous flap, and the DCIA flap.Notably, the present case was the first with a brown tumor associated with secondary hyperparathyroidism to be treated by 3D planning with a subsequent microvascular reconstruction of the jaw for functional rehabilitation. The iliac crest bone is an excellent option for reconstructing the mandible, as its cancellous bone structure allows future dental rehabilitation with dental implants. Furthermore, the shape reconstruction of the mandible, including the anterior mandible, can be effectively planned with 3D planning techniques using the iliac crest due to their similarity in shape. Another advantage of DCIA flaps is the simultaneous reconstruction of intraoral mucosal defects caused by radical resection using the internal oblique muscle, which is more reliable than skin flaps . One oftConsidering the young age of our patient and relatively stable nephrological status, we decided to reconstruct the large mandible defect with a DCIA flap as a one-stage procedure. The iliac crest free flap provided a sufficient amount of cortical and cancellous substance to reconstruct the height of the native mandible. Further, it provided the basis for successful dental rehabilitation with implants.Brown tumors are rare osteolytic bone lesions that may occur in patients with primary, secondary, or tertiary hyperparathyroidism. Surgical intervention may be required in cases with extensive lesions. We found no reported recurrences after surgical resection in the cases described in the literature to date. Radical resection and subsequent reconstruction with a microvascular iliac crest graft were successfully performed in our patient presenting with a large progressed mandibular brown tumor. We found no signs of recurrence during five years of close follow-up. Increased awareness among physicians and multidisciplinary follow-ups could allow early diagnosis and prevent radical therapeutical approaches in patients with hyperparathyroidism.


Mediastinal paragangliomas are rare neuroendocrine tumors that originate from extra-adrenal paraganglia, occasionally secreting catecholamines. Nonfunctional mediastinal paragangliomas present nonspecific clinical and radiological features and represent a diagnostic challenge.A 53-year old woman presented with cough and dyspnea increasing over time. CT-scan and ultrasonography showed a large vascularized cervico-mediastinal mass, consistent with an intrathoracic ectopic goiter. Preoperative angiography showed a blood supply from neck vessels. The lesion was completely removed through a cervical approach. The diagnosis of paraganglioma was a histological surprise. The patient is alive without recurrence 30 months after surgery.123I-metaiodobenzylguanidine can be an useful diagnostic tool when a paraganglioma is suspected. In case of vascularized cervico-mediastinal mass, such as paragangliomas or intrathoracic goiter, preoperative angiography should be performed to study the blood supply and orient the surgical approach.When preoperatively diagnosed, the treatment of choice of a mediastinal paraganglioma is surgical excision. However, a preoperative diagnosis of mediastinal paraganglioma is difficult to obtain, especially in cases of nonfunctional lesions. Distinction between an intrathoracic goiter and a nonfunctional paraganglioma can be extremely difficult and, given the rarity of the latter, an ectopic goiter is suspected in first instance. CT-scan and ultrasonography are of little use in the differential diagnosis. However, scintigraphy with Although uncommon, paragangliomas should be considered in the differential diagnosis of mediastinal masses, especially when an ectopic goiter is suspected. •Clinical diagnosis of mediastinal nonfunctioning paragangliomas is challenging.•Mediastinal paragangliomas can mimic intrathoracic goiters.•123I-MIBG can be useful.If a clinical suspicion of paraganglioma is raised, •In case of a vascularized cervico-mediastinal mass, an angiography is recommended.