Rhinosporidiosis is a chronic granulomatous infectious disease that predominantly affects the mucosal membranes of the nose and nasopharynx. The disease is caused by Rhinosporidium seeberi, an eukaryotic pathogen with distinct geographical distribution particularly in tropical and subtropical areas acquired mainly through aquatic exposure.We report a case of a young Nepalese male who presented with recurrent right nasopharyngeal mass where surgical excision and histopathological examination confirmed the diagnosis following distinct pathognomonic findings. There is no optimal effective management of the disease and surgical excision coupled with cauterization to prevent recurrence is the recommended best option since medical treatment alone is ineffective. The clinical presentation, assessment and management options are reviewed. Rhinosporidiosis is a chronic granulomatous disease caused by Rhinosporidium seeberi which has a confusing historic taxonomy spanning fungi, parasite and bacteria till eventually classified as an aquatic eukaryote, based on comparison with similar aquatic organisms and genetic sequencing ,2. AlthoA 35-year-old Nepalese male presented acutely to our medical service with right sided epistaxis of two-day duration. The patient had no chronic medical conditions and previously was working as a farmer in his home country. Further evaluation revealed previous similar episodes in the preceding year, with history of excision of hard masses in the past. Upon clinical assessment, a right-sided polypoid strawberry nasopharyngeal mass was seen. Blood investigations were unremarkable. Computerized tomography of the nose and sinuses showed a lobulated soft tissue mass occupying the right nasal cavity near the middle and inferior nasal turbinates protruding into the right nasopharynx .Fig. 1AxFollowing initial evaluation, the bleeding was halted followed by functional endoscopic sinus procedure for excisional surgery. Intraoperative findings were of a large polypoid friable mass filling the right nasal cavity attached to the inferior and middle turbinates which bled easily. Complete excision of the mass together with partial inferior turbinectomy was performed followed by cauterization. Subsequent histopathological examination demonstrated typical spores within the characteristic pathognomonic sporangia, confirming the diagnosis of naso-sinal Rhinosporidiosis . On detaThe diagnosis was made based on the patient clinical profile, radiological assessment, intra-operative findings together with the characteristic histopathological results. Reviewing the evidence from medical literature we found no strong supporting evidence for repeating medical treatment alone with associated recurrence rates. Complete surgical excision with adjunct cauterization was the best recommended treatment option, one year into follow up there is no further recurrence.Rhinosporidiosis is a rare enigmatic infectious disease with a historic contentious etiology which was only revealed over recent decades. The disease is caused by Rhinosporidium seeberi, a hydrophilic organism that was initially oscillating in classification between parasites, fungi as well as bacteria till it was revealed as an aquatic eukaryotes that infects human and animals, towards the end of the 20st century . The namRhinosporidiosis primarily affects aquatic animals like fish and reptiles while humans as well as large domestic mammals and birds are incidental hosts . The disRhinosporidiosis has a predilection for external mucosal tissues like the nasopharynx and around the eyes, probably because of vulnerability together with probable need for wet surfaces for pathogen attachment and proliferation . The disRecurrence is a well-known feature of Rhinosporidiosis even after complete excision which might be explained by a decrease in anti-rhinosporidial cell-mediated immunity mediated by switch from Th-1 to Th-2 .For practical reasons, it has been advised to consider Rhinosporidiosis in any patient coming from endemic regions with nasal masses . RadioloThe recommended management for Rhinosporidiosis particularly nasopharyngeal presentation is wide surgical excision with cauterization of the base of the lesion to prevent recurrence ,11. DespOtolaryngologists and infectious diseases physicians should keep in mind the presentation of Rhinosporidiosis when managing migrant population with nasopharyngeal masses or abnormal growths related to mucosal surfaces even from non-endemic areas. Assessment with radiological imaging together with the characteristic histological findings are crucial steps in management. Since there is no clear proven benefit of medical therapy, the recommended treatment for Rhinosporidiosis particularly the commonest nasopharyngeal presentation, is wide surgical excision together with cauterization of the excised sites.A written informed consent was obtained from the patient to include clinical presentation together with results and imaging. This was subsequently reviewed and approved by the institution ethics and research review board.No funding was received towards the publicationAuthors declared no competing interests in relation to current publication.


Benign tumor of the bladder (Leiomyoma) is a rare condition representing less than 0.5% of all bladder tumors. A 50-year-old female presented with asymptomatic hematuria for three months. Abdomen and pelvic magnetic resonance imaging (MRI) revealed a large intraluminal mass originating from the submucosal layer of the left anterolateral wall of the urinary bladder. An ultrasound-guided biopsy was performed, and a histopathological examination showed a spindle cell tumor. Cystoscopy revealed a massive indentation in the left posterolateral site of the urinary bladder extending to the bladder's dome. The patient underwent open partial cystectomy. At the follow-up, the patient was evaluated, and there was no hematuria or other complaints. Open partial cystectomy is the treatment of choice in an intramural tumor with an unfavorable position and difficulty in recognition. Bladder leiomyomas are rare benign mesenchymal neoplasms and represent less than 0.5% of all bladder tumors . BladderA 50-year-old, non-smoking female patient with a medical history of diabetes mellitus (DM) and hypertension (HTN) presented to the Urology Department of King Fahad General Hospital with a three-month history of hematuria. She denied any history of flank pain, dysuria, urgency, fever, or weight loss, or appetite. Clinical examination was normal, laboratory findings were within the normal range, urine analysis showed >40 red blood cells (RBCs) per high power field, and urine culture showed no bacterial growth. Bladder smooth muscle cell markers (smooth muscle actin (SMA), desmin, and calponin) were positive. B-catenin and CD117 were negative. An abdominal and pelvic CT scan with contrast was done and showed a large submucosal urinary bladder mass. An abdominal/pelvic MRI was performed and revealed a large submucosal well-defined round mass with a slightly lobulated smooth margin originating from the submucosal layer of the left anterolateral wall of the urinary bladder. It measured 5 x 7 x 4.5 cm and was isointense to the T1 weighted image's muscle and heterogenous with the cystic component on the T2 image. No suspicious lymph nodes in the pelvis and no infiltration into the adjacent organs and tissues were found. The finding suggests bladder leiomyoma and solitary fibrous tumor  and are rarely extravesical (30%) or intramural (7%) in location . UrinaryA bladder leiomyoma is a rare, benign disease with a good prognosis. In our experience, partial cystectomy is the treatment of choice. In case of submucosal tumors, TURBT is the mainstay of treatment for small and easily accessible tumors. However, in cases of unfavorable position and difficulty in recognition, open partial cystectomy might be required as in our case.


Pyogenic liver abscess typically occurs secondary to biliary or haematogenous spread of organisms. In the context of acute appendicitis, abscesses generally occur due to haematogenous spread through the mesenteric vasculature. Historically, few cases of direct intra-abdominal spread have been reported but this has become vanishingly rare since the development of antibiotic therapy with no recorded cases in a search of over 900 cases in the literature. The initial diagnosis was made on chest radiograph . This leThis case report demonstrates the importance of thorough radiological investigation in the context of suspected hepatic abscess in order to delineate important albeit rare causes that may not resolve with conservative management alone.4 The overwhelming majority of perforations occur intraperitoneally with an inflammatory fluid collection or phlegmon forming around the inflamed vermiform appendix.Appendicitis is not only a common surgical emergency but also a frequent indication for CT examination of the abdomen and pelvis. There is an approximate appendix perforation rate of 10–20% which can depend on different factors.5Non-specific but important sequelae of acute appendicitis are associated with single or multiple pyogenic liver abscesses. This classically occurs due to haematogenous spread of bowel organisms via the mesenteric venous drainage entering the portal venous system and subsequent seeding into the liver. The other classical common cause of pyogenic abscess would be due to the biliary spread of organisms, for example cholecystitis or ascending cholangitis.e.g., biopsy) or some form of penetrating trauma. We present an extremely uncommon case of contiguous pyogenic liver abscess in the context of a subacute/missed appendicitis that has become adherent to and subsequently fistulated into the right lobe of the liver.The third route of spread is direct seeding of organisms into the liver. This mostly occurs via direct iatrogenic inoculation following a procedure (9/L), an elevated serum lactate of 2.8 (mg/dL) and a C-reactive protein of 221 (mg/L). An admission radiograph demonstrated complex features with a large right-sided pleural effusion and a gas-fluid level that “mirrored” the diaphragmatic contour raising the suspicion for a hepatic or subdiaphragmatic abscess , slightly hypertensive at 153/90 mm Hg and with a respiratory rate of 18/min and an oxygen saturation of 96% on room air. Serum blood markers identified a neutrophilia of 14.1 . While simple bedside tests may allude the clinician to the possibility of an abscess, CT remains an important modality for a full assessment of not only the size and number of lesions but as demonstrated in this case, the potential aetiology. Direct spread from intra-abdominal infection can be an indicator for required interventional or surgical treatment in combination with antibiotic therapy.3 Management includes consideration for appendicectomy to reduce the risk of continued bowel contents entering the liver/pleural space despite optimal drainage as is likely in the case we describe.Management considerations are usually based upon an appraisal of the number and size of the abscesses where smaller lesions, particularly those under 50 mm in size, may be amenable to medical management.In the context of a pyogenic liver abscess, a patient would typically present with fever and right upper quadrant pain in greater than 60% of cases. This was not the case with our patient and this atypical presentation can require quick recognition of abnormal radiological findings in the emergency setting to guide appropriate further investigation and subsequent management. It is also worth noting that our case actually demonstrated a subcapsular abscess with two small intra-parenchymal breaches. This is even more unusual for the abscess to be tracking in the subcapsular space. Having an awareness of this pathology can help both clinicians and radiologists to think laterally and ensure they work together to investigate appropriately.8 Appendicular malignancy is found in around 1% of all post-mortem appendix specimens.9 Epithelial neoplasms (mucinous tumours of the appendix) and neuroendocrine tumours (carcinoid tumours) account for the vast majority. It is worth noting that in our case, there was no evidence of pseudomyxoma peritoneii which can be seen on CT when mucinous tumours of the appendix rupture. Interestingly carcinoid tumours of the appendix are seen within the tip in 75% of cases.10While appendicitis can occur at any age, given our patient’s age of 69 years and the atypical presentation of appendicitis in this case, for example insidious onset symptoms with no abdominal pain other considerations must be taken as to the underlying aetiology of the appendicular perforation. Acute/subacute inflammatory appendicitis is a very common pathology and while this still remains the most likely aetiology radiologically, one must consider rarer causes such as an appendiceal tumour. While it is known that an appendicular malignancy can mimic acute appendicitis, it may also have a more insidious onset and lack the typical clinical features of a benign inflammatory appendicitis.This case highlights that one must always consider common conditions presenting atypically and the importance of appropriate and timely radiological investigation in such cases. In the context of suspected hepatic abscess, this helps to localize pathology, assess for some key aetiologies and detect worrying sequelae as demonstrated in this case. This case highlights the importance of increasing both clinicians and diagnostic radiologists awareness of this now rare pathology to ensure timely diagnosis and intervention. This case also offers an opportunity to revisit the pathology of the appendix and consider both benign inflammatory and neoplastic causes of appendiceal perforation.Radiologists should consider direct intraperitoneal spread as a cause of pyogenic liver abscess.Have a low threshold for cross-sectional imaging in assessing suspected pyogenic liver abscess.Ultrasound can help to guide management in planning percutaneous drainage of intra-hepatic abscess.In older populations, one must consider differential diagnoses of appendiceal perforation such as an appendiceal tumour.


