Fibrous dysplasia is a benign, developmental bone disorder that causes fibrous replacement of normal skeletal tissue. This may lead to weakness, distortion, and tissue expansion. Fibrous dysplasia can occur anywhere in the body, including the craniofacial area. The clivus is a central skull bone formed by the bases of the sphenoid and occiput, respectively. The clivus is a rare, usually unrecognized, and seldom reported location for the development of fibrous dysplasia. Although fibrous dysplasia of the clivus (FDC) is usually discovered by incidental findings, it can sometimes present with clinical symptoms.In this case, we discuss a 30-year-old male who presents to the emergency room with headaches, altered mental status, and a prior presentation of location-related symptomatic epilepsy. Magnetic resonance imaging depicted a mass in the clivus, low in signal on T1 and mildly hypointense on T2 imaging. Follow-up computed tomography (CT) imaging, as recommended, revealed the classic presentation of FDC. In this paper, we discuss the significance of this condition and the importance of thorough investigation to rule out differential diagnoses that may present with similar acute symptoms as this patient. Fibrous dysplasia is a benign, congenital skeletal disorder that leads to fibroosseous and intramedullary bone lesions. It is a primary disease of the bone and can lead to weakening, expansion, and distortion of the affected bone . It is aThe clivus is a bone situated at the base of the central skull. It is formed by the bases of the sphenoid (basisphenoid) and occiput (basiocciput). Although rare, craniofacial manifestations of fibrous dysplasia are usually identified in the clivus. These usually present with no symptoms and are discovered incidentally on imaging for unrelated reasons. Sometimes, however, the fibrous replacement of bony tissues can lead to fractures, neurovascular and soft tissue compression, cosmetic deformity, and intracranial extension .We present the case of a 30-year-old male and discuss the symptoms, pertinent findings, management, and follow-up in this case. We also highlight and compare other cases of this condition and discuss the importance of ruling out other causes of the acute symptoms seen in this patient.History and physicalA 30-year-old man presented to the emergency room with a two-day history of headaches. On this day, the headaches had been accompanied by visual disturbances, and he had been sent by his primary care physician with this complaint, with the recommendation that he get some cervical imaging to further evaluate the possible causes of his symptoms.The patient endorsed a history of headaches that had progressively worsened in the past few months. Attempts at alleviating his discomfort with ibuprofen had been only mildly helpful. There was no significant medical history. He did not drink, smoke, or indulge in any illicit drugs. The review of systems was positive for headaches and blurry vision but negative for all other symptoms.On physical examination, the patient was alert and oriented to person, place, and time. Cranial nerve tests were intact, as well as musculoskeletal, strength, and range of motion (ROM). Pupils were equally responsive to light and accommodation. The remainder of the physical exam was largely non-contributory.Diagnostic imagingIn the emergency room, magnetic resonance imaging (MRI) of the brain without contrast was promptly ordered and performed. Figures Following the radiologist’s recommendation, a CT scan without contrast was performed as well. The CT imaging result can be seen in Figure ManagementConsidering that our radiologist promptly identified this lesion as fibrous dysplasia of the clivus (FDC), it was recommended that no further lab tests or workup be performed in this patient. His treatment team, which included his internist, the emergency medicine physician, and his neurologist, concurred with the recommendation. Choices in management were discussed with the patient. These included watchful waiting with clinical observation and more frequent imaging given his symptoms, and elective surgery to relieve him of the lesion and possibly cure the symptoms and condition. Fibrous dysplasia is thought to be the result of a missense mutation in the GNAS 1 gene on chromosome 20. This leads to an activating mutation, leading to altered osteogenesis and abnormal proliferation of fibrous tissue . It can FDC is a relatively rare presentation of this disorder. A PubMed literature search identified 44 reported cases. Of these, one case was diagnosed with the polyostotic form, associated with McCune Albright Syndrome. In this case, the patient, diagnosed at age 3, developed severe complications secondary to McCune Albright, eventually succumbing to a subarachnoid hemorrhage at age 30. This arose due to the disruption of intracranial vasculature by severe bone disease . A seconMost cases of FDC present without symptoms. In symptomatic patients, the main presentation was headaches, either sudden or persistent, as seen in our patient. In the study by Ham et al., one patient presented with bifrontal headaches and disequilibrium for six months and another with supraorbital headaches and right facial hypesthesia for two weeks . Other cConsidering that most patients present with no symptoms and this condition is usually discovered incidentally, a physical examination is usually non-contributory. In our patient, persistent headaches were the only symptom, and a physical exam proved unremarkable. In patients who present with symptoms, however, prompt follow-up testing and imaging are usually required to rule out differential diagnoses, some of which could prove debilitating or fatal without prompt intervention.Imaging in fibrous dysplasia occurs with MRI and/or CT scans, scintigraphy, radiography, and MRI. MRI interpretation of the normal clivus denotes bright T1 fatty content relative to the pons. T1 hypointensity and T2 hyperintensity usually suggest a clival abnormality . CT scanGiven the presentation of fibrous dysplasia and its location in the clivus, a number of other conditions must be considered in the differential. Two important ones are chordomas and plasmacytomas, both of which can occur in the clivus. Intracranial chordomas are rare tumors thought to originate from remnants of the primitive notochord . They arPlasmacytomas are plasma cell tumors that arise within bone or soft tissue. They rarely occur in the skull and are usually located in the orbit, dorsum sellae, and sphenoid sinus . They veTreatment and management of FDC depend on the severity of the symptoms. Because the majority of cases are discovered incidentally and patients are generally asymptomatic, most patients are observed clinically. These lesions tend to be stable, and growth is not usually observed. However, malignancies have been reported in some cases, at almost 0.05% . As suchThis case describes a 30-year-old male with fibrous dysplasia of the clivus, a rare cause of clival disease, and presentation symptoms secondary to mass effect. In this paper, we discuss the details of the case and review the existing literature. FDC is often discovered in asymptomatic patients as an incidental finding during imaging for other reasons. In a small subset of patients, FDC is discovered after symptoms develop, usually secondary to its location in the skull or mass effect. In asymptomatic patients, management is accomplished by clinical observation and follow-up imaging, usually yearly on average, as these are benign lesions and are not expected to become malignant. Patients with severe or debilitating symptoms are treated with surgery, with the most current approach being endonasal transsphenoidal resection.In our case, after extensive discussions with the treatment team, our patient opted for close clinical monitoring and deferment of surgery. He was advised to return to the hospital if, at any point in time, he developed more serious symptoms such as worsening headaches, loss of vision, ataxia, paralysis, or weakness. He was discharged with medications for his headaches and scheduled follow-up dates for clinical evaluation and imaging.


Chronic functional constipation is a common condition that can have a significant impact on a patient’s quality of life and healthcare costs. Hydrostatic enemas are a commonly observed practice among patients with chronic constipation. Rectal perforation is a rare yet serious complication that can be fatal if not diagnosed and treated promptly. Here, we present the case of an elderly lady with Parkinson’s disease who presented with upper rectal perforation after using a hydrostatic enema and was treated with Hartmann’s procedure. This case highlights the importance of having a low threshold for suspecting and diagnosing colorectal perforation in patients presenting with abdominal pain after receiving a hydrostatic enema. Constipation is a common complaint in older adults and can have a significant impact on a patient’s quality of life, healthcare costs and services, hospital admissions, and surgical procedures. In a study from Saudi Arabia, 25% of respondents to a questionnaire reported having constipation. Constipation was defined as having fewer than three bowel movements per week, along with one or more of the following symptoms: pain during evacuation, hard or lumpy stools, or feeling of incomplete evacuation .Constipation has also been associated with lower quality of life. Several studies have reported that people with constipation have lower scores on measures of physical and mental health, as well as lower levels of satisfaction with their lives , making We have observed that many patients report using self-administered retrograde water-hose enemas at home to relieve their constipation. This practice can lead to colonic perforation, which is a serious condition that can be fatal . Here, wA 77-year-old female with PD on levodopa and amantadine with chronic constipation presented to the emergency department with abdominal pain and distention. She had been experiencing abdominal pain and constipation for one week and had tried a self-administered water-hose enema on the day of the presentation. However, this only made her pain worse, and she developed nausea. On presentation, the patient was afebrile, and her vital signs were within normal limits. Her abdomen was distended and tender to palpation. There was no rebound tenderness or guarding, hepatosplenomegaly, or lymphadenopathy. Laboratory studies showed a white blood cell count of 7,800/μL . Her electrolytes and liver function tests were normal. On imaging studies, the erect abdomen X-ray was unremarkable Figure , 3.After the CT scan, the patient’s condition worsened and she started to develop abdominal tenderness and rigidity. She was taken to the operating room for an emergency exploratory laparotomy. The abdomen was full of stool and free fluid. The bowel was examined and an upper rectal perforation extending to the rectosigmoid area was noted. The surgical team performed a Hartmann’s procedure with proximal end colostomy and rectal stump left in the pelvis. The patient was admitted to the intensive care unit for further care and management. She was discharged from the hospital in good condition on postoperative day 15.The final histopathology showed a segment of the rectosigmoid colon measuring 14.5 cm in length and 6 cm in diameter. A perforation site was grossly identified measuring 1.5 × 1 cm  and 57-67% experience difficult defecation. These rates are significantly higher than those in the general population -10. The Treatment for constipation in PD patients typically begins with non-pharmacological agents, such as fiber supplements, stool softeners, and probiotic supplements. If these are not effective, pharmacological laxative treatments  may be used. In some cases, newer modalities, such as Lubiprostone (a chloride channel activator), Prucalopride (a 5-HT4 receptor agonist), and Relamorelin (a ghrelin agonist) may also be used .Retrograde rectal enemas are commonly used to treat chronic constipation, especially in the elderly. However, this practice can sometimes lead to rare but serious complications such as bowel perforation. A systemic review and meta-analysis found that most patients who developed rectal perforation after rectal enemas had received the enema from a nurse, followed