•Angiography can detect the blood supply and guide the surgical approach. They are relatively common, accounting for 3–15% of all operated goiters and 6% of all mediastinal masses We present a case of cervico-mediastinal nonfunctional paraganglioma mimicking an ectopic goiter. It was removed through a cervical approach, after a preoperative arteriographic study.This work has been performed in line with SCARE 2020 criteria 2A 53-year-old woman came to our observation for persistent cough and dyspnea, increasing over the last 12 months. On physical examination, swelling and venous distention in the left side of the neck were noted, together with dilated vein collaterals in the upper left hemithorax. Chest x-ray detected a mass in the upper anterior mediastinum, causing narrowing and right-side deviation of the trachea. Blood tests were normal, including thyroid function tests. The patient medical history was unremarkable. Contrast-enhanced CT-scan of the neck and thorax showed a mass in contiguity with the left thyroid lobe and extending in the upper mediastinum, consistent with an intrathoracic ectopic goiter A, B. An Surgical resection was indicated. Prior to surgery, arteriography was performed which showed a predominant blood supply from the neck and no significant feeding arteries coming from the mediastinum A, B. TheThe post-operative course was uneventful and the patient was discharged on the fourth postoperative day. Histological examination showed a paraganglioma with a low mitotic index <5% . The res3Mediastinal paragangliomas are rare entities, usually located in the posterior mediastinum or in the aortopulmonary window and along the great vessels. The presence of the catecholamine syndrome may orient the diagnosis of a mediastinal mass toward a paraganglioma. Conversely, nonfunctional mediastinal paragangliomas represent a diagnostic challenge. They are extremely uncommon and symptoms are only related to mass effect When preoperatively diagnosed, the treatment of choice of a mediastinal paraganglioma is surgical excision 123I-metaiodobenzylguanidine (123I-MIBG) could be used 123I-MIBG is preferable to somatostatin receptor scintigraphy and to positron emission tomography, which can be positive also in case of goiter.An ectopic goiter represents undoubtedly the favored diagnosis when approaching a mass presenting as in our patient. It is difficult to take into account a paraganglioma in the differential diagnosis, especially in the absence of a catecholamine syndrome. However, when a paraganglioma is suspected, scintigraphy with When surgery for a cervico-mediastinal mass is planned, the choice of the approach, either from the neck or the thorax, is crucial. Especially in case of vascularized lesions, the approach depends primarily on the origin of the feeding vessels. The preferred approach for an ectopic goiter is through the thorax (thoracotomy or sternotomy), because the blood supply usually comes from mediastinal vessels and the removal through a cervical incision may be dangerous. Only a few cases of ectopic goiters which were removed from the neck have been reported 4123I-MIBG can be useful for the differential diagnosis. In case of a vascularized cervico-mediastinal mass, a preoperative angiographic study is recommended to detect its blood supply. The surgical approach of such lesions should be guided by the origin of the feeding vessels, regardless of the diagnostic suspicion. When completely removed, paragangliomas do not recur.Nonfunctional mediastinal paragangliomas are very uncommon and represent a diagnostic challenge. This case report contributes for some aspects to the knowledge of this entity. When presenting as cervico-mediastinal masses, paragangliomas can be easily misdiagnosed as intrathoracic or ectopic goiters. If a clinical suspicion of paraganglioma is raised, Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not commissioned.Externally, peer-reviewed.Ethical board approval is not required for a single case report in our Center.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Alessandro Stefani is the guarantor of this study.Not applicable.Daniel Bianchi and Matteo Costantini collected data.Alessandro Stefani and Daniel Bianchi wrote the manuscript.Adriana Scamporlino, Giorgio Cavallesco and Uliano Morandi revised and approved the manuscript.None.