On 15th post-operative day, she was re-admitted with complaints of sudden onset disorientation, nausea and vomiting. On examination, she was drowsy, obtunded, did not follow commands and responded only to sternal rub. Keeping possible differential diagnosis of pulmonary embolism, infarct or delayed surgical site hematoma in mind, complete blood counts, renal and liver function tests and electrolytes were obtained which were all within normal limits. Workup for hypercoagulable state including protein C and S were negative. CT brain was obtained which revealed severe diffuse cerebral edema along with parieto-occipital acute infarct  are known to be quite variable and little information is available regarding the pathophysiology and management of this potentially catastrophic complication. POCVI usually manifests itself acutely within hours of surgery or it may be chronic. However, delayed onset acute form is not known. We describe one such unusual case. A 45-year-old female patient underwent Simpson grade I excision of her posterior parasagittal meningioma  and B. N infarct  and F.et al. [th post-operative day which has not previously been described.Endotracheal intubation was done and mechanical ventilation started along with initiation of anti-edema measures (mannitol and furosemide) and anticoagulants and antithrombotics. A magnetic resonance imaging (MRI) and venography (MRV)/CT venography was planned but could not be performed due to her critically ill condition. She did not respond to the treatment and before decompressive craniectomy could be performed, her condition quickly deteriorated and she died within 24-hrs of admission despite aggressive measures. Cushing et al.  describeet al.  further The occurrence of delayed-onset acute POCVI could possibly be related to the hypercoagulable state of the tumor bed leading to thrombosis of the vessels. However, more studies are required to study the pathophysiology and prevention of this apparently virulent condition. In cases of meningioma excision, we urge neurosurgeons to remain vigilant even after an apparently uneventful post-operative course as delayed onset POCVI could be fatal.


Pulmonary hamartomas are benign lesions that are often managed conservatively in the absence of respiratory symptoms. Increasing reports of malignant transformation question if a more aggressive treatment or surveillance practice for these lesions is warranted in adult patients. Herein, we describe a case of a 67-year-old man with a long history of pulmonary hamartoma that demonstrated malignant degeneration into spindle cell malignancy with chondromatous differentiation. This case illustrates the aggressive nature of sarcomatous disease arising from hamartomas and, with a handful of other cases in the literature, points to the question of whether pulmonary hamartomas arising in late adulthood should follow a more intensive treatment or surveillance algorithm given increased concern for malignant potential. Hamartomas are benign tumors that maintain the characteristic tissue of the skin or soft tissue site at which they are located, albeit in a disordered fashion . There aA 67-year-old gentleman who presented to thoracic surgery clinic in 2017 for evaluation of an enlarging, partially calcified right middle lobe mass, found incidentally on CT in 2012. In 2012, his mass measured 3.0x3.3 cm and was evaluated with PET-CT, which read this mass as mildly hypermetabolic and likely a hamartoma, though due to its position interfacing with an adjacent rib, a rib chondrosarcoma could not be ruled out Table . Biopsy was then performed which confirmed diagnosis of hamartoma with no evidence of cytologic atypia or malignancy identified Figure . Steady  In our case, the patient had previously had biopsy confirmed hamartoma which then rapidly degenerated to chondrosarcoma despite surveillance. It is difficult to pinpoint any predicting factor that may have helped prevent mortality in our patient. We have reviewed similar cases that have been presented in the literature. A 74-year-old woman was found to have a biopsy-proven left-sided pulmonary chondroid hamartoma that transformed to intermediate-grade chondrosarcoma over the course of seven years after radiation therapy for breast cancer . SimilarEnlarging nodules, even with the diagnosis of hamartoma, should be excised. When patients are not surgical candidates or are averse to excision, they should have a different algorithm for treatment involving repeat biopsy and serial CTs. If enlargement continues, stronger recommendations for excision should be given. We suggest imaging every six months for two years, then yearly through year five, then every two years until year ten. If no changes occur during this surveillance, imaging can be discontinued at that time, although there are no randomized studies to validate this. If changes are found, again, we recommend rebiopsy or excision given the risks we are describing. If the patient becomes symptomatic from their hamartoma after their period of surveillance, we also recommend rebiopsy or excision at that time. If clinical suspicion outweighs benign biopsy results or negative PET scans, rebiopsy or excision should be considered. Understandably, reactive decision-making can prove to be difficult, but patient goals and quality of life need to be taken into consideration as well. Needless removal of a large lesion can significantly deteriorate patient quality of life and has intraoperative risks as well. We hope to draw the attention of the thoracic community to the malignant potential of these classically benign pulmonary lesions, and to recommend a low threshold for excision. Early referral of patients with these lesions to thoracic surgery is of increased importance in light of the increasing case reports of sarcomatous transformation. Guo, et al. recommended wedge resection in uncomplicated cases, so as to reserve lobectomy or pneumonectomy for cases in which the tumors were deep and invading the hilum, involved tissue no longer provided respiratory function, or severe disease burden. They also noted the importance of procuring frozen sections during operative procedures in order to better grade the tumor .Pulmonary hamartomas are considered to have a good prognosis due to their slow growth and extremely low metastatic potential. However, rare cases of rapid expansion are reported in the literature, which should prompt a thorough malignancy workup as hamartomas have been associated with an increased rate of concurrent primary lung carcinoma. Despite this, malignant degeneration of pulmonary hamartomas is exceedingly rare and treatment recommendations regarding indications and timing of resection are controversial. We suggest that enlarging pulmonary hamartomas arising in adulthood should be resected in fit patients or aggressively surveilled and re-biopsied at regular intervals in patients who are less fit surgical candidates.