by self-administrated enemas. Phosphate enemas were the most commonly used type .Recently, there has been an increase in the number of patients who report using self-administered retrograde water-hose enemas  at home to relieve constipation. Several reports have shown that rectal or colonic perforation is sometimes a possible outcome of this common practice . The cliIn this case, the surgery team ordered a CT scan but it failed to demonstrate a clear radiological picture of perforation. Subsequently, they explored the patient’s abdomen based on her history of using a water-hose enema at home and her abdominal findings during the physical examination. It is crucial to maintain a high index of suspicion for this rare but serious complication, as delays in diagnosis are associated with worse outcomes and higher morbidity and mortality .CT scan is a valuable diagnostic tool for confirming the diagnosis of rectal perforation and assessing other causes of acute abdomen. Pneumoperitoneum is usually seen with the perforation of the colon or intraperitoneal part of the rectum, but it is reported less with perforation of the extraperitoneal rectum. In our patient, the perforation was in the intraperitoneal part of the rectum, and a massive pneumoperitoneum is an expected finding in the CT scan. In patients with perforation of the extraperitoneal rectum, the infection may spread more slowly from the perirectal fat to the retroperitoneal space, and a CT scan may show non-specific findings such as mural thickening and mesorectal smudging .The conflict in our case is represented by the discordance between CT radiological findings, on one side, and intraoperative findings and histopathology reports, on the other. As mentioned previously, the perforation was intraperitoneal in origin at the upper rectum which was confirmed by intraoperative findings and the final histopathology report. A massive pneumoperitoneum is an expected finding in the CT scan with such a large intraperitoneal perforation (about 1.5 × 1 cm), but it was not seen in the patient’s CT scan, with the findings being inconclusive in the CT report for clear intraperitoneal perforation. Other CT findings of rectal mural enhancement and thickening can be seen with other pathological processes of the rectum or can represent extraperitoneal perforation of the rectum; however, this was not the scenario in our case. Although a CT scan is a very sensitive tool to evaluate the abdomen and diagnose these cases, sometimes it can be misleading. Physicians must always rely on patient history and physical examination and have a low threshold for diagnosing these cases.The choice of surgical intervention for patients with acute colorectal perforation depends on several factors , includiThe main goals of surgical intervention include (1) cleaning the abdomen . This is especially important in cases of fecal peritonitis with gross stool contamination. (2) Removing the perforated area of the bowel. This may be done with a primary anastomosis, or with a fecal diversion. (3) Draining the abdomen to prevent further collection and abscess formation.The specific type of intervention that is used depends on individual patient circumstances and the judgment of the surgeon . HartmanIn our case, the surgical team decided to perform Hartmann’s procedure. A primary anastomosis was not attempted due to concerns about the patient’s clinical instability during surgery. It is important to submit the specimen to the histopathology department for further analysis. The histopathology findings can confirm whether or not there is any neoplastic process.There is a need to raise awareness about the risks of water-hose enemas among the public. Many patients are not aware of the dangers of this practice and may believe that it is a safe and effective way to relieve constipation. It is important to educate people about the risks of water-hose enemas and to encourage them to seek medical treatment for constipation from a qualified healthcare professional.Hydrostatic enema is a common practice for patients with chronic functional constipation. A careful history and clinical examination, assisted by imaging studies such as a CT scan with contrast, can help in the early diagnosis of colorectal perforations and improve outcomes. Clinicians should always be on the lookout for this complication, especially in patients who report sudden severe abdominal pain after using a rectal enema. In addition, patients should be educated about the risks of hydrostatic enema.


Dyskeratosis congenita (DC) is a rare telomerase disorder affecting high turnover cells. Malfunction of protective proteins in DC results in patient genomes with shortened germline telomeres leading to genetic instability, cellular apoptosis, and overall cellular lifespan degradation. Classically, reports of DC described a triad of dysplastic nails, reticular skin pigmentation, and oral leukoplakia. However, more recent reports have focused on disease presentation affecting other high turnover organ systems including the gastrointestinal system. Patients may present with dysphagia because of esophageal stricture/web, diarrhea secondary to enteropathy or enterocolitis. We present a pediatric patient who presented with feeding difficulty secondary to an esophageal stricture as the primary manifestation of DC. She was diagnosed with Revesz Syndrome, a rare subtype of DC, along with a novel genetic variant not previously reported. This report serves to bring awareness to gastroenterologists that DC, though classically thought to present with dermatological findings, can present with primary gastrointestinal manifestations. Dyskeratosis congenita (DC) is a multisystem disorder resulting in patients with very shortened germline telomeres . TelomerDC was first described primarily with description of dermatological manifestations including a classical triad of dysplastic nails, reticular pigmentation of the neck and back, and oral leukoplakia. As more cases were reported, other organ manifestations were discovered and described. However, diagnostic criteria continues to require one of the above dermatological manifestations to meet diagnosis. To date, 11 genetic pathogenic variants have been identified. Depending on the genetic variant, inheritance pattern can be X-linked, autosomal recessive, or autosomal dominant . Because of abnormal Doppler readings, oligohydramnios with intrauterine growth restriction, and low biophysical profile, she was delivered via cesarean section.TINF2 gene, c.841C>T (p.Glu281*). Telomere analysis revealed extremely short telomeres in all lymphocytes, granulocytes, natural killer cells, all below the first percentile. Magnetic resonance imaging of the brain revealed bilateral globe thickening consistent with retinal exudate , enteropathy with diarrhea and poor growth (second most common), noncirrhotic pulmonary hypertension, hepatopulmonary syndrome, vascular ectasias of the gastrointestinal tract resulting in bleeding, anorectal adenocarcinomas, and anal strictures –6. In adTINF2 gene never published before.In addition to her diagnosis of DC, the patient was found to have a more severe variant called Revesz syndrome that, as of publication of this report, has only been reported 18 times in the English literature. It is associated with even shorter telomeres than classical DC and patients can also present with intracranial calcifications and bilateral exudative retinopathy which she had on radiological and ophthalmological exam Fig. A,B 7). . 7). In Treatment of DC is typically individually tailored and primarily supportive management . In the DC can present solely with gastrointestinal symptoms and, in our case, without any dermatological findings. In our case, the patient’s mild anemia and thrombocytopenia was not considered as part of her disease and her esophagram, although limited in evaluation, did not identify her stricture. It was only after more comprehensive and invasive evaluation that the patient’s rare and severe phenotype of DC was diagnosed. Thus, a disease such as DC can be missed if only the classical triad is considered. We hope that this case brings awareness to gastroenterologists regarding nonclassical, primary gastrointestinal presentations of DC.Jeffrey Lee drafted the manuscript, Edward Cheeseman and Maria Matheus provided imaging, and Nagraj Kasi edited and approved the final draft submitted.


The histopathological diagnosis of canine splenic mass lesions is crucial for determining prognosis. Herein, we analyzed the prevalence of canine splenic mass lesions in Republic of Korea via histopathological diagnosis. Immunohistochemistry was performed for specific tumor markers for a more accurate diagnosis. The results of this study will aid veterinary clinicians in communication with pet owners about prognoses and recommendations for splenectomy. This study will facilitate additional investigations with more detailed comparisons of splenic mass lesions between small- and large-breed dogs.The histopathological diagnosis of canine splenic mass lesions is crucial for prognostication. However, thus far, no study has been conducted on the histopathology of canine splenic mass lesions in Republic of Korea. Herein, the prevalence of splenic diseases was analyzed in 137 canine splenic mass lesions via histopathological diagnosis, and the microscopic pattern associated with each disorder was described. Immunohistochemistry was performed for CD31, CD3, PAX5, Iba1, and C-kit for a more accurate diagnosis of splenic tumors. The proportion of non-neoplastic disorders, including nodular hyperplasia  and hematoma , was 72.3%. Splenic tumors, including splenic hemangiosarcoma , splenic lymphoma , splenic stromal sarcoma , myelolipoma , and mast cell tumors , accounted for 27.7% of cases. The results of this study will aid veterinary clinicians in communication with pet owners about prognoses, recommendations for splenectomy, and subsequent histopathological diagnoses. This study will facilitate further investigations with more detailed comparisons of splenic mass lesions between small- and large-breed dogs. Disorders of the canine spleen are increasingly being recognized with the use of advanced diagnostic equipment and higher frequencies of splenic mass lesions in aged dogs. Clinical veterinary surgeons perform splenectomies in dogs for therapeutic purposes; however, hematopoietic and immune functions are inevitably reduced in dogs that have undergone splenectomies . TherefoFor decades, canine splenic mass lesions have been diagnosed using histopathology for etiologic differentiation, as the tissue structure is maintained during histopathologic processing ,4,5. HisSplenic neoplasia is commonly observed in older dogs and can be biologically aggressive . Most maSplenic tumors can have various origins, including vascular, lymphoid, fibrous, smooth muscle, myeloid, and adipose tissues. Many studies have shown that the most common canine splenic tumor is splenic hemangiosarcoma ,11,12,13Non-neoplastic nodular diseases of the canine spleen include nodular hyperplasia and splenic hematoma. Nodular hyperplasia is a benign proliferation of cells that normally exist in the spleen and is one of the most common causes of canine splenic mass formation . NodularSplenic hematoma is a pooling of blood in the splenic parenchyma caused by a hemorrhage or disrupted blood flow in the spleen . The cauMany studies have been conducted on the analysis of splenic mass lesions in dogs ,16,17,18Herein, we analyzed the prevalences of different canine splenic mass lesions using histopathological diagnosis and described the microscopic patterns of various splenic mass lesions. In addition, immunohistochemistry was performed for specific tumor markers for a more accurate diagnosis of splenic tumors.All samples were collected from local animal hospitals via partial or total splenectomies for treatment and subsequent histopathological diagnosis. No live animals were used in this study; therefore, approval from the Institutional Animal Care and Use Committee (IACUC) was waived. Informed consent for the use of clinical data and samples for research was obtained from the owners of the dogs.We randomly selected 140 samples of canine splenic tissue from the histopathological database of the Department of Veterinary Pathology, Konkuk University, Seoul, Republic