Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of cutaneous lymphoma, which was first defined as a clinical entity in 1991 as a cytotoxic T-cell lymphoma preferentially infiltrating subcutaneous tissue. Herein, we report 2 patients of SPTCL who are a pair of twin brothers.The disease afflicted the monozygotic twin brothers at different time with an interval period of 5 years. The older twin brother had disease onset at 27 years of age. In June 2012, he developed prolonged fever accompanied by subcutaneous nodules in the left upper arm and left chest due to unknown origin. The younger twin brother had disease onset at 32 years of age. In June 2017, the younger brother presented with repeated high fever for more than 10 days, accompanied by head distension.On August 7, 2012, skin biopsy was performed on the lesion of left upper arm of the older twin brother, and then, a diagnosis of subcutaneous panniculitis-like T cell lymphoma (SPTCL) was made. On June 19, 2017, the younger twin brother underwent whole-body fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography for diagnosis. Soon afterwards, abdominal subcutaneous nodule resection and biopsy was performed on June 28, 2018, and the specimen was diagnosed as SPTCL.For the older brother, a total of 14 systemic chemotherapy sessions were performed from August 16, 2012, to September 21, 2014. For the younger brother, a total of 9 systemic chemotherapy sessions were performed from July 14, 2017, to March 8, 2018, then he was switched to oral chemotherapy with chidamide twice a week for 6 months.The older twin brother died in March 2015, the younger brother has recovered well and is no longer receiving any treatmentTo the best of our knowledge, twin brothers both having this disease has never been previously reported. Moreover, some of the involved areas are also extremely rare detected by fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography at initial stage. It is beneficial to people to gain some new understanding for SPTCL by this special case and some extremely unusual involved sites in the younger twin brother. Patients usually present with multiple subcutaneous nodules and plaques involving the extremities or trunk.,3–5 Dissemination to lymph nodes and other organs on initial presentation is very unusual.,4,6Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of cutaneous lymphoma and belongs to a new subset of peripheral TCL, as determined by the World Health Organization. The SPTCL is characterized by infiltration of neoplastic cytotoxic T cells into subcutaneous tissue.–9 PET/CT may therefore be useful in detecting the occult extracutaneous involvement when staging SCPTL as well as being a useful tool to quantify disease burden and response to treatment.–8The SPTCL lesions can be assessed using various imaging modalities, such as ultrasonography, CT, magnetic resonance imaging, but positron emission tomography/computed tomography (PET/CT) detected many lesions with greater sensitivity than other image devices.–13Genetic, infectious, and environmental factors have been implicated as pathogenic causes for many types of lymphoma, and an inherited genetic lesion of the family could exist and predispose its affected members to the development of the SPTCL.22.1The older twin brother had disease onset at 27 years of age. In June 2012, the patient developed prolonged fever accompanied by subcutaneous nodules in the left upper arm and left chest due to unknown origin, and visited the rheumatology department of another hospital for treatment. The initial diagnosis was considered to be panniculitis and the patient was treated with methylprednisolone, but the therapeutic effect was poor. On August 7, 2012, skin biopsy was performed on the lesion of left upper arm, and the biopsy specimen showed infiltration of subcutaneous fatty tissue by various-sized atypical lymphocytes with partial apoptosis. Immunophenotype study revealed positive staining for CD3, CD5, CD8, and Granzyme-B, but negative staining for CD20, CD30, CD56, and CD79a. Hence, a diagnosis of subcutaneous panniculitis-like T cell lymphoma (SPTCL) was made.A total of 14 systemic chemotherapy sessions were performed between August 16, 2012, and September 21, 2014. The patient first underwent 2 cycles of VDCLP  regimen chemotherapy, but the effect was poor, with no significant improvement in body temperature. Between October 2012 and May 2013, the chemotherapy was changed to ESHAP  regimen, which lasted for 6 cycles. Three months later, new subcutaneous nodules appeared in the neck and left upper arm of the patient, and a biopsy indicated recurrence of lymphoma. In September 2013, the patient was given 1 cycle of GDP chemotherapy. In October 2013, another GDP chemotherapy cycle was given. Subsequently, the patient's body temperature improved and skin nodules were significantly reduced. In April 2014, the patient revisited our hospital due to fever and new systemic subcutaneous nodules. The bone marrow biopsy on the left posterior superior iliac spine was performed, which demonstrated numerous atypical lymphocytes, and a diagnosis of T-cell lymphoma involving the bone marrow was considered . The PET/CT showed multiple systemic metabolically active lesions , with increased fat density and FDG uptake demonstrated on whole body maximum intensity projection and axial images  chemotherapy, followed by 2 cycles of pegaspargase with CHOPE  chemotherapy. Based on the patient's abdominal CT findings, lactate dehydrogenase level, recent fever, and clinical progression, the chemotherapy regimen was changed to 2 cycles of GDP  regimen on October 23, 2017. On January 2, 2018, systemic PET/CT indicated tumor progression, and the chemotherapy regimen of the patient was subsequently adjusted to 3 cycles of cladoxabine + mitoxantrone + dexamethasone. Then the patient was switched to oral chemotherapy with chidamide twice a week for 6 months. No active lesion of lymphoma was found in PET/CT reexamination at December 2018 during the treatment of chidamide. The patient has recovered well and is no longer receiving any treatment.Ethical approval was not required, because this paper is a case report with the clinical information of the patient. Informed written consent of publishing this case report and accompanying images were obtained from the second patient who was on