Gallbladder disease is a very common condition that encompasses a heterogeneous group of diseases with a wide range of severity and clinical manifestations. Gallbladder disorders include biliary colic, acute cholecystitis, chronic cholecystitis, choledocholithiasis, Mirizzi syndrome, and gallstone ileus. We report the case of a 70-year-old man who presented to the emergency department complaining of worsening abdominal pain, located in the right upper quadrant and radiating to the right shoulder. It was aggravated by food intake and relieved with the use of antacid medications. The medical history of the patient was significant for stable angina, hypertension, diabetes mellitus, hyperlipidemia, and sickle cell trait. Examination of the abdomen revealed generalized tenderness with guarding. Abdominal computed tomography scan demonstrated the presence of significant diffuse thickening of the gallbladder wall with poor contrast enhancement. Complete resection of the gallbladder was performed because of suspected gallbladder carcinoma. Histopathological examination revealed xanthogranulomatous cholecystitis, which is a rare form of chronic cholecystitis. The differentiation between gallbladder carcinoma and xanthogranulomatous cholecystitis may not be easy and histopathological examination is the most accurate way to make the diagnosis. Gallbladder disease is a very common condition prevalent worldwide, affecting over 20 million individuals in the United States (US) alone. It is the most frequent gastrointestinal cause of hospital admissions . The preWe present the case of a 70-year-old man who presented to the emergency department complaining of worsening abdominal pain for the past one week. The pain was located in the right upper quadrant and was radiating to the right shoulder. He had been having this pain for the last two months; however, the severity increased recently. The pain was sharp in nature and the patient scored it as 7/10 in severity. It was aggravated by food intake and relieved with the use of antacid medications. However, the pain was not related to respiration or posture. He described having nausea with the pain, but it was not associated with vomiting, fever, or change in bowel habits. There was no history of anorexia or weight loss. He visited general practitioner clinics and was diagnosed as having indigestion for which he was offered oral proton pump inhibitor.The medical history of the patient was significant for stable angina, hypertension, diabetes mellitus, hyperlipidemia, and sickle cell trait. There was no history of abdominal surgeries. His medications included metformin 1000 mg, atorvastatin 20 mg, amlodipine 5 mg, captopril 5 mg, isosorbide dinitrate 20 mg, and omeprazole 40 mg. He did not have any drug allergies. There was no history of smoking or alcohol use. He was a retired school teacher and was active, performing regular physical exercise.On examination, the patient appeared in distress due to the pain. He was fully oriented in time, place, and person but he appeared drowsy. Examination of the abdomen revealed generalized tenderness with guarding. Bowel sounds were normal in frequency and intensity. No abnormal findings were noted in the examination of other systems. He underwent basic hematological and biochemical laboratory investigations Table . They reThe treating physician was concerned about the possibility of perforated viscus causing peritonitis. The patient underwent an abdominal computed tomography scan. It demonstrated the presence of significant diffuse thickening of the gallbladder wall with poor contrast enhancement. It did not reveal any evidence of complications, including abscess, perforation, or fistula formation. Moreover, no signs of hepatic invasion or biliary dilatation were noted Figure -2.The tumor markers, including carcinoembryonic antigen (CEA) and cancer antigen 19-9 (CA 19-9), were within the normal limits. The patient was prepared for laparotomy for the resection of the gallbladder because of suspected gallbladder carcinoma. During exploration, the gallbladder was found significantly enlarged. Complete resection of the gallbladder was performed successfully. The operation was uneventful for any complications. The patient was started on oral feeding on the second postoperative day. Histopathological examination of the resected specimen showed the presence of lipid-laden macrophages along with chronic inflammation features. Such findings conferred the diagnosis of xanthogranulomatous cholecystitis Figure . The patWe reported the case of an elderly man with xanthogranulomatous cholecystitis, a rare variant of chronic cholecystitis. The first case of xanthogranulomatous cholecystitis was reported by Christensen and Ishak in 1970 as a benign pseudotumor of the gallbladder. It is an idiopathic pathology in which there is a deposition of lipid-laden macrophages in the gallbladder . It reprThe pathophysiology behind xanthogranulomatous cholecystitis remains unclear. However, the most widely accepted explanation is that it develops as a result of bile extravasation into the gallbladder wall that involves the Rokitansky-Aschoff sinuses . RegardiIt might be difficult to differentiate xanthogranulomatous cholecystitis from gallbladder carcinoma based on radiological findings. The ultrasound examination may show nodules of low echogenicity within the thickened gallbladder wall . FurtherHistopathological examination is the best way to make the diagnosis with high accuracy. As in our case, it shows lipid-laden macrophages in the background of chronic inflammatory cells and fibroblasts ,4. CholeXanthogranulomatous cholecystitis is a rare form of chronic cholecystitis. The radiological features demonstrate diffuse thickening of the gallbladder wall and the differentiation between gallbladder carcinoma and xanthogranulomatous cholecystitis may not be easy. Histopathological examination is the most accurate way to make the diagnosis. Cholecystectomy is the definitive treatment of choice with the open approach being preferred in our case because of the preoperative suspicion of malignancy.