of Korea, between January and October 2022. Patient information, such as age, breed, and sex, was collected from clinical records. Splenic tissue samples were fixed in 10% neutral buffered formalin, processed routinely, and embedded in paraffin wax. Tissue blocks were sectioned to a thickness of 4 µm and stained with H&E. After an initial screening, three samples were excluded because two were non-mass lesions and one was not available for histopathologic diagnosis; thus, 137 samples were analyzed.Tumors in domestic animals, 5th edition [The H&E-stained slides were microscopically evaluated by two pathologists (YU-K and MK-B). Discrepancies were resolved by discussion with a third pathologist (JH-S). Photomicrographs were obtained with a digital camera (Olympus DP22). Histopathological diagnosis was based on  edition .Immunohistochemistry was performed for CD31 , CD3 (T cells), PAX5 (B cells), Iba1 (histiocytes), and C-kit (mast cells). Formalin-fixed paraffin-embedded tissues were processed into 4 µm thick sections and picked up with silane-coated slides. The sections were deparaffinized in xylene and rehydrated in graded ethanol before staining. The sections were washed three times in phosphate-buffered saline (PBS) and incubated in 3% hydrogen peroxide prepared in PBS for 20 min at room temperature to block the endogenous hydrogen peroxidase activity. Target antigen retrieval was performed, using a pressure cooker, in a citric acid buffer (pH 6.0) or a Tris-EDTA buffer (pH 9.0) for various periods . The secOne dog had no age information; the average age of the remaining 136 dogs was 10 years (range: 3–18 years). There were 13 intact males, 13 intact females, 65 castrated males, and 46 spayed females. A total of 127 purebred and 10 mongrel dogs were represented. Most of the purebred dogs were small breeds , and the five breeds that accounted for the highest number were the Maltese (n = 55), toy poodle (n = 17), shih-tzu (n = 10), Pomeranian (n = 8), and Yorkshire terrier (n = 6). Medium to large purebred dogs comprised the cocker spaniel (n = 3), golden retriever (n = 2), Japanese spitz (n = 2), Welsh corgi (n = 2), border collie (n = 1), German shepherd (n = 1), Jindo (n = 1), Labrador retriever (n = 1), standard poodle (n = 1), and whippet (n = 1).In total, 137 splenic tissue samples were examined. The number of splenic non-neoplastic lesions  was more than twice that of neoplastic lesions . The histopathological diagnoses of all the samples are summarized in Nodular hyperplasia was diagnosed in 66 dogs in this study. The average age at diagnosis was 9.6 years (range: 4–18 years), and there were 36 males (32 castrated) and 30 females (26 spayed). The main microscopic finding was the proliferation of lymphoid follicles. The nodules were well demarcated and non-infiltrative. The lymphoid follicles were separated from each other and had normal structures, such as the germinal center, mantle zone, and marginal zone A. Most oImmunohistochemistry was performed for CD3 and PAX5, which are markers of T and B cells, respectively. Lymphoid nodular hyperplasia contained T- and B-cell populations in separate areas, correlating with the normal lymphoid follicle anatomy. The T-cell populations were confirmed at the periphery of the follicles B, and B Hematoma was diagnosed in 33 dogs. The average age at diagnosis was 10 years (range: 3–16 years), and there were 18 males (13 castrated) and 15 females (12 spayed). Histologically, the splenic mass was formed by an accumulation of coagulated blood A. The maImmunohistochemistry was performed for CD31, a marker of endothelial cells. In splenic hematomas, CD31 was only expressed in a layer of endothelial cells that composed normal blood vessels C.Fourteen dogs were diagnosed with splenic hemangiosarcoma, and Maltese (n = 8) and toy poodles (n = 2) accounted for most of the cases. The other breeds were the Japanese spitz (n = 1), miniature schnauzer (n = 1), Pomeranian (n = 1), and German shepherd (n = 1). The average age at diagnosis was 11.1 years (range: 8–15 years), and there were eight males (six castrated) and six females (three spayed). In all cases, the tumors were poorly demarcated and infiltrated into the regional parenchyma. Necrosis and bleeding occurred broadly in these lesions. Neoplastic endothelial cells were arranged into irregular vascular spaces and trabecular structures A. Solid Immunohistochemistry was performed for CD31. All splenic hemangiosarcoma samples exhibited strong cytoplasmic immunoreactivity with CD31 C. 2)  and Toy poodles (n = 3) accounted for most of these cases. The other breeds were the bichon frise (n = 1), border collie (n = 1), golden retriever (n = 1), and mongrel (n = 1). The average age at diagnosis was 9.5 years (range: 5–13 years), and there were six males (four castrated) and five females (three spayed). Morphological characteristics in H&E staining were examined and immunohistochemistry for CD3 and PAX5 was performed to classify the splenic lymphoma cases according to the revised European–American lymphoma classification adopted by the World Health Organization. The 11 cases of splenic lymphoma were classified into 6 marginal-zone lymphomas, 1 diffuse large B-cell lymphoma (DLBCL), and 4 peripheral T-cell lymphomas. The marginal-zone lymphomas showed a nodular growth pattern and a characteristic architecture, such as a proliferating marginal zone and fading germinal centers A. The ne2) D. Immuno2) B,C,E,F. 2) G, and ne2) H,I. Ten dogs were diagnosed with splenic stromal tumors, most of which were Maltese (n = 8). The other breeds were the bichon frise (n = 1) and Labrador retriever (n = 1). The average age at diagnosis was 11.4 years (range: 10–14 years), and there were eight castrated males and two spayed females. Histologically, all stromal sarcomas were poorly demarcated and infiltrated into the surrounding parenchyma. The neoplastic cells were spindle-shaped with collagen deposition and formed broad fascicles that ran in diverse directions A. The cyImmunohistochemistry was performed for Iba1 and CD31 in all stromal sarcoma samples to differentiate histiocytic sarcoma and hemangiosarcoma. The neoplastic mesenchymal cells showed negative responses to Iba1 and CD31 C,D.Two patients were diagnosed with myelolipoma. The first was an 8-year-old castrated male whippet, and the second was a 13-year-old castrated male miniature schnauzer. Histologically, well-differentiated adipose and hematopoietic tissues were intermixed A. The adThe spleen of a 15-year-old spayed female mixed-breed dog was diagnosed with a splenic mast cell tumor. The histomorphologic characteristics included multifocal to coalescing nodules of neoplastic mast cells. Marked necrosis and hemorrhages were found throughout the splenic tissue, with distortion of the splenic architecture. The proliferating mast cells were round or polygonal and contained cytoplasmic granules B. In addImmunohistochemistry was performed for C-kit, a mast cell marker. Neoplastic cells displayed strong cytoplasmic immunoreactivity with C-kit C.In the present study, the average age of the 136 dogs was 10 years, which suggests a prevalence of splenic mass lesions in older animals. This result was similar to those of other studies in which the mean ages of dogs with splenic mass lesions were 10 to 11 years ,18,19,20Although splenic non-neoplastic lesions occurred more frequently than splenic neoplasia, the incidence of neoplasia was not negligible (28%). The most common splenic mass lesions was nodular hyperplasia , with lymphoid nodular hyperplasia being the most common histologic diagnosis, followed by hematoma . Consistent with the results of several previous studies ,10,11,12The incidence of splenic neoplasia in the present study was slightly lower than that reported in an Italian study (30%) . It was Splenic hemangiosarcoma is characterized by aggressive growth and a grave prognosis. A microscopic examination revealed aggressive histomorphologic features in all hemangiosarcoma cases, including poor demarcation of the mass, infiltration, and necrosis. They carry a risk of rupture, which can lead to disseminated metastasis by seeding tumor cells and hemoabdomen. The histologic diagnosis of splenic hemangiosarcoma is usually uncomplicated; however, if identifiable patterns of vessel formation are not apparent , a definitive diagnosis is more difficult. Additionally, the differentiation of splenic hematoma, hemangiosarcoma, and stromal sarcoma is sometimes challenging ,22. The The diagnosis of canine splenic lymphoma is mainly based on histopathological features, but it can be difficult to distinguish some types of lymphoma  from benign lymphoid hyperplasia. In splenic tissues with ambiguous H&E findings, immunophenotyping with immunohistochemistry for T- and B-cell markers can help differentiate reactive proliferations of lymphocytes from lymphoma. In this study, the differentiation of splenic lymphoma and nodular hyperplasia could be sufficiently performed via immunohistochemical staining with the homogenous expression pattern of either CD3 or PAX5. However, immunohistochemistry for CD3 and PAX5 may not allow for easy distinctions between some cases of lymphoma  and nodular hyperplasia. Moreover, the homogenous expression of either CD3 or PAX5 will not apply to null-cell (non-B-cell and non-T-cell) lymphoma. In those cases, immunohistochemistry for Ki-67 and PCR for antigen receptor rearrangements (PARR) can also be useful in differentiating nodular hyperplasia and lymphoma ,28.There are many classification systems for canine lymphoid neoplasms, and modifications have been made frequently. Recently, the American College of Veterinary Pathologists Oncology Committee presented a report adapting the World Health Organization system for human lymphoma classification to canine lymphomas . There aSplenic stromal tumors are a heterogeneous group of mesenchymal neoplasms with a spindle cell morphology. Malignant stromal tumors are more common than benign tumors in dogs . StromalThere were two cases of myelolipoma. Myelolipoma is a rare tumor in the spleens of dogs. The origin of these masses is debated; while often considered a benign neoplasm, myelolipoma may also represent a metaplastic or hamartomatous process .There was one case of a splenic mast cell tumor. Obtaining a definitive diagnosis based on H&E was difficult because of the presence of additional inflammatory cell types, including hemosiderin-containing macrophages. Immunohistochemistry for C-kit aided the definitive diagnosis by confirming the presence of numerous mast cells within the lesion. The splenic mast cell tumor in our study could not be confirmed to be primary or metastatic, as the history and clinical findings were not provided. The lack of clinical data represents a limitation of this retrospective study.The distribution of canine splenic mass lesions outlined in the current study will help veterinary clinicians communicate with pet owners regarding prognoses, recommendations for splenectomy, and histopathological diagnoses. In this study, we evaluated canine splenic mass lesions in Republic of Korea, predominantly with samples from small-breed dogs. Since this study also included a small number of medium- and large-breed dogs, the findings here cannot be exclusively applied to small-breed dogs. Additional studies comparing the prevalence of splenic mass lesions between small- and large-breed dogs are needed, including using genotype-based breed groupings (as opposed to phenotype only).In the present study, we analyzed the histopathological diagnoses of 137 canine spleens in Republic of Korea for the first time. We also confirmed that using immunohistochemistry aids in obtaining definitive diagnoses of canine splenic tumors. The distribution of non-neoplastic lesions represented approximately 2/3 of the cases, which differed from what has been reported in studies from other geographical regions. These results should be validated within Republic of Korea. The goal of this study was to provide information that will aid veterinary clinicians in their communications with pet owners regarding prognoses and recommendations for splenectomy. Moreover, this study will facilitate additional investigations with more detailed comparisons of splenic mass lesions in small- vs. large-breed dogs or genotype-based groupings of breeds.