No cytogenetic studies were performed. He died at the 25th day of hospitalization. Autopsy revealed marked leukemic infiltration to the endocardium and subendocardial myocardium. Subendocardial myonecrosis was surrounded or replaced by the leukemic blasts, and neither granulation tissue reaction nor fibrosis was observed. In the cardiovascular lumen, lard-like blood clots were formed and microscopically consisted of leukemic blasts and platelets (leukemic thrombi). Infiltration of leukemic blasts was seen in the body cavities and systemic organs including the lung. The MPO-positive blasts lacked azurophilic granules and expressed the stem cell markers, CD34 and CD117 (c-kit). No features of myelofibrosis were seen in the 100% cellular marrow. In the endocardium, liver, lymph nodes, and bone marrow, megakaryocytic cells  were distributed, while the small-sized blastic cells in the blood and tissues predominantly expressed MPO. The blasts lacked expression of CD42b/CD61. Megakaryocytic differentiation might be stimulated by certain tissue factors. AML accompanying megakaryocytic differentiation in certain tissues and organs should be distinguished from acute megakaryoblastic leukemia. The mechanisms provoking acute coronary syndrome in acute myeloid leukemia are discussed.An autopsy case  of myeloperoxidase- (MPO-) positive acute myeloid leukemia with maturation (M1) accompanying megakaryocytic differentiation is presented. The patient manifested acute coronary syndrome. Even after emergent percutaneous coronary intervention, his performance status remained poor, so no chemotherapy against leukemia was given. The final white blood cell count reached 291,700/ Acute coronary syndrome has been reported as the initial manifestation of acute leukemia: leukemia cells may provoke coronary obstruction –5. AfterWe report herein an autopsy case of MPO-positive AML with maturation (M1) of the aged accompanying megakaryocytic differentiation in certain tissues and organs. Because of poor performance status, no chemotherapy was given, nor cytogenetic studies performed. Of note, the patient manifested acute coronary syndrome, likely due to leukemia cells-induced intravascular thrombosis and direct invasion of leukemia cells to the heart muscle.μL) with 83.5% myeloblasts positive for myeloperoxidase (MPO) activity. Hemoglobin count was 5.1 g/dL, and the platelet count was 510,000/μL. Since his performance status was judged insufficient for chemotherapy, no treatment was given against AML. The patient tentatively recovered to having a meal, but he died of respiratory failure 25 days after hospitalization. The final white blood cell count reached 291,700/μL. Neither fluorescence-activated cell sorting (FACS) analysis, karyotyping, nor precise cytogenetic studies were performed.An 85-year-old Japanese male with a past history of brain stroke and chronic obstructive pulmonary disease was transferred to Shimada Municipal Hospital, Shimada, Shizuoka, Japan, because of acute chest discomfort. Electrocardiogram indicated acute coronary syndrome. Emergent percutaneous coronary intervention was carried out. The catheter occluded the left main trunk, resulting in cardiopulmonary arrest. Cardiopulmonary resuscitation rescued spontaneous circulation. A covered stent was then grafted to the narrowed portion of the left coronary artery. A peripheral blood examination revealed an abnormally high level of white blood cells  blood clots in the cardiac and aortic lumen, as well as in the pericardial and pleural spaces. The blood clot in the cardiac lumen weighed 80 g. The bilateral pleural cavities were filled with lard-like material, and the left cavity contained 1,100 mL effusion (right side: unmeasurable). Microscopically, the clot consisted of blastic cells, intermingled with numbers of platelets. The leukemic blasts infiltrated the bone marrow (100% cellularity without myelofibrosis), liver , spleen (200 g), and lymph nodes . The blasts also involved the serosal membranes, heart (410 g), lung , kidney , adrenal glands , and testis, as well as, though mildly, the pancreas, vermiform appendix, prostate, and seminal vesicles. Dense leukemic infiltration into the lung parenchyma and pleural cavity, associated with massive effusions and secondary organizing/aspiration pneumonia, was regarded as the direct cause of death. There was no microscopic evidence of extramedullary hematopoiesis: no erythroblastic islands were identified in the extramedullary organs and tissues. The brain was not examined. The final autopsy diagnoses are summarized in The blastic cells in May-Giemsa-stained preparations possessed no azurophilic granules in a scanty basophilic cytoplasm. No megakaryoblastic cells were observed. The marker study performed using formalin-fixed paraffin-embedded sections disclosed expression of MPO and the stem cell markers such as CD34 and CD117 (c-kit). CD3, CD10, CD20, CD79a, and terminal deoxynucleotidyl transferase were negative. The horizontal cut surfaces of the heart grossly displayed subendocardial ischemia, and microscopic examination demonstrated dense infiltration of leukemic blasts among cardiac muscle cells. Cardiomyocytes were replaced by the leukemic blasts, or coagulation necrosis of the muscle cells was densely surrounded by the leukemic blasts. In spite of the clinical course for 25 days, the common histopathologic features of myocardial infarction, such as granulation tissue reaction and fibrosis, were hardly seen in the myocardium, and coronary arterial atherosclerosis was mild. The leukemic infiltration was also noted in the endocardium, where megakaryocytic differentiation was microscopically evident. Immunohistochemically, the small-sized monomorphic blasts in the myocardium and endocardium were MPO-positive and CD42b/CD61-negative, while MPO-negative megakaryocytic cells strongly expressed CD42b/CD61, as illustrated in We presented here an autopsy case of acute myeloid leukemia (AML) with maturation accompanying megakaryocytic differentiation in certain tissues and organs. First, differential diagnosis of acute megakaryoblastic leukemia (AMKL) is needed. AMKL belongs to acute myeloid leukemia, not otherwise specified, acute megakaryoblastic leukemia according to the World Health Organization classification 2017, or M7 according to the French-American-British (FAB) system. AMKL is defined as an AML with >20% blasts, of which >50% belong to the megakaryocyte lineage . AMKL coWe described herein an adult case of AML with strong MPO immunoreactivity and the expression of stem cell markers (CD34 and CD117). Myelofibrosis was absent in the leukemic marrow. Of note, there was distinctive differentiation toward CD42b/CD61-positive megakaryocytes in the bone marrow, lymph nodes, liver, and endocardium. Supposedly, such megakaryocytic differentiation was induced by certain tissue factors. Reports say that megakaryocytic differentiation is accelerated by thrombopoietin (c-myeloproliferative leukemia (c-Mpl) ligand) , proteasTissue-dependent differentiation toward megakaryocytes may be related to a hidden potential of differentiation in the immature blastic cells of AML. Theoretically, there might be a separate population of dysplastic megakaryocytes with no actual labeling of CD42b/CD61 on the morphologic blasts. Or a minor population of blasts might express CD42b/CD61. It is difficult for us to get the direct morphological proof for such suspicion. During the hematopoiesis, a series of committed progenitors produce all kinds of blood cells, and megakaryocytes are derived through the multipotent progenitor, common myeloid progenitor, or megakaryocyte-erythroid progenitor . HematopThe association of thrombocytosis in the peripheral blood was related to the megakaryocytic differentiation, a unique feature of our case. Acute megakaryoblastic leukemia accompanying thrombocytosis has been reported , 29. In μL. The patient suffered from acute coronary syndrome as the initial manifestation of acute leukemia, as has been reported previously [The formation of lard-like blood clot consisting of leukemic blasts and platelets in the cardiovascular lumen and pleural cavities was quite characteristic of the present case. The platelet count in the peripheral blood was elevated to 510,000/eviously . After ceviously . In the


Nevus sebaceus of Jadassohn, or “organoid nevus,” is a common, benign hamartoma of the skin consisting of epithelial and adnexal components. Its natural history and association with neoplastic growths is well documented. The majority of concomitant neoplasms are benign—trichoblastoma and syringocystadenoma papilliferum are most frequently discovered—but malignant tumors have been described. We present the case of a 58-year-old male with a congenital nevus sebaceus of Jadassohn on his left parietal scalp that had been enlarging, changing color, and bleeding over the prior year. Clinical exam and histology disclosed the presence of a trichoblastoma and porocarcinoma arising within the nevus sebaceus. Porocarcinoma is a rare, intermediately aggressive, malignant eccrine gland tumor that is frequently metastasized at presentation. Otolaryngology performed wide local resection with sentinel lymph node biopsy. This case highlights the diversity of tumors associated with nevus sebaceus of Jadassohn, potential for malignant expansion, and necessity for close monitoring and maintaining a low threshold for biopsy in evolving lesions. Nevus sebaceus of Jadassohn (NSJ), also known as “organoid nevus,” is a common, benign hamartoma composed of abnormally developed hair follicles, papillomatous epidermal hyperplasia, and ectopic apocrine glands that presents at birth or in early childhood –4. TypicThe natural history of NSJ and its association with neoplastic growths is richly documented such that ‘Tumoral' is the accepted final developmental stage . Althoug.A 58-year-old male presented for an evolving mass on his left scalp. The mass was noted at birth and had been enlarging, changing color, and bleeding over the past 12 months. On exam, a 3.0 cm × 3.0 cm yellow-to-tan verrucous, papulated plaque with central, large pink, and violaceous-to-blue nodules was present on the left posterior parietal scalp with overlying alopecia .A shave biopsy of the entire lesion was performed. Pathology disclosed changes consistent with an organoid nevus with two distinct neoplasms within. A basaloid tumor arising from the epidermis with a focal ribbon and pigmented pattern consistent with trichoblastoma and an adjacent downgrowth of atypical squamous epithelium with dysplasia and easily identified mitotic figures with a glassy stroma and eccrine differentiation consistent with porocarcinoma Figures . ImmunohThe patient was referred to otolaryngology for wide local resection with sentinel lymph node biopsy. There was no parotid gland enlargement or palpable anterior or posterior cervical lymphadenopathy. Wide local excision (6 cm × 6 cm with 1.5 cm to 2 cm margins) was performed with sentinel lymph node biopsy from the left tail of the parotid identified through nuclear medicine. Reconstruction was with opposing transposition flaps.Surgical pathology revealed no residual porocarcinoma in the scalp specimen and negative sentinel lymph nodes; therefore, no further treatment, including chemotherapy, was indicated. Three years later, the patient remained disease free.Nevus sebaceus of Jadassohn is a common, congenital lesion that overwhelmingly presents on the head with a special affinity for the scalp and persists throughout life –3, 5. GeNSJ's association with secondary neoplasms is well established. It is estimated that 10–21% of NSJs develop a tumor ; howeverHistorically, it was thought that basal cell carcinomas (BCC) were the most common neoplasm complicating NSJ, but studies from the early 1990s and forward have confirmed that what was previously believed to be BCC were, in fact, misdiagnosed trichoblastomas . It is nPorocarcinoma is the most common malignant eccrine gland tumor and accounts for an estimated 0.005–0.01% of all cutaneous tumors , 7. Its th-8th decade of life) and inconsistent reports regarding gender preference [Porocarcinomas present as a solitary nodule or mass involving the lower extremities—head/neck and upper extremity lesions are seen about equally –9. The seference –9.Porocarcinomas are an intermediately aggressive tumor with metastatic disease at presentation in up to 31% of cases, most frequently involving regional lymph nodes 60%) and the lungs (13%) 0% and th. When meThe only case describing a porocarcinoma developing in an NSJ was reported in 1985 by Tarkhan and Domingo where the lesion also arose on the scalp but had postauricular extension and metastasis . PreviouIn our case, the patient demonstrated a porocarcinoma that arose adjacent to a trichoblastoma. There are several reports of multiple neoplasms developing in a single NSJ , 2, and This case highlights the potential for malignant expansion in NSJ, the diversity of tumors that arise within it, and the clinical necessity of closely monitoring the evolution of these lesions and maintaining a low threshold for biopsy.