behalf of himself and his late twin brother.3,2Subcutaneous panniculitis-like T-cell lymphoma, as strictly defined by World Health Organization-European Organization for Research and Treatment of Cancer classification, is a rare cytotoxic α/β T-cell lymphoma (<1% non-Hodgkin lymphoma), characterized by primary involvement of subcutaneous tissue mimicking panniculitis. The typical case shows positive staining for CD3, CD8, TIA-1, granzyme, and BetaF1 and negative staining for CD4, TCR gamma, CD56, or EBV by in situ hybridization.Histologically, SPTCL is characterized by a lymphohistiocytic infiltrate, primarily confined to the interstitium of fat lobules in a pattern resembling lobular panniculitis. Lymphoid cells tend to surround individual adipocytes and have a characteristically rimmed appearance.,3 SPTCL may be associated with autoimmune disease. Patients usually present with solitary or multiple subcutaneous nodules and plaques involving the extremities or trunk, with constitutional symptoms that may include fever, myalgia, chills, and weight loss. Most patients show several abnormalities, primarily including anemia, leukopenia, and/or thrombocytopenia, as well as increased levels of liver enzymes. The disease may be complicated by a hemophagocytic syndrome, which is generally associated with a rapidly progressive course.,5SPTCL occurs in adults as well as in young children, and it has a slight female predilection. The median age at diagnosis is 36 (range 9–79) years, with approximately one-fifth of cases occurring in patients younger than 20 years.,4,6 Babb A reported a case of SPTCL with lesions involving the fat space between the muscles of bilateral chest wall. In our second case, the fat space in the muscles of upper left chest wall was involved and bilateral extra-pleural fat lesions were also observed, which has not been previously reported. Involvement of intra-abdominal fat in SPTCL has been described in recent years.,4,7,8 However, these studies only reported the involvement of mesenteric fat. In our second case, not only colorectal mesentery had several lesions but also the patient's right renal dispose capsule was diffusely involved, and the bilateral extra-peritoneal spaces were also involved, which has not been previously reported. Lymph node involvement is also rare. Kim JW reported 8 patients of SPTCL, of which only 1 showed involvement of the iliac lymph nodes. Lack of lymph node involvement may obscure the diagnosis of lymphoma on CT. Lymph node lesion was also seen in our case 2, which was located in the right axilla.Dissemination to lymph nodes and extra-cutaneous sites on initial presentation is very rare.,6 The imaging appearance of fat necrosis is mainly determined by the extent of the associated fibrotic reaction, along with the volume of liquefied fat and the presence of calcifications. When minimal fibrosis occurs, fat necrosis appears as a radiolucent mass or as an oil cyst, and the reparative fibrotic process may entirely replace the necrotic fat, resulting in a focal dense mass on plain X-ray or CT image.,4,9SPTCL is characterized by multiple, nodular, or diffuse soft tissue lesions involving subcutaneous fat tissue. Subcutaneous fat necrosis is the primary radiological imaging feature of SPTCL.,6–8 Our second case also showed FDG-avid lesions in the initial pre-treatment PET, which correlated with a high index of proliferative activity of lymphoid cells. The clinical application value of PET/CT in SPTCL diagnosis is as follows:On FDG-PET, SPTCL lesions appear as multiple foci of FDG uptake, and often show dramatic resolution after chemotherapy.(1)FDG PET/CT can clearly show the extent and degree of SPTCL lesions, which provides valuable information for detecting occult lesions.(2)FDG PET/CT can guide the selection of clinical biopsy sites and improve the accuracy of puncture.(3)Chemotherapy is currently the main treatment for SPTCL. There are many chemotherapies for SPTCL, which offers a wide range of options for clinicians. It is necessary to monitor the curative effect of chemotherapy in the early stage and adjust the chemotherapy regimen in time. FDG PET/CT can play a very important role in disease staging and monitoring of treatment response, which help in choosing the first-line therapy.(4)PET/CT can be used for follow-up and prognosis of SPTCL. Schneider BF reported a familial occurrence of cutaneous T cell lymphoma (CTCL) in 1995, in which the patients were monozygotic twin sisters. Although the CTCL in that report was not SPTCL, it suggests that T cell lymphoma may have familial inheritance. Genetic, infectious, and environmental factors have been implicated as pathogenic causes for CTCL.In this report, the 2 patients were monozygotic twin brothers. This is the first report of twins with SPTCL. Gau JP reported a familial occurrence of SPTCL, in which the disease afflicted a boy and his non-twin elder sister at different times with an interval period of 11 years, but their eldest brother did not have any relevant disease. Highest lifetime risks were found when NHL was diagnosed in a twin (3%–13%), a sister (1.9%–2.5%) or 2 first-degree relatives (2.1%).Familial aggregation of NHL has been reported in 3.5% of newly diagnosed patients, suggesting the existence of a hereditary subset.In summary, SPTCL is characterized by multiple nodular or diffuse soft tissue lesions involving subcutaneous fat tissue, but it can also involve lymph nodes, and extra-cutaneous sites. The presence of multiple subcutaneous nodules detected by CT throughout the body may be an important finding to suggest a diagnosis of SPTCL. FDG PET/CT detected many lesions with greater sensitivity than did physical examination or CT in this report. FDG PET was valuable in monitoring the treatment response and detecting the extra-cutaneous lesion in SPTCL. SPTCL can have familial inheritance, and a family history of SPTCL diagnosed at any age can be considered as a risk factor for SPTCL in relatives, especially when SPTCL was diagnosed in a twin, a sister or 2 first-degree relatives.Shen XZ designed the research; Shen XZ and Luo ZY wrote the paper; Yu SL and Liu F provided and analyzed the pathological images.Conceptualization: Xun-Ze Shen.Formal analysis: Zhou-Ye Luo.Resources: Shan-Lu Yu, Fang Liu.Writing – original draft: Xun-Ze Shen.Writing – review & editing: Xun-Ze Shen.