Although diverticula are not uncommon throughout the gastrointestinal tract, GDs are the least common type, and their presence in the prepyloric area is extremely rare. GDs are frequently asymptomatic but can present with serious complications that require surgical intervention in rare cases.A 54-year-old woman with a history of morbid obesity, hyperlipidemia, and diabetes mellitus (DM) presents with acute onset left upper quadrant (LUQ) abdominal pain. Based on presenting symptoms, an esophagogastroduodenoscopy (EGD) was performed to evaluate possible causes of abdominal pain. Interestingly, EGD revealed a moderately sized (3 cm) prepyloric diverticulum with a small polyp surrounded by normal gastric tissue. Biopsying of the intra-diverticular polyp revealed no abnormal pathology. Further assessment with Computerized Tomographic (CT) scan identified the diverticulum but with no other related gastric or gastrointestinal changes or pathology.The diverticulum was excluded as a cause of the pain. Hence, a conservative management approach was followed with no change in status for the following three months of observation. The patient continued to report non-specific symptoms but denied further episodes of abdominal pain or serious symptoms.GDs present with a wide variety of symptoms making the diagnosis difficult without thoroughly examining the entire anatomic region of potential pathology. Although GDs are rare, they are easily identified via EGD or imaging modalities. However, long-term follow-up information is needed to understand this clinical entity's behavior fully. •Gastric diverticula in the pre-pyloric area the rarest kind.•Pre-pyloric gastric diverticula are often asymptomatic but may present with a variety of symptoms including severe symptoms.•Gastric diverticula are often discovered incidentally on esophagogastroduodenoscopy.•Conservative management and diet modification are commonly used methods in the management of gastric diverticula.•It is important to document gastric diverticula in order to analyze trends of incidence in the population. Compared to other gastrointestinal tracts (GIT) diverticula, GDs are the least common form of diverticula. They are observed in 0.03–0.1% of upper gastrointestinal contrast studies, 0.03–0.3% of autopsy reports, and 0.01–0.11% of oesophagogastroduodenal endoscopies [Gastric diverticula (GDs) are outpouchings of the stomach that most frequently form along the posterior wall . They usDue to the rarity of this anatomic variation, studying GDs relies mainly on the case reports and series to understand this anatomic entity better. With the variation in presentation, size, and location, more information can be utilized to describe the clinical varieties and available management approaches. We report a case report of a middle-aged woman with a GD finding in the prepyloric area identified incidentally on the esophagogastroduodenoscopy (EGD) exam. In addition, we provide a literature review and discussion of the various presentations and management options.2A 54-year-old woman with a history of morbid obesity, hyperlipidemia, and diabetes mellitus (DM) presents with episodes of the left upper quadrant (LUQ) abdominal pain of two months duration. Other than occasional heartburn and bloating, she denied other significant symptoms of upper GIT. A recent ultrasound examination revealed a Gall Bladder polyp. Three months earlier, she was diagnosed with COVID-19 and was hospitalized with pneumonia. At presentation, her physical examination revealed reproducible tenderness in the LUQ. The treating team performed an EGD exam. The EGD revealed a moderate size prepyloric diverticulum on the lesser curvature side .Interestingly, a small polyp was identified inside the diverticulum surrounded by normal gastric tissue. Biopsy of the polyp tissue revealed normal gastric mucosa. Further assessment of the patient with Computerized Tomography (CT) showed the diverticulum  with no 3Gastric diverticula are rare findings that are usually identified incidentally during EGD examination. Pre-pyloric antral diverticula, such as the case we present, is even rarer than the average GD. Two types of GDs generally occur in two different locations of the gastric wall. Congenital GDs comprise 72% of all GDs, and they are commonly found within 2–3 cm of the gastroesophageal junction along the posterior wall of the stomach. Congenital GDs are considered “true” diverticula because they contain all layers of the GI wall  .Acquired GDs are less common than congenital GDs, and they generally occur near the antrum. They are considered pseudodiverticula or “false” diverticula because they do not include all three gastric wall layers  . Hence, GDs are equally distributed between men and women between the ages of 20–60 years old, with only 4% of GDs occurring in patients under 20 . The majGDs are often identified using radiological examinations using upper gastrointestinal contrast radiographic studies or abdominal CT scanning with oral contrast administration. Upper gastrointestinal endoscopic examinations also identify GDs, often incidentally . Most GDIt is well documented that asymptomatic GD's do not require specific treatment. Conservative management is the preferred