Dear Editor,Trichoblastomas (TB) are benign tumors derived from the hair follicle, with a low risk of malignant transformation, and are the most common neoplasm arising from nevus sebaceus of Jadassohn. They are neoplasms characterized by a biphasic differentiation into epithelium and the follicular germinative stroma. Histopathology shows a basaloid cells neoplasms in a stroma similar to follicular mesenchyme, well delimited, non-ulcerated, and restricted to the dermis, with numerous histopathological variants described.Melanotrichoblastoma is rare, with less than ten cases described in the literature; and it is different from pigmented trichoblastoma by the proliferation of intratumor melanocytes.A 59-year-old female patient presented an asymptomatic lesion that had been growing for one month on the scalp, without the presence of associated nevus sebaceus . DermoscThis is the sixth case of this neoplasm described in the current literature. When analyzing the clinical data of the previously described cases , one canFirst described in 2002,DermoscopyOne study evaluated BRAF, NRAS, KRAS, and HRAS gene expression, and found HRAS mutation in both a nevus sebaceus and the TB arising from it, indicating a possible common origin.There is one reported case in the literature of melanoma associated with melanotrichoblastoma, called trichoblastomelanoma, in a 62-year-old female patient, with a slow-growing lesion since childhood, measuring 8 cm, with associated axillary lymph nodes.The present case is similar to the cases previously described in the literature, of slow-growth tumors on the scalp, in a 59-year-old woman. However, it was not associated with nevus sebaceus or seborrheic keratosis, as previously described.It remains under discussion whether a melanotrichoblastoma would be a variant or a synonym of pigmented trichoblastoma, since only after the advent of immunohistochemistry it became possible to identify the presence and quantify melanocytes in tumor blocks. Melanotrichoblastoma highlights the strong association of epithelial and melanocytic cells present in the structure of the hair follicle.The interaction between keratinocytes and melanocytes in the onset of neoplastic lesions remains a little explored and a little known field, and the presence of lesions consisting of a mixture of these components indicates that it is important to better understand this relationship, even in a pathophysiological context.None declared.Juliana Polizel Ocanha-Xavier: Study design, investigation, follow-up and surgical excision, original manuscript drafting, review and editing.José Cândido Caldeira Xavier-Júnior: Investigation, histopathological analysis, manuscript writing, review and editing.None declared.


Uterine fibroids are a common, benign gynecological smooth muscle neoplasm highly prevalent in women of reproductive age which can lead to multiple complications including small bowel obstruction.We report a case of a 31-year-old female, primigravida at 13 weeks gestation, a known case of uterine subserosal fibroids who presented to the emergency with complaints of dark red vaginal bleeding and cramping abdominal pain. On examination, her abdomen was 38 weeks in size. An abdominal ultrasound showed intrauterine retained products of conception measuring 5x5 cm. She was admitted as a case of incomplete miscarriage and underwent an evacuation of retained products of conception (ERPOC) immediately.A post-procedure computed tomography (CT) scan done showed the presence of multiple large uterine fibroids. The patient continued to worsen clinically with complaints of abdominal pain and diarrhea. Further laboratory tests revealed a continuous rise in inflammatory markers with positive stool clostridium toxins. She was hence shifted to the intensive care unit (ICU) as a case of sepsis. In the following days, she developed signs and symptoms of small bowel obstruction, and the diagnosis was supported by abdominal X-rays. Despite starting her on conservative management for the same, she deteriorated clinically, and a repeat CT abdomen showed new signs of small bowel obstruction. The gynecology team performed an exploratory laparotomy during which a myomectomy was done. The patient recovered well post-operatively and was discharged in a stable condition.In view of the presented case, small bowel obstruction should be considered as a complication of uterine fibroids in females with a history of large leiomyomas despite it being quite rare as it can cause considerable morbidity and mortality. Uterine fibroids are common, benign smooth muscle tumors that typically present with abnormal uterine bleeding, pelvic fullness, and subfertility or infertility . HoweverA 31-year-old female, a known case of uterine subserosal fibroids with a term-sized abdomen, presented to the emergency at 13 weeks gestation with complaints of dark red vaginal bleeding, cramping abdominal pain, and the reported passage of a well-formed fetus followed by pieces of placenta one hour prior. She was a primigravida with spontaneous conception and no other significant medical or surgical history.On arrival, she was vitally stable except for tachycardia of 117 beats per minute. On physical examination, she appeared pale. Abdominal examination revealed a hard mass of irregular shape occupying the abdomen, with a symphysio-fundal height of approximately 36 cm. On digital vaginal examination, the cervical os was open and fresh bleeding was seen with products of conception felt in the os. Blood tests done in the emergency are summarized in Table She was diagnosed as a case of incomplete miscarriage and underwent an urgent evacuation of retained products of conception (ERPOC). The procedure was successful and completed with per rectal misoprostol 800 mcg insertion to control post-procedure bleeding. Post-operatively, she was vitally stable, she received two units of packed red blood cells (RBCs) to improve her pre-procedural hemoglobin and was started on dual antibiotics, doxycycline and metronidazole.Despite an initial improvement in her symptoms, on post-operative day 1, she developed worsening abdominal pain and distension. Biochemistry showed elevated inflammatory markers namely white blood cell count (WBC), C-reactive protein (CRP) and procalcitonin. Hence, a computed tomography (CT) scan was done due to suspicion of uterine perforation which showed multiple large uterine degenerated leiomyomas pushing the small and large bowels superiorly and laterally Figure , 1B. An Based on the CT findings of the left femoro-iliac vein thrombosis, she was started on a therapeutic dose of low-molecular-weight heparin, and vascular surgery was consulted who advised to continue anticoagulation with no other intervention required.In the following few days, inflammatory markers continued to increase, and the patient became tachypnoeic while complaining of worsening abdominal pain, diarrhea, and bilious vomiting. Laboratory investigations done to identify the source of infection revealed a positive stool clostridium toxins A and B test. A diagnosis of sepsis was established, and the patient was shifted to the intensive care unit (ICU) for optimal management.Antibiotic therapy was upgraded to metronidazole and meropenem. The vascular surgeon advised the placement of an inferior vena cava (IVC) filter if the patient was to be operated on. The patient failed to pass flatus for the next 24 hours. Physical examination revealed a distended abdomen with generalized tenderness to palpation and increased bowel sounds on auscultation. In view of her symptoms and physical exam findings, an abdominal X-ray was done that revealed the presence of multiple air-fluid levels with dilated bowel loops suggestive of small bowel obstruction Figure , 2B. TheDespite the advice of the gynecology team to perform an exploratory laparotomy and a total abdominal hysterectomy, the patient and her partner chose the fertility-preserving option of abdominal myomectomy instead. A large pedunculated fibroid weighing 3.5 kg with a large vessel on the surface and attached to the anterior left lateral uterine fundus was intra-operatively removed along with two other fibroids measuring 5 cm each Figure , 4B. TheUterine fibroids are a gynecological benign smooth muscle neoplasm with an incidence on the rise, affecting around 25% of women in their reproductive age while causing symptoms in around 25% of the patients . The patIncreased risk is identified in African descent, a positive family history, women older than 40 years of age, nulliparity, and obesity . While aThe clinical presentation is usually related to the location, type, and size of the mass. While history and physical exam guide adequately to the diagnosis, a significant amount of asymptomatic cases have resulted in transvaginal ultrasound imaging being considered the first and most sensitive diagnostic modality for this condition . With reManagement of uterine fibroids is multifactorial, depending on size, symptoms, type, and most importantly, desire for fertility. Conservative therapy revolves around hormonal manipulation in the form of Gonadotropin-releasing hormone (GnRH) agonists and GnRH antagonists, oral contraceptives, and progesterone receptor modulators alongside non-steroidal anti-inflammatory drugs (NSAIDs) and tranexamic acid. Surgical therapy encompasses myomectomy, uterine artery embolization, and lastly, hysterectomy . In the Despite bowel obstruction being a rare complication of uterine fibroids, it can be fatal if not promptly diagnosed and managed early, causing considerable morbidity and mortality. Diagnosis of this complication becomes even more challenging if the concerned fibroid(s) is large enough to occupy the abdominal cavity and displace the bowel. When dealing with such large fibroids, large vein thrombosis also needs to be considered and evaluated for as it may increase the morbidity of any surgical intervention. This condition needs to be addressed and managed adequately before any surgery is performed. Small bowel obstruction as a complication of uterine fibroids, therefore, requires better reporting in literature. More research and extensive studies are needed to have a better understanding of the nature of this condition.