We report the case of a 69-year-old male patient with extensor pollicis longus (EPL) tendon rupture associated with a scaphoid nonunion advanced collapse (SNAC) wrist. He could not actively extend the left thumb interphalangeal joint and visited our institution for an examination. Plain X-rays revealed advanced stage SNAC and an enlarged soft tissue shadow owing to dorsal ridge growth. The patient was diagnosed with EPL tendon subcutaneous rupture due to SNAC. During surgery, the EPL tendon was found to be absent, a proximal-type scaphoid nonunion was detected, and bone growth to the dorsal part of the dorsal ridge was observed. Considering that the EPL tendon rupture was associated with the bone growth, we performed scaphoid lunate advanced collapse (SLAC) reconstruction and extensor indicis proprius tendon transfer which needed a revision tendon surgery afterward. To the best of our knowledge, EPL tendon ruptures caused by SLAC or SNAC are considered rare and have not yet been reported. Rupture of the extensor pollicis longus (EPL) tendon may result from fractures, surgery, trauma, rheumatoid arthritis, or corticosteroid use. We report the case of a patient with an EPL tendon rupture associated with a scaphoid nonunion advanced collapse (SNAC) wrist  and a liThe patient, a 69-year-old man, could not actively extend the left thumb interphalangeal (IP) joint approximately 10 days before presentation and was referred to our institution for an examination. The patient had experienced pain in the wrist joint since a long period. However, the patient did not participate in sports and had no recollection of any trauma and infection. His medical history included pyogenic spondylitis and autoimmune hepatitis, and he had been undergoing corticosteroid therapy (5 mg) for 1 year. Although inability of active extension of the left thumb and swelling in the snuff box were observed, there was no tenderness. Grip strength was 25 kg in the right hand and 11 kg in the left hand, right wrist extension and flexion were 65° each, and left wrist extension and flexion were 60° and 50°, respectively.Plain X-rays revealed advanced stage SNAC in the left hand; supinated oblique view revealed an enlarged soft tissue shadow due to the growth of the dorsal ridge . We diagWe performed the surgery 10 weeks after the initial diagnosis, because the patient's general condition was not favorable due to pyogenic spondylitis. During the surgery, when the dorsal part of the wrist was opened, the EPL tendon was found to be absent. We noted scarring surrounding the snuff box, perforation of the joint capsule, and a cartilage defect in the capitate head. A proximal-type scaphoid nonunion was detected, and bone growth to the dorsal part of the dorsal ridge was observed . The EPLAt 6 months after surgery, extension and flexion in the left wrist were 30° and 35°, respectively. The patient was unable to extend his thumb, improvement in the transferred tendon function could not be confirmed, and the transferred tendon was not palpable in the snuff box . AccordiEPL tendon rupture is caused by mechanical irritation due to Lister's tubercle and rheumatoid arthritis. Björkman and Jörgsholm  retrospeFJ Harvey and PM Harvey  describeIn the present patient, a SNAC wrist due to scaphoid nonunion was observed, which we believe caused the EPL tendon rupture. Although the patient reported corticosteroid use, it was unlikely that this was the cause of the tendon rupture owing to the low corticosteroid dose; however, the possibility of an effect could not be ruled out.Although SLAC reconstruction was performed as a therapeutic approach, the transferred tendon rerupture was observed. Because the transferred tendon rerupture occurred in the dead space where the scaphoid bone was resected, ischemia was considered the cause. Although the surgery was performed by an experienced surgeon, the possibility of technical errors occurring during surgery cannot be ruled out.To the best of our knowledge, EPL tendon ruptures caused by SLAC or SNAC are considered rare and have not yet been reported.