course of action following the diagnosis of an asymptomatic diverticulum. In cases without serious complications, the use of proton pump inhibitors and a soft diet may alleviate mild symptoms .Around 10% of patients with true GD's require surgical intervention. Unfortunately, these cases often present with serious complications such as bleeding, perforation, or ulceration. Other indications for surgery include cases that are not resolved using conservative management. The optimal surgical procedure is resection of the GD with primary repair . LaparosIt is essential to study this clinical finding further and investigate the potential correlations with symptoms and complications. Practitioners are encouraged to report GDs to improve our understanding of their behavior. Further studies and attention to this clinical finding should be considered to document the incidence of this rare abnormality and analyze trends of prevalence in the population. Due to the lack of data in the field, the prevalence and association of GDs with symptoms or complications are currently unknown. With a better understanding of the behavior and trend of GDs, practitioners are more informed to manage GDs.4GD is a rare entity that is usually identified incidentally during EGD examination. Therefore, adequate assessment of the upper GI region is indicated to characterize the finding further and assess potentially associated abnormalities. Most GDs are asymptomatic and require no active management. However, significantly symptomatic or complicated GDs may benefit from surgical excision.The authors report no conflicts of interest and no funding. IRB was not consulted informed consent obtained.Not commissioned, externally peer-reviewed.N/a.Our institution does not require ethical approval for reporting individual cases or case series , N/a.Written informed consent was obtained from the patient to publish this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.2: Writing the draft, data collection, editing, submitting, revising.Connor Shea, MSAnn Sheets, RN: Data collection, documentation gathering.Faiz Tuma, MD, MME, MDE, EdS, FACS, FRCSC: Conceptualizing, writing, editing, supervising.N/a.Faiz Tuma, MD, MME, MDE, EdS, FACS, FRCSC.None declared.


The sellar and parasellar regions are a rare site of brain metastasis, most commonly from breast and lung cancer. Pituitary metastasis (PM) often presents as the first sign of metastatic disease but may herald early disseminated cancer. The diagnosis of PM requires differentiation from a benign pituitary adenoma. Although this may be proven definitively via surgical biopsy, a constellation of clinical findings including oculomotor palsy, visual disturbances, retroorbital pain, and diabetes insipidus is more suggestive of PM. Imaging is neither sensitive nor specific for differentiation but may inform the broader clinical picture. Due to its rarity, treatment guidelines for PM lack consensus, often including a mixture of radiation and surgery. Gross resection is challenging because of the vascular, invasive nature of these lesions. Stereotactic radiosurgery may be used to good effect either alone or in addition to resection. Even with treatment, the prognosis is poor. In this article, we present the third reported case of urothelial carcinoma metastasis to the pituitary. In addition, we review the clinical presentation, diagnosis, and treatment options including surgical resection and radiosurgery. The sellar and parasellar regions are relatively rare sites of brain metastasis, comprising between 0.14% and 28.1% of all brain metastasis [When symptomatic, it is important to differentiate metastasis from benign lesions such as pituitary adenoma. The most common presenting symptoms of PM include headache, oculomotor dysfunction, vision changes, and diabetes insipidus (DI) . The treHere, we present a rare case of urothelial carcinoma metastasizing to the sellar region. This is an unusual pathology, with only two previously reported cases found on literature review ,9. In thA 61-year-old female with a medical history of high-grade urothelial carcinoma presented with altered mental status, poor oral intake, and falls for one week. She was first diagnosed with urothelial carcinoma five years prior, which was complicated by a number of relapses in situ, including a recent recurrence three months prior. She underwent transurethral resection and recently completed chemoradiation therapy in the preceding month. Upon presentation, a computed tomography (CT) scan revealed a 2.4 × 1.2 cm lesion in the sella and suprasellar cistern. Magnetic resonance imaging (MRI) demonstrated a lesion involving the sella turcica with heterogeneous signal intensity and enhancement , free T4, free T3, prolactin, luteinizing hormone (LH), and follicle-stimulating hormone (FSH). Diagnosis of anterior hypopituitarism was made in addition to central DI. The patient was taken for biopsy via a transsphenoidal transsellar approach. The lesion was firm and not able to be removed by suction, thus a complete resection was not attempted. Pathology revealed metastatic urothelial carcinoma which was positive for CK903, GATA3, and p63. The patient underwent CT of the chest, abdomen, and pelvis which did not reveal