Soft tissue calcifications in the oral cavity and maxillofacial region are most often detected as incidental findings on routine radiographic examination. But sometimes these soft tissue calcifications can be serious and may need treatment or follow-up of the underlying cause. Deposition of calcium salt as a result of chronic inflammation, necrosis or scarring in injured tissues despite normal phosphorous and calcium metabolism is called dystrophic calcification. A variety of systemic disorders can be associated with this type of calcification but, still, the pathophysiology is not clear. Here we present a case of dystrophic calcification in the floor of the mouth of an 18-year-old female patient associated with dysphagia which was excised by intraoral route. The human skeleton mostly has deposition of calcium salts, mainly calcium phosphate. If this type of deposition occurs in soft tissue in an un-coordinated way, then it is known as heterotrophic calcification and categorized as metastatic, dystrophic, or idiopathic. Minerals depositing into normal tissue because of higher than normal serum levels of phosphate  or calcium (hyperparathyroidism) is known as metastatic calcification. The calcium deposition in normal tissue despite normal serum phosphate and calcium levels is known as idiopathic calcification. The pathologic calcification that occurs in dead and degenerative tissue or scarred tissue despite normal levels of serum phosphate and calcium is known as dystrophic calcification . Here weAn 18-year-old patient reported with dysphagia and sensation of foreign body in the throat region on the left side. Difficulty in swallowing solid food was present, while there was no difficulty for swallowing semi-solid and liquid diet. The patient gave a previous history of infection in the left mandibular angle region which was treated with incision and drainage eight years back. The patient does not recollect any history of trauma in that region. The patient was healthy, and her blood investigation values were within normal limits.Extra-oral examination revealed an old linear scar in the left mandibular angle region. Intra-oral examination revealed a yellowish-white mass in the lingual sulcus anterior to left anterior faucial pillar and distal to left mandibular third molar region Figure .There was no evidence of inflammation of surrounding tissues. The mass was firm on palpation and was not associated with pus discharge. The patient did not have problems in salivation or symptoms of ductal blockage.Radiographic examination (OPG) Figure , 2B and The removal of this calcified mass was carried out intraorally under local anesthesia (2% lignocaine with epinephrine 1: 80000). An incision was given on the floor of the mouth and by extending the opening of the lesion, total removal of the mass was carried out. Hemostasis was achieved, and closure was carried out using 3-0 vicryl.The calcium salts deposition in the tissue other than enamel or osteoid is called heterotrophic or pathological calciﬁcation . When deDystrophic calciﬁcation is mostly seen in subcutaneous tissues secondary to infection or trauma and is described in systemic lupus erythematosus, scleroderma or dermatomyositis. Injured tissue of any kind may be predisposed to dystrophic calciﬁcation . In our Tonsillolith was one of the differential diagnoses. Tonsilloliths are calcium salt deposits arising from material retained in the tonsillar crypts and occur most frequently in young adults affected by recurrent upper airway infections . It may The differential diagnosis considered was calcifications in the lymph nodes, carotid artery, metastatic, stylohyoid ligament and, salivary gland.Carotid artery calcification is mostly located in the soft tissue beneath the mandibular angle and between the cervical spine image and the hyoid bone .The pathological condition caused by the deposition of calcified products in normal tissues because of hypercalcemia with or without hyperphosphatemia is called metastatic calcification .The lymph nodes calcification commonly occurs beneath or near the angle of the mandible in the submandibular region. On radiographic examination, these calcifications mostly appear as well-defined, irregularly shaped opacities and can be described as “cauliflower” like. One prospective method to differentiate between lymph node calcification and sialolith is that submandibular sialolith is most frequently solitary whereas calcified lymph nodes are often multiple .Standard occlusal projections and panoramic radiographs of sialoliths reveal that they are most common in the submandibular glands (83% to 94%), followed by the parotid gland (4% to 10%) and finally sublingual gland (1% to 7%). Patients with sialolithiasis may be asymptomatic, or they may develop obstructive sialadenitis characterized by pain and periodic swelling, especially around mealtime when salivary flow is stimulated. But these symptoms were not seen in our case.The panoramic image of the ossified stylohyoid ligament extends from the mastoid process crossing the posteroinferior aspect of the ramus and towards the hyoid bone . The styThe deposition of calcium salt in degenerated tissues in the absence of a systemic mineral imbalance is called dystrophic calcification. It is mostly associated with trauma, inflammation or infection, and rarely appears in the head and neck area.Different types of calcifications may occur in the head and neck region, and they need to be distinguished from dystrophic calcification by thorough history taking, clinical examination together with blood testing and radiographic examinations.


Lymphatic leakage is an infrequent but relatively serious complication after resection of retroperitoneal tumors. Different approaches have been attempted in treatment of lymphatic leakage. However, to date none of them have been demonstrated consistently effective. We hereby report our preliminary experience with Lymphangiography and embolization for resolution of Lymphatic leakage after retroperitoneal tumor resection.The patient, a 55-year-old woman with massive retroperitoneal tumor, Computed tomography (CT) revealed that retroperitoneal cystadenoma. After the right ureteral stent was placed, the retroperitoneal tumor resection was performed, postoperatively she presented with lymphatic leakage unresponsive to several treatment measures. From the thirty-seven postoperative day (POD37), the patient underwent Lymphangiography and embolization, which resolved her lymphatic leakage.Lymphangiography is useful for detecting lymphatic leakage occurring after retroperitoneal tumor resection. Furthermore, lymphatic embolization is feasible, effective, and safe for managing leaks demonstrated on lymphangiography. Lymphatic leakage following this is a common occurrence. A variety of approaches have been attempted to the treatment of lymphatic leakage. However, so far none has been consistently effective or optimal . We here2A 55-year-old female patient visited our hospital, who reported to have abdominal mass and complained that the mass gradually increased to affect sleep. B-ultrasound examination revealed mixed echo zone with approximately 243 × 118 in size in the abdominal cavity. Computed tomography (CT) showed a large mass on the right side of the abdomen, with the right ureter and inferior vena cava compressed to the right, indicating retroperitoneal cystadenoma. Magnetic Resonance Imaging (MRI) suggested a large multilocular cystic space in the retroperitoneal and hepatorenal space, approximately 261 × 181 × 150 mm in size, which was very likely to be epidermoid cyst . After aPostoperative pathology : mature cystic teratoma showed mucinous tumor and mild to moderate atypical hyperplasia of glandular epithelium. After the operation, the drainage of the abdominal drainage tube was in light blood liquid in small amount. CT revealed peritoneal effusion after operation on POD7 , therefoAfterwards, the patient was instructed to fast and switch to total parenteral nutrition for one week. The drainage tube was clamped and the patient did not complain of any discomfort. After two weeks of lipiodolography, the peritoneal puncture drainage tube was removed, and the patient was instructed to eat a light diet and gradually make a transition. After one week of observation, no peritoneal effusion was found by b-ultrasound and the patient discharged from hospital. The patient was reviewed CT regularly after discharge, no abnormalities were found.3Lymphatic leakage is a rare and well-known complication following retroperitoneal tumor resection. Great attempt has been made to the treatment of lymphatic leakage, as listed in the following. (1) Abdominal drainage. In spite of no direct evidence supporting that abdominal drainage can promote the healing of fistula, it can provide clinical diagnosis basis and alleviate a series of clinical symptoms such as abdominal pain and distension to a certain extent. In addition, the therapeutic treatment plan can be adjusted according to the amount of drainage fluid. (2) Antisecosis. Mid-chain triglyceride diet with parenteral nutrition can reduce the amount of lymphatic leakage, which is because the short-chain triacylglycerol contained in food can be absorbed directly into the blood through the intestinal tract, while the long-chain triacylglycerol should be transported and absorbed through the lymphatic pathway . (3) OctNevertheless, some leaks still persist despite conservative treatment, therefore, more effective treatments are needed . LymphatOur case demonstrates that postoperative lymphatic leakage that was successfully treated by performing repeated lymphangiography and embolization. This technique should be considered for further use and investigation for abdominal surgery and lymph node dissections.The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.The authors declare that they have no competing interests.No funding was received.The study was approved by the Ethics Committee of Second Affiliated Hospital of Suzhou University, China. Patients who participated in this research, signed the informed consent and had complete clinical data. Signed written informed consents were obtained from the patients and/or guardians.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.JG wrote the manuscript, interpreted and analyzed the data. WC designed the study and performed the experiment. JC was responsible for the analysis and discussion of the data. Both authors read and approved the final manuscript.Dr. Chen Wei.Not commissioned, externally peer-reviewed.