We report the successful management of a paratesticular liposarcoma, which, to the best of our knowledge, is the largest known of its type. A 62-year-old male presented with a painless, gradually progressive left testicular “giant” mass measuring 60 × 40 cm, weighing 30 kg and growing over a period of three 3 years. Additionally, a 5 × 5 cm trophic ulcer could be seen at the bottom of the scrotum. The ultrasound of the left testis revealed the testis having been completely replaced with a cystic and solid tumour. Preoperative serum testicular tumour markers (STM) were within normal limits. The markers included Alpha Feto Protein, Beta Human Chorionic Gonadotropin and Lactose Dehydrogenase. A left sided high inguino-scrotal approach with a huge skin resection including the trophic ulcer with complete removal of the tumour and a primary complex closure of the wound was performed. The post-operative period was uneventful, and histopathology revealed a dedifferentiated liposarcoma. We believe social taboo and fear of disfigurement impart a sense of shame in patients which led to the delayed presentation in a hospital in the index patient. The absence of metastases even with a protracted course is surprising. Even though liposarcoma accounts for 20% off all sarcomas, it is rarely found in the para-testicular region. The spermatic cord is the most favoured location for paratesticular liposarcomas, which most often start growing just below the external inguinal ring and over time it presents as scrotal swelling rather than inguinal swelling . NeverthA 62-year-old male introduced himself in our urologic outgoing patient clinic with a painless giant swelling of the left scrotum that slowly progressed over a period of three years with occasional bleeding from the trophic ulcer at the bottom of the swelling. Clinically, the scrotum appeared pear-shaped and measured around 60 × 40 cm. A 5 × 5 cm trophic ulcer could be seen at the bottom of the scrotum, which at the time of presentation was not bleeding. His only pre-existing disease was bilateral varicose veins. Additionally, the penis was buried within the scrotal mass Fig. .A physical examination of the right testis, as well as an examination of the penis, could not be performed due to the left testicular mass. These organs could only be found by ultrasound. The ultrasound of the left testis showed an enormous cystic, solid tumour replacing the left testis completely. Preoperative clinical examinations and postoperative staging computed tomography (CT) did not show metastatic spread, but a few enlarged lymph nodes in the bilateral inguinal areas and the right iliac area, each measuring approximately 2 × 1 cm. Preoperative STM were within the normal range. A left-sided high inguinal orchiectomy with complete removal of the tumour and a primary complex closure of the wound was performed (Picture 1). Intraoperatively, the right testis was successfully spared. Post operative pathological examinations diagnosed a dedifferentiated liposarcoma (G2 according to FNCLCC) from the left spermatic cord with R0-resection  and histiocytoma 13%) [9% and hi% [9% andSurgical removal of the tumour is the unanimously accepted first tier of the treatment. Currently, there are no widely accepted adjuvant treatment protocols for paratesticular liposarcomas. The role of prophylactic lymph node dissection remains unclear. Proponents of lymphadenectomy enunciate the need of the same in almost 29% of cases where metastases could be found in the regional lymphatics . Althoug


The report urges considering rare neoplastic tumor, like IMT, in intussusception cases and underscores the vital role of comprehensive and swift diagnosis in influencing treatment choices and patient results.We present a case of a 36‐year‐old male who presented with abdominal pain and vomiting. Inflammatory markers were elevated in routine investigations, while other laboratory parameters were within normal limits. Ultrasound imaging revealed a target lesion, which required further evaluation with a CT scan and confirmed ileocolic intussusception. An exploratory laparotomy showed a solid lesion measuring 5 by 6 cm in the cecum, along with evidence of ileocolic intussusception. A standard right hemicolectomy was performed, and the postoperative course was uneventful. Histopathological examination showed an inflammatory myofibroblastic tumor with nodular hyperplasia. The report highlighted the importance of assessing rare neoplastic causes in patients with intussusception. Singly scattered spindle fibroblastic cells along with stellate cells and ganglion like polygonal cells intermingled with acute and chronic inflammatory cells. The pain started from the right lower quadrant and later generalized. On physical examination, mild tenderness was present on deep palpation in the right lower quadrant of the abdomen. The patient had no significant medical history and denied any recent weight loss or changes in bowel habits.Laboratory investigations revealed raised inflammatory marker. A complete blood count revealed elevated WBCs 17,100/μL) with neutrophilia (80%). Blood urea nitrogen and creatinine level were normal and other hematological and biochemical parameters were within normal limits. An ultrasound examination of the abdomen showed a target lesion in the right lower quadrant, raising the suspicion of intussusception. A subsequent CT scan confirmed segmental, circumferential thickening of the terminal ileocolic junction showing a target stratification pattern  the patient was transferred to the surgery ward. He recovered well and discharged after fifth postoperative day.Histopathological examination of the resected specimen showed an inflammatory myofibroblastic tumor. The tumor showed nodular hyperplasia with diffuse eosinophilic infiltrate, subserosal bland spindle to stellate cells proliferation along with lymphoid aggregates, lymphocytes, plasma cells, and eosinophils. Figures , 6.3Inflammatory myofibroblastic tumors being rare neoplasms with diverse clinical presentations can affect various organs like lungs, liver, endolarynx, maxillary sinus, oral cavity etc.Some common causes of adult ileocolic intussusception are adhesions, polyp, inflammatory bowel disease, gastro intestinal tumor (GIST), leiomyosarcoma etc.In our case, the patient's presentation with abdominal pain, vomiting, and imaging findings of intussusception prompted further investigation, leading to the diagnosis of an IMT. Surgical resection followed by histopathological examination of the resected specimen performed.Immuno histochemical staining (IHC) which helps to identify specific marker expressed by tumor cells like smooth muscle actin (SMA), anaplastic lymphoma kinase (ALK Protein), vimentin, and CD34. These IHC features can help in distinguishing benign or malignant nature of the tumor. Together in conjunction with histopathological features, they can help to establish confirmatory diagnosis. However, in suburban area like ours, patient presents with their complaints and after getting relief from their issues, they do not come up to hospital for follow up. Therefore, in our case we are unable to give immunohistochemistry (IHC) reports as he was lost to follow up.Further studies, research and case reports are necessary to understand the etiology, behavior, and optimal management strategies for IMTs, particularly those involving gastrointestinal tract. Additionally, increased awareness among clinicians is also essential to ensure early recognition, prompt diagnosis, and appropriate surgical intervention, as demonstrated in this case, are crucial for optimal management and favorable patient outcomes of these rare tumors.4This case report shares a very rare condition of inflammatory myofibroblastic tumor (IMT), presenting with ileocolic intussusception in a middle‐aged man, which is extremely uncommon and can be confused with other inflammatory conditions; however, through diagnostic evaluation and multidisciplinary approach can result in early diagnosis, optimal management, and favorable patient outcomes of these rare tumors.Nischal Khanal: Conceptualization; data curation; formal analysis; resources; supervision. Rupak Subedi: Data curation; formal analysis; investigation; software; writing – review and editing. NIrajan Shrestha: Conceptualization; project administration; resources; supervision. Shristi Shrestha: Conceptualization; resources; writing – review and editing.There are no conflict of interest regarding this manuscript.Please note that due to patient confidentiality and privacy considerations, access to specific clinical information may be subject to approval by our institution's ethics committee and may require compliance with relevant data protection regulations.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.


Zinner syndrome is a rare developmental anomaly of the distal Wolffian duct. It is characterized by a triad of unilateral renal agenesis, cysts in the ipsilateral seminal vesicle, and ipsilateral obstruction of the ejaculatory duct. While some patients are asymptomatic and diagnosed incidentally, other patients may present with symptoms related to obstructed ejaculatory ducts and seminal vesicle cysts. We report a unique case of a 32-year-old male who presented with pelvic pain for three days. Zinner syndrome is a rare congenital urogenital developmental anomaly. The embryologic abnormality develops before the 7th gestational week in the distal portion of the mesonephric or Wolffian duct. This congenital maldevelopment is characterized by a triad of renal agenesis , ipsilateral obstruction of the ejaculatory duct, and cysts in the ipsilateral seminal vesicle. Patients may present with dysuria, increased urinary frequency, painful ejaculation, and epididymitis . However, some patients are asymptomatic and diagnosed incidentally . Here, wA 32-year-old male with no significant past medical history presented to the emergency department with complaints of pelvic pain for the last three days. He reported associated symptoms of dysuria and difficulty urinating but denied any urgency, frequency, painful ejaculation, or hematospermia. He reported being sexually active but had no diagnosed history of infertility. A physical exam revealed mild suprapubic tenderness.On work-up, complete blood count and comprehensive metabolic panel were within normal limits. Urinalysis was unremarkable. Ultrasound was negative for testicular torsion. Computed tomography (CT) abdomen/pelvis revealed left renal agenesis Figures , 1B.Magnetic resonance imaging (MRI) of the pelvis showed the right kidney to be normal with a normal collecting system and no calculus. There was left renal agenesis noted however with a 7.6 cm left seminal vesicle cyst communicating with a left retroperitoneal structure suggesting ectopic ureteral insertion Figures , 2B.There was non-visualization of the left ductus deferens. Cystoscopy and retrograde pyelogram were performed which showed a cystic mass, trabeculations and cellules on the bladder indicating bladder outlet obstruction. Transrectal ultrasound revealed similar findings Figures , 3B.The patient was treated with robotic left seminal vesicle excision with excision of the left ureter and atrophic left kidney. Intraoperative findings confirmed the diagnosis of Zinner syndrome.Zinner syndrome was first described in 1914 . Since tA pooled analysis published in 1998 by Van den Ouden sought to provide a review of common symptoms for clinical reference. This study found the most common presentation to be dysuria 37%), followed by increased frequency (33%). Perineal pain was found in 29% of patients, epididymitis in 27%, pain after ejaculation in 21%, and scrotal pain in 13%. 17% of patients suffer from infertility 7%, follo. The patAppropriate imaging modalities are essential in the evaluation of complex development anomalies of the urogenital tract. Differential diagnoses, such as ejaculatory duct cysts, ureterocele, acquired seminal vesical cysts, or prostatic utricle cysts, could be ruled out based on imaging findings and patient history. The most frequently used imaging modalities for diagnosis were magnetic resonance imaging (MRI) 67.8%) and ultrasonography (65.0%) . On ultr.8% and uZinner syndrome is a rare congenital urogenital developmental abnormality. The incidence is low and clinical presentation commonly includes urinary symptoms, epididymitis, and painful ejaculation. Our case illustrates an atypical presentation of Zinner syndrome and highlights the importance of holding a high index of suspicion in appropriate clinical settings. A careful review of imaging is crucial as it is the primary modality of diagnosis and misdiagnosis can lead to adverse outcomes like infertility at a young age.