other metastatic sites. She then underwent stereotactic body radiation therapy (SBRT) consisting of 25 Gy in five fractions to the sellar region and was discharged.The patient presented two months later with a left proximal diaphysis fracture. During this hospitalization, the patient developed acute adrenal insufficiency and severe epistaxis. A head CT revealed a large soft tissue mass in the sella extending to the sphenoid sinus. Further workup included CT chest, abdomen, and pelvis which revealed a significant burden of metastases within the liver, new from prior examinations.A repeat MRI of the lesion revealed significant progression to 5.2 × 2.6 cm which now extended along the infundibulum of the pituitary gland to the floor of the third ventricle causing a mass effect on the optic chiasm (Figure Approximately 20-40% of cancer patients suffer from brain metastasis . The incThe most common metastatic tumors to the pituitary region are breast and lung, making up nearly two-thirds of all PM ,3,4. ThiBladder metastasis to the brain is rare, accounting for approximately 0.6% of all brain metastasis . Of thesOur case presented similar to the first case mentioned above, occurring shortly after the conclusion of treatment for urothelial carcinoma. Both abovementioned patients presented with symptoms of altered mental status, visual disturbances, and anterior and posterior hypopituitarism. In our case, we were able to obtain pathologic diagnosis through an endoscopic endonasal approach.Between 6.8% and 16% of PM are symptomatic, likely leading to an underreporting of their incidence ,12. It iIn patients with known cancer, the discovery of a pituitary lesion should raise suspicion for a metastatic etiology. This is particularly important for surgical planning as many of these are not amenable to radical resection. When PM are discovered, they are often part of larger dissemination of the disease, often with five or more lesions and frequently involving the bone . HoweverThe differentiation between benign and metastatic lesions may be predicted by analyzing clinical symptoms as well as imaging characteristics, though surgical biopsy is the only way to obtain a definitive diagnosis. While there is some debate over which PM symptoms are most common, the main manifestations include DI, oculomotor palsy, visual disturbances, and retro-orbital pain. The presence of all four of these symptoms has a sensitivity of 81.8% and a specificity of 93.5% for PM [The presence of DI is specific for PM, only occurring in 1% of pituitary adenoma ,5. CompaImaging findings may also afford clues to whether a lesion is benign or metastatic, though there is significant overlap with the presentation of benign lesions making this modality nonspecific. A CT scan may show a hyperdense or isodense mass that is either homogeneously or heterogeneously enhancing, the latter if there is cystic degeneration, hemorrhage, or necrosis associated with the mass . The MRIThere is a lack of consensus on formal treatment guidelines for PM. Most commonly, patients often undergo a combination of surgery and radiotherapy. Gross total resection is often challenging because PM are often heavily vascular, dense, invasive of the surrounding bone and the cavernous sinus, with frequent infiltration of the hypothalamus and optic nerves. The complexity of these lesions may lead to significant complications including new-onset pituitary insufficiency and oculomotor palsies . SurgicaIn addition to surgery, radiation is a common treatment for PM with or without surgery . A case While data are limited, there are several case series describing treatment for PM that are worth highlighting. Zacharia et al. found that extended endoscopic transsphenoidal approaches resulted in an 81% rate of gross total resection with overall survival of 16 months. They also demonstrated an 18-month progression-free survival when surgery was combined with radiotherapy, demonstrating excellent local control . BurkharOverall, the prognosis for PM is poor as it signifies a more widely disseminated disease, which ultimately leads to mortality . RegardlPresented here is an exceptionally rare case of PM from urothelial carcinoma which is only the third reported case from this primary etiology. The patient presented with a solitary metastatic lesion in the sella and suprasellar cistern. The lesion was not amenable to surgical resection and was treated with SBRT. The patient presented again two months later with significant liver metastasis and PM progression. The patient passed away soon thereafter due to rapid disease progression.PM is most commonly a manifestation of breast or lung cancer, though instances have been reported from nearly all tissues. Diagnosis of PM requires differentiation from benign lesions. This may be done by reviewing the clinical picture and imaging findings, but tissue pathology is required for a confirmed diagnosis. Treatment of PM consists of a combination of radiation therapy and surgical resection which is indicated for palliation. Overall, the prognosis for PM is poor given the often widely disseminated nature of primary cancer. More reported cases would help clarify the appropriate treatment course for these rare but important cases.