Pleomorphic adenoma (PA) is a salivary gland tumor that may rarely occur in the nasal cavity. It can be a clinical diagnostic dilemma in many instances due to many possible differential diagnoses. We report the case of a 26-year-old female who presented with a 3-year history of a right nasal growth associated with ipsilateral nasal blockage, nasal pain, and rhinorrhea. Radiological image showed a mild enhancing lesion in the right nasal cavity. The patient underwent a lateral rhinotomy with wide excision of the mass. Histopathological exam was consistent with PA. Nasal PA is a rare entity and should be suspected as a diagnosis for intranasal tumors. Pleomorphic adenoma (PA) is the most prevalent salivary gland tumor in both children and adults, and it has a 2 : 1 female-to-male ratio. The parotid gland is the most common site accounting for 70-80% of the cases followed by the submandibular gland at 10% –6.PA rarely arises in the respiratory tract, but when it does, the nasal cavity is the commonest site . About 8Here, we report the case of a 26-year-old female who presented with nasal PA.A 26-year-old female presented with a 3-year history of a right nasal growth associated with ipsilateral nasal blockage, nasal pain, and rhinorrhea. On examination, she was noted to have a right-sided nasal bridge deformity. On anterior rhinoscopy, the right nasal cavity had a hard mass emerging from the inferior turbinate abutting on the nasal septum. The mass was completely filling the nasal cavity and obstructing the middle meatus. Nasal decongestion with a topical decongestant was unsuccessful. Baseline routine laboratory investigations and HIV tests were normal . HIV tesWe found a round, well-encapsulated mass measuring 40 mm × 30 mm arising from the inferior lateral wall encased by the inferior turbinate mucosa. The inferior turbinate appeared to be thinned by the mass. The mass was excised with its pedicle and part of the inferior turbinate.Gross examination revealed a greyish brown tissue measuring 40 mm × 30 mm × 30 mm with foci of bony elements and a capsule on cross-sectioning. The mass was then sent for histopathology , which rThe patient was discharged on postoperative day 3, and she recovered without any complications. On follow-up 2 years later, the patient had adequate nasal patency with no recurrence of the nasal mass.Nasal pleomorphic adenoma commonly presents as a nasal mass with unilateral nasal blockage which slowly progresses and may cause external nasal deformity associated with pain , which wThe clinical differential diagnoses include several lesions, most of which were polypoids, such as angiofibroma, osteoma, squamous cell carcinoma, adenocarcinoma, lymphoma, and melanoma. Recommended imaging options include CT scan and MRI. CT scan awards the clinician the chance to assess bone involvement or erosion.Although it was not done in our patient, preoperative fine needle aspiration of the mass is advisable as a cytological diagnosis guides surgical approach, given the wide spectrum of clinical differential diagnoses. Histologic examination of PA classically demonstrates myoepithelial and epithelial cells, some with duct formation, and mesenchymal or stromal elements .PAs in the nasal cavity may be misdiagnosed as an aggressive epithelial tumor due to high cellularity and a significantly reduced stromal component, features less common in tumors found in major salivary gland sites . In our Increased cellularity gives the nasal PA a biologically aggressive nature. Malignant transformation is seen in 2-6% of all salivary gland tumors . BetweenNasal PA has a lower rate of recurrence compared to PA of the parotid gland at 10 and 50%, respectively . An averNasal pleomorphic adenoma is a rare entity and should be suspected as a diagnosis for intranasal tumors. Wide local excision with clear margins and close follow-up postoperatively is necessary due to the potential risk of local recurrence.


Diffuse tenosynovial giant cell tumor (D-TSGCT) is a benign but locally destructive tumor of synovium that may involve joints, tendon sheaths, and bursae. Its occurrence in the temporomandibular joint (TMJ) is extremely rare. The authors reported a case of 48-year-old man with an extra-articular D-TSGCT in the TMJ with medial cranial fossa extension. computed tomography (CT) and magnetic resonance imaging (MRI) features are described. The lesion was a cystic-solid mass centered at the temporal bone without involvement of the condylar head, and its solid component presented high-density on CT and hypointensity on MRI. No signs of recurrence or metastasis was observed during 12-months of follow-up. The present report suggested the potential characteristics of radiologic imaging of D-TSGCT in TMJ. Diffuse tenosynovial giant cell tumor (D-TSGCT), also referred to as pigmented villonodular synovitis (PVNS), is benign disorder arising from the synovium of the joints, bursa, and tendon sheath . It ofteAlthough any synovium can be affected, only more than 100 cases of D-TSGCT involving the temporomandibular joint (TMJ) region have been reported, only 10 cases were classified as the extra-articular form –9. Due tThe patient was diagnosed with D-TSGCT at 48 years of age in West China Hospital. He started experiencing mild unilateral tinnitus, hearing loss from 3 years ago, the symptoms exacerbated gradually and trismus presented 6 months ago. Three months ago, the patient was diagnosed as cholesteatoma in the community hospital and no intervention was received. Soon afterwards, the patient presented to the otolaryngology clinic of our hospital. No facial numbness, facial muscle dysfunction, dysphagia, diplopia, or vision loss was reported, and the patient stated that there was no history of trauma to the face or jaw. A hard, non-tender, non-movable and poorly defined soft tissue mass in the right temporal area was revealed by physical examination. The high-resolution computed tomography (HRCT) of the temporal bone was performed first and suspected diagnosis of giant cell tumor (GCT) of the bone was made. The diffusion-enhanced Magnetic resonance imaging (MRI) was performed for further diagnosis, GCT of the bone or D-TSGCT was suspected diagnosed. The patient was then treated with surgery and the pathological examination was conducted. According to the histopathological features, in combination with radiological manifestations and clinical history, the final diagnosis of D-TSGCT was made. The timeline of diagnosis and treatment was showed in The HRCT revealed a mixed-density mass with soft tissue density and slightly hypodensity (5.2 cm × 3.2 cm × 3.3 cm) in the right temporal squama. Incomplete bone shell was visible, and the articular surface of temporal bone was eroded while the condylar head was not involved Figure 1The patient underwent surgical resection on September of 2019 and the mass was completely removed. The lesion was extradural, and its texture was mixed with hard and soft component. The external auditory canal and the bone of skull base were eroded. The mass showed adhesion to the adjacent dura and adhered tightly to the surrounding tissues. The cross-section of the lesion was dark red with hard capsule.Microscopically, the lesion mainly composed of small, round to spindle-shaped mononuclear cells, accompanied by varying numbers of osteoclast-like giant cells, foamy cells, and hemosiderin pigments. The mononuclear cells are characterized by round or reniform nuclei, and pale cytoplasm. Osteoclast-like giant cells are usually readily apparent, which contain a variable number of nuclei. Haemosiderin deposits are virtually identified. Mitotic activity reaches up to 10 mitoses per 10 high power field (HPF). Focal necrosis and foamy cells are rarely seen. No cellular atypia was reported Figure 3During 12-months of follow-up, the patient had no signs of recurrence or metastasis, the clinical symptoms were ameliorated obviously. The last time MRI data was showed in The etiology of D-TSGCT remains unclear and some possible pathogenetic theories include trauma, osteoclastic proliferation, infection, immune mechanisms, inflammation, neoplasia, and metabolic disturbances are proposed . The theMacroscopically, D-TSGCT are usually large, firm or sponge-like. It has a multi-nodular appearance, with ranging in color from yellow to dark red or brown. Microscopically, most lesions are infiltrative and grow as diffuse, expansile sheets. The cellularity varies depending on the relative proportion of mononuclear cells, multinucleate giant cells, foam cells and the amount of fibrous stroma. The mononuclear component comprises larger cells and small histiocyte-like cells. Osteoclast-like giant cells, which contain a variable number of nuclei, are usually readily apparent, but may be inconspicuous in highly cellular tumors. Large amounts of haemosiderin are frequently observed in most cases and sheets of foam cells are commonly identified in the periphery of lesions. The stromal fibrosis may appear hyalinized, although it is less marked than in the localized type , 19.CT and MRI provide valuable information in the diagnosis and developing treatment plan of D-TSGCT, delineating the extent of lesion as well as destruction of bone . ClassicThe classification of the lesion type regarding to intra- or extra-articular can be difficult in clinical practice, because the anatomy of the TMJ region is complex . Wang etThe main differential diagnosis of D-TSGCT at TMJ includes GCT of the bone, synovial chondromatosis, aneurysmal bone cyst (ABCs), rheumatoid arthritis (RA), and cholesteatoma. GCT of bone characteristically involves extremities of long bones, usually manifests as expanded and soap bubble like inner structure with no calcification or mineralization. Synovial chondromatosis usually occurs in the synovium of large joints, characterized by multiple calcified nodules in the lesion area on CT. Uncalcified bodies generates hypointensity on T1WI and hyperintensity on T2WI. There is no hemosiderin deposition in the above two diseases. RA often invades multiple joints with demineralization and narrowing of the joint space. On CT or MRI, ABCs demonstrate as expansile osteolytic lesions with thin sclerotic margins, and the typical features are fluid-fluid levels with low signal intensity in the inferior layer and high signal intensity in the superior layer on T2WI. Cholesteatoma commonly affects the petrous part of temporal bone, presenting as bone destruction, accompanying with homogenous density soft tissue mass on CT, and the lesion manifests as remarkable high intensity on T2WI. Other diseases addressing preauricular swelling such as brown tumor, and synovial sarcoma are also needed to be taken into differential diagnosis. With the rare incidence, accurate diagnosis can be challenging before operation. Solid mass generating high-density on CT and hypointensity on MRI with cystic changes may be of some specificity to D-TSGCT of TMJ, in combination with clinical history and histopathological features suggest the diagnosis.Recurrence rates of D-TSGCT after treatment varied ranging from 9 to 46%, depending on the joint affected and the length of follow-up , 25. The“Four years ago, I started to find hearing loss in my right ear, the symptoms worsened gradually, and the mouth opening was restricted. I went to the community hospital first and was diagnosed as cholesteatoma. Afterwards, I went to the department of otorhinolaryngology, orthopedics and neurosurgery of West China Hospital, hoping to receive thorough treatment. After various examinations, the doctor told me that my disease might be a benign bone tumor and suggested the surgery to remove the mass. After the pathological examination, I knew that my disease is a rare tumor that occurs in the temporomandibular joint. My symptoms gradually improved after the operation and there are no signs of recurrence so far. The doctors and nurses in West China Hospital were nice, and the attention and care I received was well-organized and excellent. I do not mind my case being reported and happy that other doctors learning from that.”We describe a rare case of D-TSGCT in the TMJ without involvement of the condyle head and its CT and MRI characteristic, which is highly valuable in establishing the diagnosis.The original contributions presented in the study are included in the article/Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.XY and LY performed the data acquisition. XY, LY, and XD performed the radiological analysis of MRI and CT images. TY performed the histopathological observation. XY, LY, and QY performed the manuscript preparation. All authors contributed to the article and approved the submitted version.This work was supported by the Sichuan Provincial Foundation of Science and Technology (Grant No. 2019YFS0428 and 2021YFS0077), the Foundation of the National Research Center of Geriatrics (Grant No. Z2018A07) and Science and Technology Project of the Health Planning Committee of Sichuan (19PJ078 and 19PJ247).The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Leber’s congenital amaurosis (LCA) is a known inherited retinal disease (IRD) associated with severe visual loss, nystagmus, amaurotic pupils, oculo-digital sign and markedly reduced or absent electroretinograms (ERG). Retinal astrocytic hamartomas (RAH) is a benign vascularized glial tumor of the retina. There is no known association of these two entities, more so in siblings.A pair of siblings diagnosed as LCA who presented with RAH with no extraocular symptoms or signs of phakomatosis were imaged. Multimodal imaging was performed and are elaborately described in this article.LCA in siblings with multiple RAHs is an extremely rare association. Recent advances in retinal imaging tools have aided in diagnosing even subtle and early RAH with high sensitivity using Infrared imaging (IRI) and Optical coherence tomography (OCT). Lebers Congenital Amaurosis (LCA) is an inherited retinal disorder (IRD) which is characterized by severe visual loss which maybe present congenitally or in early infancy. It is associated with nystagmus, amaurotic pupils, oculo-digital sign and markedly reduced or absent electroretinograms (ERG) . We repoAn 8 years old boy presented with complaint of painless visual deterioration in both eyes (BE) since early childhood. On examination, the best corrected visual acuity (BCVA) in the right eye (RE) was 6/18, N18 and in the left eye (LE) was counting finger at 10 cm, N60 with nystagmus in BE. Supero-nasal subluxation of the lens was present in BE. Applanation tonometry was normal in BE. Fundus examination of BE showed normal optic disc with generalized attenuation of vessels with retinal pigment epithelium (RPE) atrophy and pigmentary alterations in the retina. A reddish-orange hue was seen at the fovea and atrophic maculopathy was noted. There were characteristic multiple off -white relatively well defined lesions, which were more marked along the arcades in the RE with a single lesion located on the inferonasal retinal quadrant in the fellow eye  depicted sub-foveal thinning with RPE atrophy in the RE Fig. a. Scans SS-OCT  through the fovea of RE showed an opaque lesion arising in the NFL with a smooth transition from retina and posterior shadowing. All other retinal layers appeared compressed along with mild thinning of choroid beneath the tumorous lesion Fig. a. HoweveSystemic evaluation by a pediatrician revealed no signs of Tuberous sclerosis or any associated syndrome. There were no systemic features of renal or hepatic dysfunction, neurological deficit, phacomatoses or deafness. MRI (Magnetic resonance imaging) cranium and abdominal ultrasound was normal along with normal renal functions. Gene mapping could not be performed due to limited financial resources.His pedigree charting revealed a high refractive error in his sister. She was also assessed for any associated syndromes. She was 11 years old, and her BCVA was 6/9, N6 with normal slit-lamp biomicroscopic findings in both eyes. Fundus evaluation revealed some pigmentary alteration in BE with normal macula Fig. . An isolBased on the above clinical findings of severe visual loss, nystagmus, hyperopia, amaurotic pupils, abnormally pigmented fundi and macular atrophy and with detailed systemic investigations, a diagnosis of presumed non- syndromic LCA with RAH was made. Since multiple alleles have been associated with LCA, gene therapy is highly costly  and sincThe prevalence of LCA is between 1 in 33,000 to 1 in 81,000 and accounts for ≥5% of all IRD . It is cAmbiya et al.  reportedRecently, studies on RAHs have highlighted the role of IRI and SD-OCT in detecting subtle changes which were otherwise not visible on clinical examination or colored fundus photography or AF , 7 whichIn conclusion, we describe siblings with clinical and ERG findings suggestive of LCA associated with RAHs. The RAHs appear as hyper-reflective dome shaped lesions at the level of NFL and reveal a feeder vessel on the OCT-A scans. We furthermore highlight the role of IRI in detecting subtle changes which are otherwise not detectable on fundus examination and monitoring these patients.