Staghorn calculi (SC) are defined as large kidney stones that fill the renal pelvis and at least one renal calyx. They represent 10–20% of all renal stones in developing countries and require prompt diagnosis and management. Massive SC (over 5 cm) are treated exclusively via open surgery, despite percutaneous nephrolithotomy (PCNL) being the gold standard treatment for large stones. Descriptions of PCNL for massive SC are very limited in the literature.We report a case of a 63-year-old male who presented with chronic abdominal pain, hepatosplenomegaly, and normal renal function. He was later diagnosed with polycythemia vera. Computed tomography of the abdomen revealed massive, bilateral staghorn stones measuring 7.3×5.5 cm and 1.8×4.5 cm on the right and left, respectively. Additionally, the right stone was found to be compressing the inferior vena cava (IVC). The patient was promptly scheduled for right-sided PCNL and the target of 80% stone fragmentation was successfully attained.We present the first case of a stone of such size in the Middle East, as well as the first known case of a renal stone compressing the IVC. Unlike previous reports, the stone was successfully fragmented via PCNL – a procedure that has not been described for stones of such size.This report highlights that ultrasound-guided PNCL without any other intervention is sufficient for the successful treatment of giant SC. Greater research is needed on the potential utility of using ultrasound-guided PCNL for the fragmentation of stones sized over 5 cm. HighlightsStaghorn calculi (SC) are large kidney stones that fill the renal pelvis and at least one renal calyx.Due to their significant morbidity and mortality, prompt treatment and diagnosis of SC is vital.We report the first case of a staghorn stone found to be compressing the inferior vena cava.Current guidelines recommend the use of open, laparoscopic, or robotic surgery for the treatment of very large or bilateral SC.Ultrasound-guided percutaneous nephrolithotomy without any other intervention was successfully utilized for the treatment of this stone.1. Therefore, SC are usually associated with chronic urinary tract infections, alkaline urine, and urease-producing bacteria. Struvite stones account for up to 30% of urinary tract stones worldwide2. Particularly, struvite stones represent 10–20% of the urinary stone burden in developing countries, but only 4% of the burden in developed countries, due to improved diagnosis and management4. While the exact prevalence of struvite stones in the Middle East is unknown, it is estimated that the general occurrence of urolithiasis is higher in this region as compared to rest of the world – likely due to the combination of several risk factors unique to the area, such as hot and dry weather, dietary habits, and obesity5. Struvite stones are also twice as common in women than in men, despite the prevalence of renal stones being higher in men overall4. Most SC are unilateral, with only 15% of cases being bilateral6.Staghorn calculi (SC) are defined as large kidney stones that fill the renal pelvis and at least one renal calyx. They are typically radiopaque and composed of struvite; it is worth noting that struvite stones only form under conditions of increased ammonia production, which results in elevated urine pH7. Due to their significant morbidity and mortality, timely diagnosis and management of SC is crucial. While large renal stones are usually managed via percutaneous nephrolithotomy (PCNL), it is recommended that bilateral SC or stones of unusual size be managed exclusively via surgical intervention – namely open, laparoscopic, or robotic surgery9. Additionally, conservative treatment of staghorn stones has been shown to carry higher mortality10. Herein, we report a case of a giant staghorn stone sized over 7.0 cm×5.0 cm, which was successfully managed via ultrasound-guided PCNL. Additionally, this staghorn stone was found to be compressing the inferior vena cava (IVC), possibly causing associated hepatomegaly – the first to be reported in the literature. This case report is reported in line with the SCARE  criteria11.Other known risk factors for SC include female gender, neurogenic bladder, indwelling Foley catheters, or urinary diversionsA 63-year-old Saudi male presented to our clinic with a complaint of chronic abdominal pain and new-onset flank pain. The patient was alert, oriented, and vitally stable. A physical examination was conducted, and hepatosplenomegaly was noted; however, no ascites or jaundice were identified. Mild tenderness was noted in the right upper quadrant, along with slight lower extremity edema. All other physical examination findings were unremarkable. The patient was admitted, and intravenously (i.v.) paracetamol was administered.12. After a comprehensive workup, a JAK2-positive diagnosis of polycythemia vera was made. Consequently, the patient was started on hydroxyurea, followed by venous thromboembolism prophylaxis. A noncontrast computed tomography (CT) scan of the abdomen was performed, and bilateral staghorn kidney stones were identified, measuring 7.3×5.5 cm on the right  and 1.8×4.5 cm on the left , an elevated hematocrit of 57% , and increased red cell mass at least 25% greater than the mean normal predictive valueIntraoperatively, the patient was placed in a prone position under general anesthesia. A flexible cystoscope was inserted through the urethra, and the right ureteric orifice was identified. This was instrumented using a solo guidewire, and the coiling around the renal pelvis was visualized. After this, a 5-French (Fr) ureteric catheter was advanced over. Then, a 16-Fr Foley catheter was inserted, and both the Foley and ureteric catheters were secured. The stones were localized after injecting contrast material, and the guidewire was advanced. The puncture site was at the lower pole subcostal accesses  was also responsible for the hepatosplenomegaly seen in this case.To date, the largest renal stone ever described is one measuring 13 cm, which was successfully removed via transperitoneal laparoscopic nephrectomy15. On the other hand, the enlarged liver and spleen seen in this patient may also be the result of increased cell turnover, as caused by polycythemia vera. Budd–Chiari syndrome (BCS) – a disorder that occurs due to hepatic vein and/or IVC obstruction secondary to a chronic myeloproliferative neoplasm (e.g. polycythemia vera) – is another potential cause. Budd–Chiari typically presents with three findings: abdominal pain, tender hepatomegaly, and ascites. While our patient only fits two of these, it appears that JAK2 mutations are, in fact, detected in 35–50% of patients with Budd–Chiari, making it a credible diagnosis16. Importantly, however, is that the baseline renal function tests and liver function tests were also normal. It is unclear to what extent the patient’s hepatosplenomegaly may have been caused by IVC compression. Previous cases of IVC compression in literature list thrombus formation, tumor extension, and extrinsic compression as other causes of IVC compression17. Additionally, the symptoms reported in these cases include ascites, anasarca, and swelling of the lower limbs, while our patient only presented with slight lower limb edema – another unusual aspect of this case18. While the exact cause of hepatosplenomegaly is undetermined, significant conclusions may be drawn from this case to aid in future research. Greater studies are required on the possibility of SC causing IVC obstruction.On the one hand, kidneys are retroperitoneal organs, with the right renal vein directly draining into the IVC. In theory, a large staghorn stone located in the right renal calyces may expand to apply extrinsic pressure on the IVC, eventually reducing blood flow, causing backward congestion of the liver sinusoids and splenic blood vessels, and ultimately hepatosplenomegaly21. Surprisingly, however, our patient was successfully treated with only PCNL. While such a case regarding a stone of this size that was treated by PCNL only – particularly one compressing the IVC – has not been reported, Gavande and Gavande22 discuss a case of a 10 cm×7.5 cm stone, also successfully managed via PNCL. However, the authors admit to performing calyceal puncture, which was evaded in our patient. Greater research is needed on the potential utility of using ultrasound-guided PCNL for the fragmentation of stones sized over 5 cm.Several authors have recommended an invasive surgical approach for the management of massive SC of such proportionsSC are growing to become exceedingly common, causing great financial and economic strain. In this case report, we present a unique case of IVC compression secondary to a massive staghorn stone seen in a 63-year-old male with polycythemia vera. To our knowledge, this is the first stone of such size to be reported in the region, as well as the first to be discussed in the context of IVC compression and hepatosplenomegaly. We hope this report elucidates the role of minimally invasive surgical procedures in the fragmentation of massive SC, as well as the association between SC and IVC compression. We highlight that ultrasound-guided PNCL without any other intervention is sufficient for the treatment of such stones, suggesting this strategy may be applicable to other massive stones.Ethical approval is exempted in the case of a case report as per the King Faisal Specialist Hospital and Research Centre.Written informed consent was obtained from the patient for the publication of this case report and accompanying images. Patient anonymity is maintained throughout this manuscript. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.This study did not receive funding from any source.M.A.A., S.A.R., A.A., and M.T.A.: conceptualization; M.A.A., A.A., and T.A.: supervision; S.A.R., H.M.R., B.N.S., A.E.M., M.I.E.S., and A.E.A.: writing – original draft; M.A.A., S.A.R., B.N.S., A.E.M., M.I.E., A.E.A., A.A., T.A., M.T.A., and M.A.: writing – review and editing. All authors reviewed the manuscript for intellectual content and approved the submission.The authors declare no conflicts of interest.Mohammad A. Alghafees, MD.Not commissioned, externally peer-reviewed.None.