Intraosseous schwannomas represent an extremely rare subgroup of schwannomas, accounting for <1% of all primary bone tumors. They mostly occur in the mandible, the maxilla, the sacrum, and they are also seen in long bones. We herein report a rare presentation of an intraosseous schwannoma in the glenoid of a 49-year-old patient. She complained of shoulder pain and was referred to the orthopaedic oncologist after detection of a suspicious lesion on imaging. Biopsy revealed benign spindle cells and immunohistochemistry was positive for S100. Because of the rarity of these intraosseous schwannomas it is important to recognize their radiological and histological features and make a differential diagnosis with other lytic tumors. Only if these characteristics are recognized, correct treatment can be given with definite curettage and bone grafting and correct follow-up with avoidance of unnecessary adjuvant therapy. Schwannomas are benign nerve sheath tumors, comprising approximately 5% of all benign soft tissue tumors. They arise from Schwann cells that form the myelin sheath, insulating the nerve . There iA 49-year-old female patient presented to our orthopedic unit with left shoulder pain for 3 months. She had a history of cervical scoliosis. The pain had been increasing progressively with exertion and was situated posteriorly on the scapula and trapezius muscle, but also in the deltoid region spreading to the distal part of the arm. She was complaining of paresthesia in the hand, without any evidence for a specific dermatome delineation. She experienced the exact same complaints 2 years ago. Magnetic resonance imaging (MRI) of the cervical spine was performed at that time together with electromyography in another hospital, which did not show any explanation for her pain. Eventually, her complaints improved with acupuncture. On physical examination in the outpatient clinic, there was minimal tenderness over the deltoid muscle. The skin showed no swelling, erythema, or warmth. She had a full painless range of motion of the shoulder and elbow joints. There was no evidence of lymphadenopathy. Neurological examination of the cervical spine and upper limb was normal. Laboratory results did not reveal any obvious abnormality.Conventional plain radiographs demonstrated a well-defined osteolytic lesion in the left glenoid with a thin sclerotic rim. Some trabeculation could be noticed. Subsequent MRI with gadolinium contrast of the left shoulder revealed a lobulated and expansile bone tumor in the inferior 2/3 of the glenoid measuring 26 × 24 × 17 mm. The posterior glenoid cortex had important thinning with a focal breach. The tumor was isointense to skeletal muscle on T1-weighted images (T1WI) and more heterogeneous hyperintense with some intermediate and hypointense signals on T2-weighted images (T2WI). There was heterogeneous contrast enhancement corresponding to the hyperintense zones in T2WI. There was no perilesional edema .® needle  and sent for histopathological analysis and cytogenetic examination. This showed benign features with spindle cells and immunohistochemical staining for S100 protein was diffusely positive . Physiotherapy was started the day after the operation and a sling was given for comfort.The patient was discussed at the multidisciplinary bone and soft tissue tumor meeting and it was thought that the appearances of this lesion on the plain X-rays and MRI scan are very non-specific. It was therefore advised to obtain ample tissue through an open biopsy for the histopathological assessment of the lesion. A posterior approach was used in the lateral decubitus, with a 5 cm transverse incision along the lateral part of the scapular spine. The deltoid was detached from the exposed part of the scapular spine and distally retracted. An intramuscular approach through the infraspinatus muscle into the glenoid lesion was used. The suprascapular nerve was identified and retracted. There was no intra-articular involvement and as such the capsule was left intact. Samples were taken using a Jamshidipositive . The diaThe patient gradually regained mobility and pain subsided. No recurrence was seen 12 months after surgery .The first histologic description of a neurilemmoma, later called a schwannoma, was made by Verocay in 1908 and elaborated upon by Stout in 1935 . In the The discussion arises about the origin of these intraosseous schwannomas. First of all, one of the possibilities is that they are located along a sensory nerve in an intraosseous canal. This is an explanation of why these tumors frequently occur in the mandible, the maxilla, and the sacrum. Bones like these have many nerves traversing in canals or foramina. In a long bone, schwannomas can occur in a nutrient canal . This reSecondly, an intraosseous schwannoma can originate in the medullary cavity from an intraosseous sensory nerve. Schwannomas show a predilection for myelinated, sensory nerves and most intraosseous nerves are non-myelinated and participate in vasomotor functions. Intraosseous sensory nerves are scarce which can be the explanation why intraosseous schwannomas are very rare. Intraosseous sensory nerves access the medullary cavity in a nutrient canal, creating an intramedullary tumor with characteristics of a true primary bone tumor. This is a reasonable hypothesis for intraosseous tumors involving the long bones. To support this theory, an association was found between the position of an intraosseous schwannoma and the position of nutrient vessels in the distal humerus, proximal ulna, distal radius, and the fibula as examined by Suzuki et al. . ConcernFinally, an outside-in tumor growth pattern has also been suggested in which schwannomas destruct the bone from the outside. They originate from periosteal sensory nerves and give periosteal reaction , 11. It Histologic features, in this case, were consistent with findings in the literature. Intraosseous schwannomas are microscopically comparable to soft tissue schwannomas with alternating cellularity of Antoni type A and B areas. The cellular area of this lesion may be confused with sarcoma, but no other malignant features such as atypia, mitotic figures, or necrosis are seen in schwannoma . ImmunohSchwannomas are slowly growing lesions. Some patients have some vague pain for several years. There can be some local muscle tenderness. X-rays show benign osteolytic lesion with sclerotic borders, trabeculation, cortical thinning or breach with no central calcification , 11. Theen bloc open excision for larger lesions [Intraosseous schwannoma can successfully be treated by intralesional curettage and filling of the tumor cavity with either bone graft, PMMA, or bone graft substitute, or  lesions . We have lesions , 4, 14.  lesions .Intraosseous schwannomas are rare tumors with benign features. They grow slowly and cause local tenderness. Careful radiographic interpretation is necessary and diagnosis is made after open biopsy and examination by the pathologist. They grow within canals and foramina, appear in the medullary cavity originating from an intraosseous sensory nerve, or grow from outside in. The common treatment is curettage and bone grafting. The patient can be reassured that no malignant transformation has ever been seen in intraosseous schwannomas. Recurrence is very rare and can be due to incomplete resection.No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article.