Growing teratoma syndrome is a rare condition described in both testicular and ovarian cancer. We present a case of a 26-year-old male with known mixed germ cell tumor which exhibited new and progressive secondary lesions during imaging surveillance, later to be histologically characterized as teratomas. Growing teratoma syndrome (GTS) was first reported by Logothetis in 1982, as a rare entity among patients with nonseminomatous germ cell tumors, characterized by growing masses developing during or after chemotherapy despite normal levels of tumoral biomarkers.A close imaging surveillance is essential for early recognition of GTS, particularly in patients with risk factors, and because of the chemoresistant nature of GTS, there is a need to prevent further uneffective systemic therapy. Surgical resection is currently the gold standard for GTS treatment.Although GTS typically has a good prognosis, regular follow-up is critical to detect malignant degeneration.A 26-year-old male was referred to our institution to study a potential mass of the right testicle, noted in the previous weeks on physical exam. The patient had no significant medical background or history of previous testicular trauma. A testicular ultrasound and serum tumoral biomarkers were ordered.A testicular ultrasound was performed which showed an enlarged right testicle (estimated volume of 32 cc), presenting highly heterogenous parenchyma with multiple nodules, most of them hypoechogenic and demonstrating high vascularization. A right moderate hematocele was also present. Doppler analysis also showed bilateral varicocele , without significant reflux with Valsalva maneuvers. This finding precluded an abdominal ultrasound to exclude extrinsic causes for venous congestion. It revealed one superior retroperitoneal lymphadenomegaly with almost 4 cm on its major axis, associated with densification of its surrounding fat. Ultrasound findings suggested a testicular mass suspicious for malignancy with metastatic retroperitoneal lymph node .A thoracoabdominopelvic computed tomography (CT) with IV contrast was performed for staging, confirming a retroperitoneal lateroaortic lymphadenomegaly and also finding a left supraclavicular lymphadenomegaly (2 cm of major axis). No other signs of secondary lesions were found Figures .N 1-8 mg/mL)), chorionic gonadotropin (hCG -4.3 mUI/mL (N 0-4 mUI/L)), and lactate dehydrogenase (LDH -239 U/L (N 135-225 U/L)) were elevated  was chosen based on lymph node spreading.At the beginning of chemotherapy, about 2 months after surgery, tumoral markers reached a maximum peak. Then, in the following two months, they finally decreased to normal values and remained normal until the present day .On the other hand, follow-up CT imaging, performed about every 2 to 3 months, demonstrated increased number and larger cervical and retroperitoneal lymph nodes. Furthermore, it showed new mediastinal lesions with similar appearance—diffuse hypodensity—suggesting cystic/necrotic nature Figures  and 7.Five months postsurgery, when the chemotherapy regimen was completed, a microbiopsy was performed on a left cervical lymphadenomegaly . Since tOn the following multidisciplinary team meeting, excision of the cervical and retroperitoneal secondary lesions was proposed and the patient underwent cervical and retroperitoneal lymphadenectomies without major complications.At 24 months of surveillance, tumoral biomarkers remained normal, and there were not new cervical or retroperitoneal secondary lesions to report on imaging. However, the mediastinal lesions persisted and showed slow size progression over time, but considering the known risks associated with their anatomical location, an active surveillance approach was decided. At the moment, the patient is compliant with the follow-up protocol without major events or complications associated.Growing teratoma syndrome (GTS) was first described by Logothetis in 1982, as a rare entity among patients with nonseminomatous germ cell tumors .According to Logothetis, the diagnostic criteria of GTS were (a) normalization of elevated serum AFP and hCG, (b) tumor growth during or after systemic chemotherapy, subsequently to a significant reduction in tumor burden or a disease-free interval, and (c) the exclusive histologicallypresence of mature teratoma in the resected specimen , 7.GTS is now considered a condition related to both testicular and ovarian cancer (NSGCT), with a prevalence of about 1.9–7.6% in patients with testicular NSGCT [The most common site of involvement is the retroperitoneum, but it can be found in other locations such as the pelvis, chest, liver, or even in the cervical region as our case , 7.GTS presentation is very variable regarding disease time course: during chemotherapy, after a large disease-free period, or after an initial reduction in size during chemotherapy, followed by regrowth , 7.The etiology is still uncertain but there are two main hypotheses described in the literature based on the assumption that embryonal cells are pluripotent and that their origin results from differentiation of tumoral cells. One theory states that chemotherapy only cures the immature malignant cells but not the mature elements, which remain untreated and can transform into benign teratomatous elements. The other theory explains the transformation of a totipotent malignal germ cell into a benign mature teratoma by cell kinetics alterations stimulated by chemotherapy. There is still another lesser known hypothesis proposed by Hong et al., in which inherent and spontaneous differentiation of malignant cells into benign cells is pointed out as the cause of this pathology , 7–10.Since this entity is rare, it is difficult to infer a statistically significant association between the primary pathology and the likelihood of GTS .The most common risk factors include presence of mature teratoma in the primary tumor, incomplete resection after chemotherapy, no size reduction or even growth of metastatic lesions during chemotherapy, and presence of mature teratoma tissue in postchemotherapy residual masses , 11.Among these mentioned risk factors, our patient presented with the two last ones.However, according to recent studies, the only predictive factor associated with GTS syndrome seems to be cystic/necrotic lesions in the preoperative imaging as well as areas of low density on them at postchemotherapy CT scans compared to pretreatment images. Therefore, close imaging surveillance is crucial for early recognition of GST, particularly in patients with risk factors , 12, 13.The residual masses of GTS do not demonstrate enhancement with 18-FDG PET (positron emission tomography) scans, which can be helpful in the differential diagnosis .The chemoresistant nature of GTS makes additional cycles of chemotherapy ineffective; thus, early recognition is critical to prevent further systemic therapy and to allow for readily surgical planning. Surgical resection is currently the gold standard treatment for GTS. Despite the large tumor burden, complete resection is reported to be often curative. Failure to promptly intervene leads to death from either compression of vital organs and/or subsequent malignant transformation , 7.Giant GTS is a variant of this syndrome, with extensive retroperitoneal disease, defined as lesions with larger than 10 cm. Circumferential involvement of major vascular structures is often seen. Surgery is usually technically challenging but should not be disregarded [Although GTS generally has a good prognosis, regular follow-up is critical, as malignant degeneration could occur. Imaging findings and tumor markers can suggest the presence of malignant lesions, but surgical excision or sampling of the lesions is mandatory to confirm the diagnosis and other differential diagnosis as incomplete response to chemotherapy or recurrent malignancy .GTS is rare condition related to both testicular and ovarian NSGCT with generally good prognosis, but close imaging surveillance is crucial for early recognition, particularly in patients with risk factors. Surgery is the gold standard treatment.