In the past, eye tumours were generally not assessed with magnetic resonance imaging (MRI) due to low image quality. MRI of the eye has significantly improved in the last decade and is, therefore, used more and more to visualise the tumour and surrounding structures. This review provides an overview of how MRI can be used to improve the clinical care of patients with a uveal melanoma, the most common eye tumour in adults. MRI can help in diagnosis, because it provides anatomical and biological information which cannot be attained by the conventional ophthalmic techniques. Dimension measurements on MRI are generally in agreement with ocular ultrasound, but MRI is more reliable when the tumour is located in the anterior part of the eye. Additionally, MRI can provide information about prognosis and treatment response, without performing a biopsy.Conversely to most tumour types, magnetic resonance imaging (MRI) was rarely used for eye tumours. As recent technical advances have increased ocular MRI’s diagnostic value, various clinical applications have been proposed. This systematic review provides an overview of the current status of MRI in the clinical care of uveal melanoma (UM) patients, the most common eye tumour in adults. In total, 158 articles were included. Two- and three-dimensional anatomical scans and functional scans, which assess the tumour micro-biology, can be obtained in routine clinical setting. The radiological characteristics of the most common intra-ocular masses have been described extensively, enabling MRI to contribute to diagnoses. Additionally, MRI’s ability to non-invasively probe the tissue’s biological properties enables early detection of therapy response and potentially differentiates between high- and low-risk UM. MRI-based tumour dimensions are generally in agreement with conventional ultrasound (median absolute difference 0.5 mm), but MRI is considered more accurate in a subgroup of anteriorly located tumours. Although multiple studies propose that MRI’s 3D tumour visualisation can improve therapy planning, an evaluation of its clinical benefit is lacking. In conclusion, MRI is a complementary imaging modality for UM of which the clinical benefit has been shown by multiple studies. Although magnetic resonance imaging (MRI) has been used to image uveal melanoma patients since its introduction in the 1980s , it was For uveal melanoma (UM) patients specifically, multiple clinical applications of ocular MRI have been proposed ,28,29,30With MRI emerging as a clinically valuable imaging modality for the eye, it is important to understand its strengths and limitations from an ophthalmic perspective, especially since a high level of care has already been attained with the conventional techniques. However, as most ocular-MRI studies are described from a radiological or physics perspective, their implications for ophthalmic clinical practice are often not fully explored. Therefore, we systematically reviewed the ocular MRI literature from an ophthalmic perspective, with particular attention to the clinical implications of MRI for patients with uveal melanoma. In this review, we will describe the characteristics of UM on MRI, followed by possible clinical applications of MRI in the differential diagnosis, treatment planning and follow-up of these patients. Finally, these evaluations will be combined into indications for ocular MRI in the care of patients with an intraocular mass. (1) AND (“magnetic resonance imaging [MeSH] OR “MRI”)This systematic review was carried out according to the PRISMA 2020 statement . PubMed In Rayyan , all recIn this review, data from several studies were combined for quantitative MRI measures of the tumour perfusion and diffusivity ,43,44,45Modern ocular MRI scans are preferably performed on a 3T MRI scanner with a surface coil as the increased signal to noise ratio delivers a higher diagnostic value compared to scans acquired with a head coil or a 1.5 tesla scanner ,21,48. TIn MRI, multiple images with different contrasts are generally jointly evaluated. These different contrasts provide the complementary information needed to distinguish different pathologies such as intraocular masses, associated retinal detachment, necrosis and other treatment related effects ,56,57,58The signal intensity characteristics of UM on these types of MR-images have been described extensively ,74,75,76One of the advantages of MRI compared to ultrasound and fundoscopy is that the complete orbit can be evaluated. This allows for a more accurate screening for extrascleral extension, optic nerve invasion and inflammatory processes ,93,94,95In addition to these anatomical assessments, functional MRI can provide multiple types of quantitative information on function and biology of an intraocular mass ,99,100. Similar to fluorescein angiography, in PWI, an image is acquired every few seconds during and after the administration of an intravenous contrast agent A,B. Currn = 51) . S. S28]. SThe second commonly used functional MRI technique in oncology is DWI, a technique which assesses the diffusion  of water molecules within a tissue. This diffusion, quantified as an apparent diffusion coefficient (ADC) , is a re−3 mm2/s [−3 mm2/s ; 14.7 mm vs. 14.0 mm). The full tumour extent was more often not visible in anterior tumours compared to posterior tumours , which resulted in an increased median absolute difference in prominence of 0.2 mm and LBD of 1.2 mm, respectively (In general, a good agreement between ultrasound and MRI-based tumour dimensions is found  30,32,33,46,47. ectively C, with bAlthough several studies have shown the feasibility of fully three-dimensional MRI-based therapy planning ,151,152,Furthermore, two studies have shown the feasibility of verifying brachytherapy plaque position with 1.5 tesla MRI ,158. HowA reduction in tumour prominence or volume, as obtained by ultrasound, is clinically one of the primary signs of therapy response ,160,161.Several studies showed that MRI can be used to quantify the reduction in tumour size after treatment ,164,165.Different studies propose that functional MR-imaging can provide early biomarkers of therapy response ,44,46. FFinally, exudative retinal detachment and sick tumour syndrome, a known side effect of irradiation in patients with an intraocular mass, is sometimes treated with vitrectomy with tumourectomy and silicon oil tamponade. As the silicon oil prevents accurate ultrasound imaging, follow-up with conventional ophthalmic imaging is hindered. Monitoring these patients with MRI has been reported to prevent secondary enucleation by differentiating local tumour recurrence from scarred tumour residue . In recent years, different MRI techniques dedicated for imaging the eye and orbit have become clinically available, enabling the acquisition of high quality MR-images of the eye in regular clinical care. As a result, an increasing number of ophthalmic applications of MRI has been proposed in the last five years, especially related to ocular oncology. This increase can also be observed in the scientific literature, where the number of ocular oncology-related MRI-studies increased from 55 in 2000–2005 to 151 for the last 5 years. Moreover, since the radiological characteristics of the most common primary ocular tumours, UM and retinoblastoma, have now been extensively described, MRI can provide a similar contribution to the care for ocular oncology patients as it does for patient groups with other tumours.From an imaging point of view, the eye is a relatively unique organ as it can be accurately imaged with optical techniques. Conversely to other tumour sites, adequate care can often be provided for intraocular masses without radiological imaging, as the ophthalmic imaging modalities provide sufficient information for its diagnosis, treatment and follow-up ,28. NotwAlthough fundoscopic and ultrasonic imaging are generally sufficient for accurate diagnosis of intraocular masses, in various situations they cannot provide a definite diagnosis, such as for lesions behind the iris, and in cases with an accompanying vitreous haemorrhage or dense cataracts. Now, the anatomical and functional characteristics of a lesion on MRI can aid in the diagnosis for these cases, instead of an invasive biopsy. For a reliable evaluation of the MR-images, it is, however, critical that the radiologist is sufficiently acquainted with the appearance of intraocular masses on MRI, especially since not all radiological characteristics of all types of intraocular masses are known, nor have a 100% specificity. As a result, definite diagnoses based on MRI alone can be challenging. However, as a comprehensive radiological description of UM is available, a differentiation between UM and other masses is, in our experience, often possible, especially in a combined evaluation with ophthalmic data. In this context, communication between the radiology and ophthalmology professions is vital. In our experience, an MRI can also be beneficial in an atypical presentation of a mass, for example, for a very young patient with a lesion which, on ophthalmic imaging, appears as a UM. While especially the functional MRI measures can provide an independent confirmation of the diagnosis, we have also seen different cases where these biomarkers combined with MRI’s visualisation of the mass’s internal structures, point towards a different diagnosis, which was then later confirmed through biopsy. In general, MRI can be used as an additional diagnostic tool, especially for atypical presentations of intraocular lesions, if visualisation is impossible with conventional ophthalmic imaging methods or in situations where the diagnosis is uncertain. For the treatment selection of intraocular masses, the benefit of MRI mainly depends on the type of lesion and the available treatment options. For example, in retinoblastoma, the main goal of the MRI is diagnostic confirmation and detection of local tumour extent . AlthougGiven the relatively the set dimensions of ruthenium plaques used in ocular brachytherapy, an MRI is generally of limited value for treatment planning purposes in ruthenium brachytherapy. When treating with COMS iodine plaques, which allow for a customisation of the spatial dose profile , MRI couCompared to the diagnosis and treatment of intraocular masses, the value of MRI in the follow-up after treatment has been studied less extensively. Conventionally, a reduction in tumour prominence is considered the primary sign of therapy response. UM treated with brachytherapy generally show a rapid reduction in prominence, making the use of MRI for follow-up of little clinical value for these patients. For patients treated with ocular proton therapy, however, such a reduction in prominence can take up to a year to become apparent, suggesting that an alternative biomarker of therapy response could be of value. Until present, only two studies have prospectively assessed MRI in the follow-up of these patients ,46. AlthWith the advent of new treatments for specific types of metastatic spread of UM ,171, MRIAlthough performing an MRI-scan has proven to be cost-effective for specific indications, such as for treatment decision-making in tumours with an intermediate size ,172, theWith the advances of ocular MRI, a new way of visualising the eye and orbit has emerged which complements the information provided by the more commonly used fundoscopic and ultrasound imaging. MRI can aid differential diagnosis for atypical presentations of intraocular lesions, especially when visualisation is impossible or limited with conventional ophthalmic imaging methods. For these patients, the additional information provided by MRI might mitigate the need for an intra-ocular biopsy. Furthermore, when the tumour thickness and basal diameters make the patient eligible for multiple treatments, the three-dimensional visualisation of MRI can aid treatment decision making. Furthermore, we advise performing an MRI if optic nerve invasion or an extrascleral component is suspected. Finally, first evidence has been provided of the benefit of including MRI for ocular proton beam therapy planning and the use of perfusion-weighted MRI as an early biomarker for treatment response assessment.