Gallbladder tuberculosis (GT) is an extremely rare disease entity, even in our country Morocco known for being an endemic area. The lack of pathognomonic clinical presentation and radiological features of GT makes preoperative diagnosis unlikely and poses a diagnostic dilemma regarding gallbladder carcinoma (GC). The diagnosis is usually made by histological examination after cholecystectomy, highlighting the importance of sending every gallbladder specimen to pathology. We report an exceedingly rare case of GT mimicking GC and refer to a recent review of the literature to discuss the clinical and radiological features of GT. Gallbladder tuberculosis (GT) is an extremely rare clinical entity that represents only 1% of abdominal tuberculosis cases . The diaA 71-year-old male patient, a former miner, was diagnosed with silicosis 30 years ago. The patient presented with complaints of vague, dull pain in the right upper quadrant with a significant loss of weight and appetite. There was no history of tuberculosis. Physical examination found a right hypochondrial tenderness. Laboratory investigations and chest X-rays were normal. Ultrasonography showed a gallbladder with a large gallstone and an irregularly thickened wall with dilatation of the intrahepatic bile ducts (IHBD) and the common bile duct (CBD)  and IHBD, making GC the most likely diagnosis Figure .Surgical exploration of the presumptive diagnosis of GC was done. Intraoperatively, we found a hard gallbladder with a thickened wall and contiguous infiltration into segment 4b of the liver. The patient underwent radical cholecystectomy. The histopathological examination revealed an ulcerated mucosa surrounded by epithelioid cells mixed with rare giant Langhans cells without caseous necrosis Figure ,B. The diagnosis of GT was established in front of a bundle of clinical, radiological, and histological arguments.On postoperative day nine, the patient became febrile and complained of abdominal pain and vomiting. An abdominal examination revealed diffuse rebound tenderness. Bile was noted in the drain. An urgent abdominal CT objectified fluid collections in the perihepatic space and at the cul-de-sac of Douglas, suggesting biliary peritonitis. The patient benefited from lavage and drainage of the peritoneal cavity and had a satisfactory recovery before being discharged from the hospital on day 15.A complete course of anti-tubercular treatment was administered for six months . The patient remained asymptomatic after a six-month follow-up.Isolated GT is an extremely rare clinical entity that was originally described for the first time by Gaucher in 1870 . Only arThe rarity of tuberculosis in the gallbladder is possibly due to the hypovascularity of the gallbladder sac and the alkaline nature of gallbladder bile, which has an inhibitory effect on Mycobacterium .The clinical presentation of GT is varied and nonspecific, making the preoperative diagnosis of GT difficult; the most frequent clinical signs and symptoms are abdominal pain, loss of weight, and loss of appetite, according to the series of GT published in the literature, as seen in our case Table .Only a few papers on the radiological findings of GT are found in the literature. Xu et al.  revealedAccording to Govindasamy et al., the MRI morphology features of GT were identical to GC . To summThe diagnosis is usually made upon histological examination after cholecystectomy  highlighThe treatment of GT is based on anti-tubercular chemotherapy based on a two-month attack treatment plan involving isoniazid (5 mg/kg), rifampicin (10 mg/kg), pyrazinamide (25-30 mg/kg), and ethambutol (20 mg/kg), followed by maintenance therapy for four months of isoniazid and rifampicin .There is no pathognomonic presentation of GT, and it is usually misdiagnosed as GC. As the preoperative diagnosis is difficult, all resected cholecystectomy specimens should be sent for histopathological examination for evidence of tuberculosis. A well-managed anti-tuberculosis treatment allows healing.


Transient global amnesia (TGA) is a disorder characterised by a temporary, reversible disruption of short-term memory. While the diagnosis of TGA is based on its clinical features, neuroimaging is important to exclude other sinister causes of global amnesia. Furthermore, classical MRI changes in TGA have been well described in the literature. These consist of unilateral or bilateral punctuate areas of hyperintensity in the hippocampal cornu ammonis 1 (CA1) region on diffusion-weighted imaging.We describe a case of a 61-year-old gentleman, presenting with symptoms of transient memory loss and confusion. A stroke was initially suspected in view of his multiple risk factors. Timely MRI demonstrated the typical findings associated with TGA. Recognition of these imaging features is of the utmost importance for radiologists in order to allow for an accurate diagnosis and differentiation from ischaemic pathology. A 61-year-old gentleman presented to the Mater Dei Hospital emergency department following a short episode of transient memory loss. According to his wife, he went to work as usual that morning, however on returning home in the afternoon he was forgetting a number of recent events and was asking a number of questions repeatedly. There were no features of retrograde amnesia, with the patient still being able to recall his identity, recognise his family members and carry out usual functions. This lasted for about 30 min, after which his memory slowly returned; however, he could not remember what had happened during this episode. He denied any associated headache, weakness, speech problems or sensory disturbance. There was no witnessed loss of consciousness or seizure activity. The patient also denied any recent stressful event or head injury and this was the first such episode of memory impairment.His medical history was notable for hypertension, diabetes and a 20-pack-year history of smoking. There was no previous history of transient ischaemic attacks, strokes or migraine. On examination, he was fully alert, oriented and cognitively intact. Parameters including blood pressure were normal and full neurological examination was unremarkable.Blood investigations were within normal limits and a CT brain showed no abnormalities. An electrocardiogram showed normal sinus rhythm. In view of his risk