Nowadays, the early diagnosis of tumoral diseases is more possible and accurate with multiple diagnostic imaging modalities such as chest X-ray, echocardiography, computed tomography, and magnetic resonance imaging, especially for cardiac tumors which are usually asymptomatic, even in large sizes. In cardiac masses, the patients’ presentations are non- specific and dependent on the tumor size and site as well as its compressive effect on the adjacent structures. On the other hand, the first and last signs could be sudden cardiac death. However, cardiac masses are either benign or malignant and metastatic in their malignant type, and their definite diagnosis is only possible by surgical tumor resection and tissue biopsy. In this paper, we describe an old patient with severe pericardial effusion and an unusual intrapericardial tumor in transthoracic echocardiography, representing a rare case of a giant ectopic thymoma after surgical resection and pathologic assessment. They can be asymptomatic and benign or malignant with fatal invasion to the adjacent mediastinal structures. Ectopic thymomas originate from migrated embryonic thymic cells and can be found in the neck, lungs, and pleural and pericardial spaces. With regard to their unusual site, the most important point is to differentiate them from other mediastinal tumors.A 73-year-old man with a history of controlled hypertension and progressive dyspnea of 3 months’ duration  and recent nonproductive coughs referred with the water-bottle heart sign in his chest X-ray, suggesting pericardial effusion . PhysicaA 2D transthoracic echocardiographic examination showed severe circumferential pericardial effusion up to 25 mm anterior to the right ventricular outflow tract without right atrial and right ventricular collapse 5-8 Cardiac masses are usually benign or secondary to malignant extracardiac tumors, and their definite differentiation is only possible by surgical tumor resection and tissue biopsy.4In patients with cardiac masses, the presentation is nonspecific and is dependent on the size, site, and compressive effects of the tumor on the adjacent structures; nevertheless, the condition could prove fatal, with the first and last signs being sudden cardiac death.9Germ cell tumors (tratomas and yolk sac tumors) are the most prevalent benign tumors of the pericardium, and sarcomas are the most malignant ones. About 50% of benign pericardial tumors are tratomas, but rare cases of ectopic tumoral tissues of thyroid and thymus origins have been reported in the literature too.10 Thymomas constitute a type of anterior mediastinal tumors and are asymptomatic, even in large sizes, in most patients. There have been a few cases of ectopic thymomas at unusual sites such as the neck, lung, chest wall, and pleural and pericardial space. Ectopic thymomas may originate from distributed ectopic thymic cells whose primary migration has failed. Thymomas might be symptomatic due to progressive compressive effects, invasion to the adjacent tissues, and associated immunologic disorders such as myasthenia gravis.11Transthoracic echocardiographic is the most available and cost-effective noninvasive cardiac imaging, and it is used as the first choice inasmuch as it can reveal the properties of the mass such as its size, mobility, attachment site, and compressive effects.12Three-dimensional echocardiography has an additive value in that it is able to assess the volumetric properties of the mass. The cropping techniques available with 3D echocardiography can help identify benign and malignant masses with regard to the homogeneity, vascularity, and calcification of the tissue.13CT and cardiovascular magnetic resonance imaging, considered complementary imaging techniques to 2D and 3D echocardiography imaging, can boost diagnostic accuracy and help decision-making for the proper therapeutic approach. The individualized modality selection is essential regarding the patient’s condition and the probable diagnosis. The role of these modalities is more prominent in the detection of the invasion and other associated complications of the mass, especially as regards pericardial tumors.1 The maximum size reported thus far is 7.5 cm, while our patient’s tumor was about 13 cm in size. Its histologic type was similarly a rare form of the ectopic thymoma B3. Our review revealed rare cases of ectopic intrapericardial thymomas, and the present case adds to the existing limited literature on intrapericardial large thymomas without invading the adjacent structures. This case underscores the key role of multimodal imaging in the early diagnosis and management of cardiac tumors; be that as it may, the ultimate diagnostic strategy and the mainstay approach vis-à-vis the treatment of cardiac tumors is surgical resection and lesion biopsy.2, 3According to the latest literature review of ectopic thymomas in the middle mediastinum (13 reports in English), most patients were women at a mean age of 59.2 years. Only 2 patients were symptomatic, and the histologic type B3 was seen only in 1 patient.3Imaging studies can assist in the description of the differential diagnosis of the mass as well as its size, compressive effects, extension, and invasion characteristics, but the most accurate method for diagnosing the nature of the tumor is the pathologic assessment of the tissue. A definite diagnosis is often impossible on the basis of merely imaging findings, especially when the tumor occurs in unusual locations, and complete surgical excision without the penetration of the tumor capsule can be diagnostic and curative.In light of the case presented herein, it should be borne in mind that pericardial effusion in the elderly can be a sign of malignancy and the early detection and diagnosis of associated lesions can be life-saving. Although it is not possible to determine the type of tumor in the initial evaluation, it is necessary to remember that rare different diagnoses and different cardiac imaging techniques can be helpful.To watch the following videos, please refer to the relevant URLs:http://jthc.tums.ac.ir/index.php/jthc/article/view/1049/890Video 1. Severe circumferential pericardial effusion without compressive effects on the cardiac chambers in 2D transthoracic echocardiography


Mucosal Schwann cell hamartomas are rare neurogenic tumors which present most commonly in the distal colon. They are usually discovered as small, single polyps in asymptomatic patients.An asymptomatic 64-year-old man was referred to us after a 12 mm subepithelial lesion was discovered incidentally on screening colonoscopy. Follow-up colonoscopy conducted 2 months later revealed that the tumor had disappeared, leaving multiple edematous, submucosal tumor-like elevations presenting as skip lesions throughout the sigmoid colon. Lesions had elongated pits on magnifying endoscopy and were limited to the first layer on endoscopic ultrasound. Biopsies revealed unclearly delineated foci of spindle-shaped cells limited to the lamina propria. On immunohistochemistry, all lesions were positive for S-100 protein and negative for CD34, CD56, and neurofilament protein. The patient was diagnosed with multiple mucosal Schwann cell hamartomas of the sigmoid colon. He remains asymptomatic after 18 months of follow-up, but the lesions have also remained unchanged.We report a case of multiple non-polypoid mucosal Schwann cell hamartomas. Endoscopic findings may assist in the differential diagnosis, particularly when presenting as non-polypoid, submucosal tumor-like lesions. Mucosal Schwann cell hamartomas (MSCH) are rare neurogenic lesions found in the lamina propria of the gastrointestinal tract. They arise most commonly in the distal colon and are generally asymptomatic small, single sessile polyps which may be discovered incidentally on screening colonoscopy . MSCH haThe differential diagnosis of neurogenic tumors in the colon includes mucosal neuroma which is associated with multiple endocrine neoplasia type 2B (MEN 2B), neurofibroma which is associated with neurofibromatosis type 1 (NF1), schwannoma, ganglioneuroma, intramucosal perineuroma, and mucosal benign epithelioid nerve sheath tumor , 6.While there are currently over 80 reports of colorectal MSCH in the English literature, reports of non-polypoid MSCH and of multiple MSCH are scarce , 7. FurtA 64-year-old man was referred to our hospital after a 12 mm subepithelial lesion in the sigmoid colon was discovered incidentally on screening colonoscopy (Fig. Follow-up colonoscopy conducted 2 months later revealed that the tumor had disappeared completely, leaving numerous edematous, submucosal tumor-like protrusions presenting as skip lesions throughout the sigmoid colon, over a length of 15 cm (Fig. Multiple biopsies of the thickened mucosa all revealed proliferation of uniform spindle-shaped cells in the lamina propria Fig. a–c. EosiUpon detailed questioning, the patient denied any personal or family history of café au lait spots, neurofibromatosis, or thyroid or adrenal disease which could suggest MEN 2B or NF1. No relevant findings were found in a dermatological examination, laboratory tests, or in imaging studies. Based on the above, the patient was diagnosed with multiple MSCH of the sigmoid colon.The patient remains asymptomatic without treatment during 18 months of follow-up. However, the multiple MSCH lesions in the sigmoid colon remain unchanged despite frequent colonoscopies. Pathological findings consistent with MSCH are also observed on follow-up biopsies taken at every colonoscopy.Gibson et al.  first reBae et al.  reportedThere are only 3 other reports of non-polypoid MSCH. The first presented as many small whitish nodules somewhat similar to lesions found in our case, the second discovered incidentally on random biopsy conducted for evaluation of chronic diarrhea, and the third was located at the mass base following endoscopic mucosal resection , 8. It iDetails on endoscopic MSCH findings in the literature are scarce . ElongatThe cause of the vanishing tumor identified by the referring physician remains a mystery. Reports of vanishing colonic tumors include cytomegalovirus infection, angioedema resulting from acquired type II C1-inhibitor deficiency, and colorectal cancer –11. VaniIn conclusion, we report a case of multiple MSCH presenting as edematous, submucosal tumor-like lesions in the sigmoid colon. NBI and endoscopic ultrasound findings may help the endoscopist to suspect atypical MSCH which does not take the form of a small sessile polyp. Discovery of similar cases may shed light on the pathogenesis of this seemingly harmless entity in the future.