A 73‐year‐old man presented with an abdominal mass that gradually swells over 3 months. He denied any subjective symptoms. Physical examination revealed massive enlargement of the spleen—the spleen had crossed the midline and its lower margin extended into the lower abdomen. The liver function test results were normal. Computed tomography showed massive splenomegaly (Figure A 73‐year‐old man presented with an abdominal mass that gradually swells over 3 months. He denied any subjective symptoms. Physical examination revealed massive enlargement of the spleen—the spleen had crossed the midline and its lower margin extended into the lower abdomen Figure . LaboratSplenic marginal zone B‐cell lymphoma occurs in <2% of non‐Hodgkin's lymphomas and presents with splenomegaly or dull abdominal pain.None.All authors had access to the data and a role in writing the manuscript.We have obtained the consent of the patient for publication.


Case Presentation. After multidisciplinary consultation, in July 2015, postoperative adjuvant radiotherapy was offered to a 52-year-old man with a multiple recurrent ossifying fibromyxoid tumor in the right frontal sinus causing TIO. The patient had a history of multiple bone fractures and pain since more than 20 years. The tumor had been removed in 2003 for the first time. Subsequent endoscopic resections of the tumor had been performed for recurrences of TIO in May 2012, October 2013, and July 2015. Starting from October 2015, external beam radiotherapy was delivered with a volumetric modulated arc technique to the tumor bed with a daily dose of 2 Gy up to a total dose of 60 Gy. After five years from treatment, the patient is free from local tumor relapse, TIO progression, and radiation-induced side effects.  Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome caused by protein fibroblast-growth-factor-23 (FGF-23) secreting tumors. Complete tumor resection is the current standard of care for TIO; however, some patients may develop tumor recurrence. Due to the rarity of this paraneoplastic syndrome, the role of radiotherapy is unclear. This case is worth reporting because it adds to our knowledge some insights about the potential role of radiotherapy in this rare condition.  Radiotherapy may provide long-term TIO remission and tumor control, thus being a treatment option in cases where surgery is unfeasible or unsuccessful. Tumor-induced osteomalacia (TIO) or oncogenic osteomalacia is a rare paraneoplastic syndrome resulting from excessive production of the protein fibroblast growth factor-23 (FGF-23). FGF-23 act as a phosphaturic hormone and also favors inactivation of 1, 25 dihydroxyvitamin D, thus leading to compensatory hyperparathyroidism. Patients typically present with symptoms of gradually progressive weakness, bone pain, and pathological fractures. Benign mesenchymal or mixed connective tissue tumor is the most common histological feature of neoplasms associated with TIO. Complete tumor resection is the current standard of care for TIO . We repoThe patient was a 52-year-old man with no family history of cancer and no significant comorbidity except hypercholesterolemia and arterial hypertension.The patient had a history of bone pain, weakness, and multiple pathological fractures for more than 20 years. In 2003, osteomalacia was diagnosed by bone biopsy at the level of the right knee. In the same period, the patient underwent also surgical removal of a small lesion on the left frontal sinus that was diagnosed as an ossifying fibromyxoid tumor. After surgery, the patient experienced a period of symptom improvement, but again, over the following years, bone pain progressively worsened, and many nontraumatic bone fractures occurred in the right femur, ribs, right tibia, right fibula, and metatarsal bones despite therapy with vitamin D3 and calcium. In 2012, the diagnostic hypothesis of osteomalacia of oncogenic origin finally arose, and the patient underwent targeted investigations.68Ga-DOTATATE