factors, the working diagnosis was that of an ischaemic stroke and he was admitted for further investigation and started on antiplatelet therapy. Echocardiogram and carotid Doppler showed no abnormalities. MRI of the brain using standard stroke protocol , axial diffusion-weighted imaging (DWI) and corresponding apparent diffusion coefficient (ADC) map, time-of-flight MR angiography, susceptibility-weighted imaging and axial isotropic T1-weighted sequences) was performed approximately 26 hours from onset of symptoms. This revealed normal gray-white matter differentiation, with no mass lesions and normal flow voids. Note was made of punctate hyperintensities in the CA1 region of the hippocampi bilaterally on DWI .The case was discussed at the neurology multidisciplinary team meeting, where it was agreed that the clinical and MRI findings supported the diagnosis of transient global amnesia (TGA). The patient remained well and was discharged with no driving restrictions or need for imaging follow-up.1Transient global amnesia is a clinical disorder characterised by sudden onset anterograde amnesia with temporal disorientation and iterative questioning due to inability to form new memories. Long-term memory, language, visual-spatial orientation and executive functions are typically preserved, with no other associated focal neurological deficit. Episodes characteristically resolve completely within 24 hours, with return to baseline cognition. Retrograde amnesia is variably present.2This syndrome usually occurs between the fifth and seventh decade of life and there is no significant gender predilection. The reported incidence is of 5–10 per 100 000 population. Cited precipitating factors include emotional stress, physical exertion, pain and the Valsalva manoeuvre.3 focal ischaemia, migraine as well as epilepsy. None of these however clearly explain all the condition's characteristics.4While this is a well-described disorder, the exact aetiology of this condition remains obscure. Various theories have been postulated, including venous congestion ,5 MRI is nonetheless recommended to exclude stroke or structural pathology, where there is diagnostic uncertainty. While imaging in such cases is usually normal, it is now well-known that TGA may be accompanied by characteristic MRI changes involving the hippocampus. Structurally, the cornu ammonis or hippocampus proper is divided into four distinct zones, based upon their sensitivity to hypoxia and histological differences. CA1 (Sommer’s sector) is known as the vulnerable zone, CA2 and CA3 as the resistant sector and CA4 as the medium vulnerability sector.6 Classic DWI findings in TGA consist of unilateral or bilateral small punctuate hyper-intense lesions in the CA1 region of the hippocampal cornu ammonis. These changes were first described in 1998 by Strupp et al.7 Since that time, various studies have been carried out and shown that certain factors may improve the visibility of these findings on MRI. These include high-resolution DWI, higher B-values, thin slice thickness (2–3 mm) and a delay of 48–72 h between symptom onset and scanning.8,9 In fact in cases where the initial MRI performed within 24 h is normal, however clinical suspicion for TGA remains high, repeat imaging is recommended.10The diagnosis of TGA is based on its clinical features. Criteria, such as those established by Hodges and Warlow, serve as guidance.11 Interestingly, the fact that DWI changes in TGA do not appear in the hyperacute phase, unlike the restricted diffusion seen in ischaemia, further supports the theory that these imaging findings are due to an entirely different pathological process. These lesions have been noted to resolve on follow-up MRI performed after 6–12 months.12The localisation of this pathological process to the CA1 area on imaging may give an indication to its causation. It is understood that neurons in this region of the hippocampus are particularly susceptible to metabolic stress, suggesting that TGA may result from a physiological temporary inhibition of memory formation.13Differential diagnoses for abnormal signal intensity in the hippocampi include posterior circulation ischaemia, limbic encephalitis and protracted seizures. These conditions, besides being readily distinguishable clinically, can also be distinguished radiologically to a certain degree. While in TGA, the signal hyperintensities seen on DWI are punctate and located laterally within the hippocampi, in posterior circulation infarcts the hippocampal lesions are larger in size with associated additional areas of restricted diffusion seen in the posterior cerebral artery territory, such as the occipital lobe, thalamus and corpus callosum. In protracted seizures, besides hippocampal signal abnormalities, additional changes may occur in the pulvinar and cortex. Furthermore, limbic encephalitis involves other limbic structures besides the hippocampus, such as the parahippocampal gyrus, cingulate gyrus and insula.14 Of note some studies have raised the possibility of an association between recurrent TGA and primary progressive aphasia, a rare neurological disorder characterised by progressive language impairment.15No treatment or driving restriction is required and the condition does not usually recur in the majority of cases. Outpatient follow-up with additional imaging if clinically indicated is the mainstay of management. Several studies have shown that there is no difference in prognosis or cognitive function in patients with and without DWI changes. This also applies to comparison of patients with TGA with age-matched controls.In conclusion, the recognition of imaging findings in transient global amnesia is essential for radiologists in order to avoid such patients being misdiagnosed with ischaemia or other pathology, especially in cases where the clinical picture is atypical.While the diagnosis of transient global amnesia is based on its clinical features, neuroimaging is important to exclude other sinister causes of global amnesia.Classic diffusion-weighted imaging findings in TGA consist of unilateral or bilateral small punctate hyper-intense lesions in the CA1 region of the hippocampal cornu ammonis.Recognition of this sign is of the utmost importance for radiologists in order to allow for an accurate diagnosis and differentiation from ischaemic pathology.