A football player was diagnosed with myositis ossificans of his right adductor longus muscle after an acute injury. Conservative treatment failed and 1 year after the initial trauma the patient underwent surgical excision of a large ossification. Seven months postoperatively, the patient was fully recovered and returned to his preinjury activity levels. We present our approach to this case and discuss our considerations, referring to background information about this rare disease. Myositis ossificans (MO) is a non-neoplastic heterotopic ossification that develops within skeletal muscle or soft tissue. The exact pathophysiological pathway of this rare disease is still not fully understood. It is believed that, in response to tissue injury and subsequent inflammation, stem cells are dysregulated and stimulated to follow osteogenic pathways, resulting in ossification.MOT can be mistaken for malignant lesions such as osteosarcoma or rhabdomyosarcoma. Therefore, appropriate diagnostic tests are of paramount importance. However, the stage of the disease affects its presentation and might lead to difficulties in the interpretation of radiological and histological findings.As MOT is self-limiting and self-resolving in most cases, conservative treatment with rest, adequate pain relief, and restoring function and range of motion is often effective for returning to sports and activities.We present the first reported case of MOT of the adductor longus muscle in a football player, which was treated successfully with surgical excision.A 23-year-old male football player, without any relevant medical history, was referred by the general practitioner to our outpatient clinic with complaints of the right groin. One year earlier, during intensive training, the patient felt acute intense pain in the right groin twice when kicking the ball. After training, he felt swelling and pain in the right adductor region. After a period of rest, physiotherapy was started. Low-intensity exercises were gradually performed and he started cycling. He tried to resume his football training sessions. Unfortunately, the pain in his groin recurred. He repeated physical rehabilitation from the beginning three times. However, the intensity of the pain increased over time and began to also limit his activities of daily living.On physical examination, palpation of the origin of the adductor muscles at the right pubic bone was painful and revealed a small gap in the tendon located just distal to the insertion. Distal to the gap, a very firm mass of about 7 cm long and 1 cm wide could be palpated. It felt as if a short pencil was lying under the skin. Palpation of the mass was extremely painful. The right adductor muscle was shortened and resisted adductor muscle strength testing was weak and painful. Further examination showed no signs of nerve entrapment with normal sensation of the skin. Clinical examination of the hip joint range of motion was pain free.Ultrasound imaging showed an old rupture of the right adductor longus muscle with a small fluid collection located proximally. Distally, a calcification of 8 cm was seen, indicating a chronic disease process with signs of active inflammation.An X-ray clearly showed a heterotopic ossification of the right adductor longus muscle .The differential diagnosis of MOT includes benign and malignant diseases. Benign diseases that may be confused with MOT are local abscess formation, a calcified fibrous tumour and periosteal reaction, whereas osteosarcoma and rhabdomyosarcoma are important malignant diseases to consider.Surgical excision of the MOT was performed. Incision was made in the direction of Langer’s lines at the level of the palpable mass. Subsequently, the great saphenous vein was ligated. After identification of the mass, it was peeled off the adductor longus muscle in one piece . The musHistopathological analysis showed a round nodulus consisting of bone tissue with bone trabeculae surrounded with osteoblast cells. No typical zones could be recognised and the centre of the nodulus did not contain any cells.At 3 months of follow-up, the patient was free of symptoms and followed the rehabilitation programme at his local football club. Exercises were intensified using weights, as well as with cycling, running and resuming light training sessions. No abnormalities were found on physical examination of the adductors on palpation, stretching and resistance testing. Dynamometry showed adductor strength of 157 N on the right (R) and of 177 N on the left (L), add abductor strength as this is needed to calculate with adductor/abductor ratio of 1.03 R and 1.32 L.At 5 months of follow-up, the patient, although not at full intensity, participated in football training sessions three times a week. Dynamometry showed adductor strength R of 181 N and L of 176 N, with adductor/abductor ratio of 1.16 R and 1.17 L.After 7 months, the patient was fully recovered and participated in football matches without any pain. Dynamometry showed adductor strength R of 213 N and L of 190 N, with adductor/abductor ratio of 1.20 R and 1.10 L. All dynamometry outcomes collected during the follow-up period are presented in MOT of the adductor muscles in athletes has been described previously.Surgical excision of MOT can be considered when pain persists, if muscle weakness is present, or in case of a limited range of motion.et alet alImaging can be useful to diagnose MOT correctly. Especially when considering that malignant diseases such as osteosarcoma and rhabdomyosarcoma can have similar clinical presentations.et alGuideline recommendations on return to sports for athletes with MO treated conservatively are lacking, let alone for athletes after surgical excision. Orava To the best of our knowledge, this is the first case of MOT of the adductor longus muscle described in the literature. This case emphasises that surgical excision of MOT of the adductor longus muscle can be an effective option when conservative treatment options have failed.After the last follow-up moment, we asked the patient to describe the clinical process, including the surgical treatment on his perspective. He described this as follows: at first, I was a bit shocked when I was told that MOT of the adductor longus muscle is a rare disease and that it was never seen by the sports doctor. At the same time, I trusted the sports doctor, thanks to his open and professional approach. I was informed about the potential risk in losing some of the muscle strength of my right leg when choosing to undergo surgery. However, surgery was the obvious choice for me because the MOT affected my activities of daily living. After surgery, the pain was slightly worse than before but this reduced quickly and I could resume my activities of daily living. Furthermore, due to the close follow-up and the rehabilitation programme, I experience no (pain) complaints anymore during football and I am able to fully participate in football matches. I am very satisfied with the treatment and the end result.Myositis ossificans traumatica (MOT) of the adductor muscles is extremely rare.Immediate treatment of a skeletal muscle injury with the rest, ice, compression and elevation principle might help in the prevention of MOT.Surgical excision of MOT of the adductor longus muscle is a good option when conservative treatments fail.


However, even with the use of these imaging techniques, since intrathyroid parathyroid tumors exist as a mass within the thyroid, it is often difficult to distinguish from thyroid masses. Although there have been various reports on US images of intraparathyroid tumors, we experienced a case with US images that were distinct from previous reports. Herein we present a case of an intrathyroid parathyroid adenoma (IPA) that was difficult to diagnose, with a main focus on US images.With advances in diagnostic imaging such as ultrasonography (US), computed tomography (CT), and 99mTc-MIBI scintigraphy showed accumulation at the same location in delayed phase, it was difficult to determine the presence of a parathyroid tumor on the image. The patient underwent an operation on April 2019 and the tumor could not be identified on both naked eye and palpation. We used US intraoperatively to define the location and resected the tumor. A parathyroid adenoma was diagnosed by frozen section and the final diagnosis was an intrathyroid parathyroid adenoma.A 53-year-old man with a diagnosis of hyperparathyroidism was referred to our department in December 2018. Ultrasonography revealed a tumor that was located in the inferior pole of the right lobe of the thyroid gland and no parathyroid mass was observed. The tumor had an irregular round shape and showed heterogeneous hyperechogenicity with a defined margin, but within it, there were a few irregular and hypoechogenic area with unclear margins, while the tumor had a mosaic appearance at first glance. Although We experienced an IPA presenting an US image that was atypical and has previously not been reported. IPA has no established US image to confirm the diagnosis and even with the use of other imaging techniques, a definitive diagnosis often cannot be established. Thus, our recommendation based on the current situation is that operation with intraoperative diagnosis using frozen section should be conducted if hypercalcemia and high I-PTH are observed and when localization sites in MIBI and US coincide. With advances in diagnostic imaging such as ultrasonography (US), computed tomography (CT), and accuracy . Howeveraccuracy –3 on US A 53-year-old man with a diagnosis of hyperparathyroidism was referred to our department in December 2018. The results of laboratory examinations were compatible with hyperparathyroidism: intact-PTH 94 pg/ml , serum Ca 11.9 mg/dl , and serum P 2.4 mg/dl . Thyroid function tests  were all within normal limits. Cervical tumors and lymphadenopathy were not identified on palpation. The patient showed no signs of hoarseness, dysphagia and dysphonia. His past medical history included ureter stones and type 2 diabetes, and he had no kidney diseases that can cause secondary hyperparathyroidism. There were no notable family medical histories. US was performed with a 7.5-MHz linear transducer . A tumor was found in the inferior pole of the right lobe of the thyroid gland and no parathyroid mass was observed. The tumor had an irregular round shape and showed heterogeneous hyperechogenicity with a defined margin, but within it, there were a few unclear margins, irregular and hypoechogenic area, and the tumor had a mosaic appearance at first glance  imaging is useful for localization of parathyroid lesions and identification of the size of adenomas or hyperplastic glands , 15. SPESome authors have suggested that it may be possible to determine whether a tumor is of parathyroid origin or not by measuring intact-PTH of the lavage fluid from fine needle aspiration of the lesion . HoweverAt present, it is difficult to make a definitive diagnosis of IPA before operation, although the accuracy of diagnosis of IPA can be increased by combining various imaging techniques. In particular, there are no typical images that are specific enough to establish a definitive diagnosis with US. In the case of suspected IPA, currently, the optimal measure may be to perform resection together with intraoperative US and to confirm parathyroid tissue with frozen section. Development of new diagnostic imaging techniques is warranted for preoperative diagnosis of IPA.We experienced an IPA presenting an US image that was atypical and has previously not been reported. IPA has no established US image to confirm the diagnosis and even with the use of other imaging techniques, a definitive diagnosis often cannot be established. Thus, our recommendation based on the current situation is that operation with intraoperative diagnosis using frozen section should be conducted if hypercalcemia and high I-PTH are observed and when localization sites in MIBI and US coincide.