Pheochromocytomas are rare tumors that arise from the sympathetic ganglia or adrenal medulla and secrete catecholamines that are known for the classic triad of headaches, profuse sweating, and paroxysmal hypertension. However, there have been instances of asymptomatic patients found to have a pheochromocytoma. Adrenal incidentalomas are accidentally discovered through radiologic imaging, and subsequent testing can confirm a pheochromocytoma. Here, we present a case of a 67-year-old female found to have an adrenal incidentaloma on kidney ultrasound (US) after presenting to the emergency room (ER) due to nausea. She had minimally elevated creatinine at the ER. At her follow-up with her primary care provider (PCP), a kidney US was ordered and showed a probable right suprarenal mass. Further abdominal computed tomography (CT) and abdominal magnetic resonance imaging (MRI) showed that the mass was indeed adrenal, but its etiology is considered indeterminant. Although asymptomatic, further biochemical tests showed elevated serum and urine metanephrines and normetanephrines. Together, these findings revealed that the adrenal mass was likely a silent pheochromocytoma. She underwent a successful right adrenalectomy with pathology confirming pheochromocytoma. This case adds to the literature on the existence of silent pheochromocytomas and highlights the importance of following up on any abnormal findings with a PCP. This patient, although asymptomatic from her pheochromocytoma, needed surgery to prevent possible pheochromocytoma crises, which could cause cardiovascular complications and even death. Pheochromocytomas are rare catecholamine-secreting tumors that arise from chromaffin cells from either the sympathetic ganglia or adrenal medulla. The release of catecholamines, such as epinephrine, is what causes the classic triad of headaches, profuse sweating, and sustained or paroxysmal hypertension seen in patients with pheochromocytomas . Other lA 67-year-old female with a past medical history of hypertension, high cholesterol, prediabetes, hyperlipidemia, degenerative joint disease, and questionable transient ischemic attack (TIA) presented to the emergency room (ER) due to nausea that started in the morning when she got up. She denied vomiting or abdominal pain. Since the nausea persisted, she sought care at the ER. She had never had anything similar to this before. Her chronic hypertension was well-controlled, and her questionable TIA was being followed by a neurologist. She had occasional, sporadic episodes of “weird” feelings in her right hand lasting for three seconds. She had been feeling well otherwise, given all her chronic conditions. She denied any flushing, diaphoresis, or tachycardia. The patient willingly consented to this case report so that the medical profession can learn about her silent pheochromocytoma. Table 2. Cardiac, pulmonary, and abdominal physical exams were unremarkable.The general appearance shows an obese female in no acute distress. The vital signs are as follows: blood pressure 134/79 mm/hg, pulse 66 beats per minute, temperature 98°F, respiratory rate 14 breaths per minute, and BMI 33 kg/mInitial laboratory workup revealed hypokalemia of 2.6 mmol/L, elevated non-fasting glucose at 152 mg/dL, and creatinine of 1.1 mg/dL, which increased from the patient’s baseline of 0.93 mg/dL (Table The abnormal findings on kidney US prompted further evaluation, and an abdominal CT scan was ordered. The imaging identified a left adrenal mass consistent with fat density and a mass in the right adrenal gland of indeterminant density Figure . Lab worDuring the patient’s endocrinologist visits, she denied any striae, bruising, dizziness, diaphoresis, tachycardia, or swelling. An abdominal MRI further showed a 1.8 cm left adrenal adenoma and 2.2 cm right indeterminate adrenal nodule Figure . A meta-A right laparoscopic adrenalectomy was performed with the removal of the right adrenal gland and 2.2 cm right adrenal nodule Figure . PatholoPheochromocytomas are rare tumors of the adrenal gland that arise from chromaffin cells and secrete catecholamines. Classically, pheochromocytomas present with symptoms, including headaches, profuse diaphoresis, and paroxysmal hypertension. A prospective study to estimate the incidence of pheochromocytomas in Spain from 1980 to 1992 found that the incidence during the period was 2.06 per a million . LikewisAlthough we associate this tumor with the triad of headache, sweating, and tachycardia, there has been an increase in the diagnosis of pheochromocytomas in asymptomatic patients. The patients have unrelated symptoms and imaging leads to the discovery of an adrenal mass or incidentaloma. Studies done have shown a significant amount of adrenal incidentalomas being pheochromocytomas ,9,12,13.One concern with unrecognized pheochromocytomas is the possibility of a hypertensive crisis or hypertensive emergency. There may be associated metabolic derangements, coagulopathy, thromboembolic events, adrenal capsule rupture risk, hemodynamic instability, and end-organ dysfunction with a pheochromocytoma crisis . Our patThe treatment of choice for pheochromocytomas is surgical excision of the tumor . HoweverOur patient initially presented to the emergency room with nausea and found to have hypokalemia. There could be several reasons for this. It has been previously reported that increased levels of epinephrine may be related to episodes of hypokalemia, and there have been a few instances of patients with pheochromocytomas that have presented with hypokalemia ,21. SincThis case exemplifies the importance following up the results from the ER. The meticulous attention to detail with follow-up imaging led to the diagnosis of a silent pheochromocytoma. A potential limitation to this case is that we could have measured the patient’s catecholamine levels. It is difficult to attribute her hypokalemia to epinephrine release without having this value. Of course, hydrochlorothiazide is a well-known cause of hypokalemia. We also acknowledge that we have only presented one case of a silent pheochromocytoma as opposed to having a series of patients.We present a case of a patient to highlight an incidentally discovered silent pheochromocytoma. This case adds to the literature on the importance of following up on minimally abnormal labs found in the ER and reviews the essential workup for an adrenal incidentaloma. The minimally elevated creatinine led to a renal US that discovered a possible adrenal mass. Thereafter, an abdominal CT and MRI were done, and workup for the adrenal incidentaloma was begun. The pheochromocytoma was confirmed on biochemical labs, which revealed elevated metanephrines and normetanephrines, upon which she had a subsequent resection through a right adrenalectomy and pathologic confirmation of a pheochromocytoma. Our patient could have had a pheochromocytoma crisis at any time even though she was asymptomatic. Now, we recommend that the patient should have continued follow-up screening of plasma or urinary metanephrines and normetanephrines annually to monitor for recurrence or metastasis for at least 10 years after the removal of her pheochromocytoma. It is important for PCPs to carefully review any ER or discharge records from their patients and complete appropriate follow-up testing. Many times, no further testing is required. However, sometimes, you may make a rare diagnosis, such as a pheochromocytoma.


Rosai Dorfman disease is generally defined as a massive bilateral painless cervical lymphadenopathy accompanied with both fever and leukocytosis with neutrophilia. Additionally, it may possibly be associated with polyclonal hypergammaglobulinemia, reversal of CD4/CD8 ratio, the elevated erythrocyte sedimentation rate (ESR), microcytic anemia, and thrombocytosis. Rosai-Dorfman disease is known as a benign self-limiting disease, so no treatment is required in many cases, although it causes death in some cases by involving vital organs like kidney. The treatment is required when there is a life-threatening situation such as airway obstruction or involvement of vital organs such as kidney, liver, and lower respiratory tract. The required treatment choices include steroid therapy, chemotherapy, radiotherapy, and surgery. Surgical treatment is performed for bulk removal to resolve the obstruction caused by the mass as well as taking biopsy for the definite histopathologic diagnosis of disease. A 26-year-old man was referred to oral and maxillofacial surgery (OMFS) clinic of Taleghani hospital with chief complaints of pain and swelling of left submandibular space. According to the patient himself, the swelling had been started three months earlier. After rejecting dental source of the lesion, we decided to remove the mass by excisional biopsy concerning the patient’s discomfort. Histopathology report verified Rosai Dorfman disease as definite diagnosis of the mass. Sinus histiocytosis with a massive lymphadenopathy (SHML) was firstly introduced and described by Rosai and Dorfman in 1969. Accordingly, it is also known as Rosai-Dorfman disease (RDD)  . This dIn about 43% of cases, RDD occurs in extra nodal form without any lymphadenopathy making this disease hard to be differentiated from other similar conditions  - 18. OThe characteristic of histopathologic finding for RDD is emperipolesis that is phagocytosis of lymphocytes, plasma cells, erythrocytes or polymorphonuclear (PMN) leukocytes by histiocytes, which is distinguished by large histiocyte-like cells, called Rosai-Dorfman (RD) cells  , 3. ThA 26-year-old man was referred to Oral and Maxillofacial Surgery Clinic of Taleghani hospital with chief complaints of pain and swelling of left submandibular space.According to the patient himself, the swelling had been started 3 months earlier . He declared no former history of swelling or tooth pain in this area. After visiting a general dentist, Antibiotics were prescribed and used, and swelling disappeared after a week. After about 2 weeks, the swelling reoccurrence was observed at the same place. Thereafter, the extraction of left mandibular wisdom teeth was performed; however, the swelling did not decrease in size. The patient visited ENT faculty because of a mass observed in nasal passage and subsequently a fine needle aspiration (FNA) was performed for him. The pathology report showed a chronic inflammation associated with fibrous granulation reaction. The patient represented history of fever and weight loss in the past 2 months. Moreover, he had no history of smoking, but he stated alcohol usage occasionally. As well, he had a history of asthma when he was 15 years old. His family had no history of the same problem. Intra oral examination did not show any dental or mucosal defects. The wisdom tooth socket had shown normal healing process. Hematological tests such as complete blood check (CBC); blood sugar profile and biochemistry tests had shown normal values and no considerable abnormalities were detected.In computed tomography (CT) scan of head and neck regions , two enhIn 1969, Rosai and Dorfman have introduced a newly recognized benign entity and later, they introduced more cases with this disorder and called it RDD  . The moSHML can definitely be diagnosed histopathologically. The histopathologic diagnostic feature of the disease is emperipolesis, which is phagocytosis of lymphocytes, plasmacytes, erythrocytes, or PMN leukocytes by histiocytes . In addition to emperipolesis, Russell bodies, and foamy histiocytes are mostly seen in the intra-nodal form of the disease  , 23. IAn informed consent of the patient was obtained to use the information and photos of the patient in the article.RDD is a rare, benign, and self-limiting disease. Although few cases of RDD have been reported up to now, the similarity of signs and symptoms of RDD, tuberculosis, and malignant tumors like lymphomas, makes the correct diagnosis of the disease more challenging. Histopathology is the definitive way for diagnosing RDD by detecting emperipolesis. Treatment is not necessary in benign cases, although in life-threatening cases, surgical treatment is needed to avoid mortality. The recurrence of disease is probable; therefore, routine follow-up is recommended.Authors declare that there are no conflicts of interest in this study.