Trousseau’s syndrome is a cancer-associated thrombosis. Trousseau’s syndrome with cholangiocarcinoma is a rare condition with poor prognosis.A 59-year-old female was admitted to our hospital with abdominal pain, headache, and nausea. Abdominal enhanced computed tomography revealed liver tumor, splenic infarction, and bilateral renal infarction. Multiple acute cerebral infarctions were also detected by magnetic resonance imaging. Her preoperative serum levels of carbohydrate antigen 19-9 (CA19-9) and carcinoembryonic antigen (CEA) were > 120,000 U/mL and 589.6 ng/mL, respectively, which were extremely high. Histopathology after right hepatectomy revealed moderately differentiated adenocarcinoma consistent with intrahepatic cholangiocarcinoma. Her serum levels of CA19-9 were trending down to 9029.2 and 2659.8 U/mL at 1 and 3 weeks after surgery, respectively. However, at 7 weeks after surgery, her CA19-9 levels increased in the presence of positive imaging findings in the remnant liver, hilar lymph nodes, and peritoneal cavity. The initiation of combination chemotherapy including gemcitabine and cisplatin had a significant effect. The patient was doing well at 6 months after the surgery.This rare case of Trousseau’s syndrome due to cholangiocarcinoma suggests that extremely high CA19-9 levels might be a pathogenic factor of this syndrome. Armand Trousseau was the first to describe the association of thrombotic episodes with an underlying malignancy –5. Recen4/μL; D-dimer, 36.6 μg/mL; prothrombin time-international normalized ratio, 1.16; and activated partial thromboplastin time, 28.1 s. Ultrasonography of the carotid arteries and lower limbs, transthoracic echocardiography, and Holter electrocardiogram did not reveal the source of embolism. Therefore, the patient was diagnosed with Trousseau’s syndrome due to intrahepatic cholangiocarcinoma.A 59-year-old female visited an emergency room with the chief complaints of abdominal pain and dizziness and was diagnosed with gastroenteritis and vertigo. Three days later, she was readmitted to the emergency room because of worsening abdominal pain and difficulty in movement due to headache and nausea. Plain whole-body computed tomography (CT) showed suspicious cerebral infarction and a low-density mass lesion in right liver lobe. Subsequent head magnetic resonance imaging (MRI) confirmed infarcted areas in multiple blood vessels defined as an embolic shower. Contrast-enhanced CT revealed about 9 × 6 × 4 cm-sized and ill-defined mass with several daughter nodules in the posterior segment of the right hepatic lobe, splenic infarction, and bilateral renal infarctions Fig. . The livContinuous intravenous infusion of unfractionated heparin at a dose of 10,000 U per day was started immediately. However, she developed left hemiplegia 1 week later, and head MRI revealed thrombosis of the right middle cerebral artery. Activated partial thromboplastin time value was 23.4 s at that time in spite of continuous administration of heparin. She was transferred to a hospital where endovascular treatment was possible, and thrombectomy was performed. Although left paralysis remained after thrombectomy, she returned to our hospital for the treatment of intrahepatic cholangiocarcinoma. In the meantime, she was treated with continuous infusion of unfractionated heparin at a dose of 15,000 U per day. After the thrombectomy, activated partial thromboplastin time value was kept nearly 40.0 s with administration of heparin.Preoperative examination did not show any issues with cardiopulmonary function. Abdominal enhanced CT and MRI showed a tumor in the posterior segment of the right hepatic lobe Fig. . HepaticDuring the surgery, anticoagulation was performed with subcutaneous injection of low-molecular-weight heparin at a dose of 5000 U per 12 h. In surgery, the tumor was exposed on the liver surface and partially adhered to the diaphragm and retroperitoneum. Right hepatectomy was performed with combined resection of the invading tumor.The resected tumor was 9 × 6 × 6 cm in size, and the pathological diagnosis was moderately differentiated intrahepatic cholangiocarcinoma  , CEA , cell surface-associated mucin 1 recognized by the cancer antigen 15-3 (CA15-3) epitope , and CA19-9 . As seen in Fig. 2 body surface area) followed by gemcitabine (1000 mg/m2 body surface area) [After the surgery, the patient’s general condition was good and no neurological changes were observed. The perioperative changes in serum tumor maker levels are shown in Fig. ce area) , both adContinuous intravenous heparin administration was changed to subcutaneous injection of low-molecular-weight heparin at a dose of 5000 U per 12 h, and she was discharged 12 weeks after the surgery. She has been subsequently followed in our outpatient clinic without further thrombosis during the last 6 months after surgery, and there has been no tumor progression.Tumors associated with Trousseau’s syndrome are usually mucin-producing adenocarcinomas derived from visceral organs. However, few studies have reported an association between cholangiocarcinoma and thromboembolism , 7, 8. TBecause most patients of Trousseau’s syndrome and intrahepatic cholangiocarcinoma are at an advanced stage by the time of diagnosis, it is difficult for them to undergo curative treatment , 3, 7, 9In the present case, the serum levels of CA19-9 were above the detectable range until 4 days after the surgery. The reported half-life of CA19-9 immediately after surgery is 0.5 ± 0.1 days . TherefoHeparin is widely preferred over oral anticoagulants to prevent thrombotic events in patients with Trousseau’s syndrome . In the In conclusion, we herein reported the rare case of a patient with cholangiocarcinoma who presented with Trousseau’s syndrome and successfully underwent right hepatectomy followed by systemic chemotherapy. As illustrated in the present case, an extremely high level of CA19-9 might trigger Trousseau’s syndrome in patients with cholangiocarcinoma.