positron emission tomography (PET) scan. Blood tests documented low levels of phosphate and high levels of FGF-23.A total body computed tomography (CT) scan documented the presence of dense tissue in the left frontal sinus. At that level, a magnetic resonance (MR) examination showed a lobulated lesion measuring 2 cm, which was hypointense in the T1 sequences and hyperintense in the T2 sequences and had intense contrast enhancement. The lesion highly expressed somatostatin receptors on a 68Ga-DOTATATE PET scan showed a small lesion in the left frontal sinus with hyperexpression of somatostatin receptors. Six months later, a MR scan confirmed the presence of a contrast-enhancing lesion measuring 1.5 cm in the medial wall of the left frontal sinus, without signs of bone or orbital infiltration. In October 2013, the patient underwent an endoscopic frontal senectomy to remove the recurrence of the ossifying fibromyxoid tumor. Again, after less than two years of clinical remission, TIO symptoms worsened. In March 2015, an MR scan confirmed the presence of a small recurrence of the tumor, without signs of bone or orbital infiltration. A fourth surgical intervention was performed in July 2015 through a combined endoscopic and open approach to completely remove the lesion. A postoperative contrast-enhanced computed tomography was performed four weeks later, and it did not show any suspect sign of disease persistence. In August 2015, the case was discussed in a multidisciplinary tumor board and adjuvant radiation therapy was proposed to reduce the risk of further tumor recurrence. This multidisciplinary decision was shared with the patient who gave his consent to the treatment. Starting from October 2015, external beam radiotherapy was delivered with a volumetric modulated arc technique (VMAT) to the tumor bed with a daily dose of 2 Gy up to a total dose of 60 Gy. The patient was treated in the supine position with a thermoplastic facemask. The tumor bed was identified on coregistered preoperative MR, 68Ga-DOTATATE PET, and planning CT simulation images. The clinical target volume (CTV) included the tumor bed plus 1 cm margin along the frontal sinus walls. The planning target volume (PTV) included the CTV plus 3 mm margin. The organs at risk (OARs) were the optic chiasm, right and left cochlea, right and left lens, right and left eyeballs, and right and left optic nerves. Details of the radiation treatment are shown in In May 2012, the lesion of the left frontal sinus was removed through an endoscopic approach. Histological examination documented an ossifying fibromyxoid tumor that was framed as a recurrence of the lesion that had been removed in 2003. After a brief period of clinical and laboratory remission, TIO symptoms reappeared and gradually worsened. In April 2013, a After 60 months from treatment, the patient is free from both tumor recurrence and TIO.The role of radiotherapy in the multidisciplinary treatment of TIO is far to be defined due to the rarity of this condition. This report described the case of a patient with TIO caused by a multiple recurrent ossifying fibromyxoid tumor, who was cured by surgery and postoperative radiotherapy. To our knowledge, only eight cases of radiotherapy treatment for TIO have been reported so far , 6, 7, 9External beam radiotherapy was used in six cases. Peptide receptor radiotherapy (PPRT) was used in the other two cases. External beam radiation treatment was poorly detailed in published reports. When specified, the total radiation dose ranged between 45 and 66 Gy, and treatment was delivered with conventional fractionation. All patients who underwent radiotherapy had complete remission of TIO. Particularly, three patients who had more than three years of follow-up experienced long-term tumor control and TIO remission.In conclusion, external beam radiotherapy might provide long-term tumor control and TIO remission, thus being a possible option whenever surgery is unfeasible or unsuccessful.