Atraumatic renal pelvis rupture without pre-existing renal or ureteric pathology is an uncommon event. It is reported in the setting of acute urinary tract obstruction, most often secondary to ureteric calculi. Typical symptoms include acute flank pain and nausea, mimicking pyelonephritis or other causes of acute abdomen. Spontaneous rupture occurring bilaterally without identifiable urinary tract obstruction is exceedingly rare, and has yet to be reported in current English literature. Possible contributing pathophysiological mechanisms can be postulated from reported cases of rupture with observed obstruction.A 58-year-old woman undergoing multiphasic computed tomography (CT) for evaluation of asymptomatic microscopic haematuria developed on-table bilateral renal pelvis rupture seen only after contrast administration, on the delayed phase. There was no significant past medical history of note. The patient remained asymptomatic throughout and after the study, and was managed conservatively. Follow-up radiographical imaging over a month showed resolution of urinoma and no further contrast extravasation. No complications or recurrence was subsequently noted.Spontaneous rupture of the renal pelvis can be a rare complication of intravenous contrast administration even in cases without identifiable urinary tract obstruction, and it can occur bilaterally. Cases can uncommonly be asymptomatic but typical symptoms should prompt evaluation of the kidneys, particularly when they are not included in the initial study or no delayed phase is protocolled. Interval imaging for resolution of urinoma and contrast extravasation is clinically relevant to monitor for and avoid infective sequelae. Spontaneous renal pelvis rupture, also known as forniceal rupture or pyelosinus backflow, is an uncommon complication of acute urinary tract obstruction. In the context of intravenous urography during acute renal colic, Schwartz et al. defined a rupture as ‘spontaneous’ if it occurred in the absence of trauma, abdominal compression, destructive renal lesion or prior surgical and endoscopic intervention .It is thought to occur as a result of rapidly increasing intraluminal pressure  presumabAsymptomatic spontaneous rupture occurring bilaterally in the absence of urinary tract obstruction is hence extremely unusual. To the best of the authors’ knowledge, this has not been described in current English literature. We report one such case precipitated by CT urography and discuss possible pathophysiological mechanisms.A 58-year-old woman with no significant past medical history was referred to the outpatient urology clinic for asymptomatic microscopic haematuria noted incidentally on urinalysis. Routine CT urography was performed in the pre-contrast, nephrographic and delayed phases. A total of 75 mL of iodinated contrast agent (300 mg I/mL concentration) was administered, and no diuretics were given.On the pre-contrast and nephrographic phases, a 2 mm non-obstructive renal calculus was noted on the right but the kidneys were otherwise unremarkable. No calculus was seen in the ureters and urinary bladder Fig. . The latOn the excretory phase performed at 6 min 13 s post-contrast, peripelvic contrast extravasation was observed bilaterally - suggestive of acute rupture Fig. . No otheThroughout the study, no flank pain or other symptom was reported; the patient remained asymptomatic even after the event was observed acutely on-table. No abdominal or flank tenderness was elicited on examination.She was managed conservatively and a radiograph performed the next day revealed no persistent contrast pooling. Follow-up intravenous urography performed a month after showed no recurrence of contrast extravasation Fig. . ThereafThis is a case demonstrating an exceedingly rare radiological event in the absence of classically associated risk factors. Reviewing related case reports, we discuss possible pathophysiological mechanisms though these are difficult to prove with a single case alone.Rupture is reported to occur when intra-pelvic pressures exceed 25 to 75 mmHg and is seen most frequently at the fornices [However, osmotic diuresis alone is an insufficient factor in the absence of obstruction. The primary mechanism is nonetheless likely dependent on this diuretic effect, given the temporal relationship between contrast administration and onset.Unilateral renal pelvis rupture in the absence of obstruction has been described in the emergency care setting. Patients present first with flank pain, with a following CT demonstrating rupture where no obstruction is identified. Eken et al. suggest that unwitnessed transient obstruction, such as from a passed calculus, may be the most plausible mechanism . Though The bilateral nature of rupture in this case makes transient obstruction by a passing ureteric calculus less probable. Moreover, a calculus was only demonstrated on one side prior to contrast administration. Transient obstruction more distally can affect both kidneys and remains a possible mechanism.One other report of bilateral renal pelvis rupture occurring during contrast-enhanced CT was described by Chien et al. . The casNotably, the case we report did not manifest flank pain. There was also no known history or symptom of urinary obstruction, though the bladder did appear distended on CT. It is plausible that voluntary urinary retention acted as a form of transient ‘obstruction’ at the bladder outlet.Even so, majority of patients with a filled bladder do not develop this complication. The initial presentation of microscopic haematuria in this specific case may hence be relevant. Urinary calculi predispose to urinary tract rupture, with one postulated mechanism of urothelial injury during passage . As no uIn the case reported by Chien et al., the patient was also treated conservatively. Interval imaging and urinalysis showed resolution of both microscopic haematuria and contrast extravasation. Serial imaging is of value, as large or persistent urinomas carry a risk of infection and loss of renal function . Other mIn conclusion, spontaneous rupture of the renal pelvis is a potential  complication of intravenous contrast administration in the absence of urinary tract obstruction, and it can occur bilaterally. Although cases can uncommonly be asymptomatic, typical symptoms such as flank pain following contrast injection behoove physicians to evaluate the kidneys – particularly when they are not included in the initial study or no delayed phase is protocolled. A missed or untreated complication (i.e. large urinoma), could become a nidus for intra-abdominal infection and damage the urinary tract by fibrosis and granuloma formation. Rupture should also prompt physicians to actively evaluate for underlying causes. Interval follow-up imaging for resolution is clinically relevant to monitor for and avoid such infective sequelae through radiological-guided drainage or urological intervention.


Primary renal extra-skeletal Ewing sarcoma is a rare neoplasm, often metastatic at diagnosis, and with a poor outcome. A multimodal approach is often the treatment of choice in this aggressive neoplasm. We present a case of primary renal extra-skeletal sarcoma in a 45-year-old woman who underwent tumor resection without clear margins. After no response to the first cycle of chemotherapy, we documented an early onset of local recurrence. The patient refused any other treatment and died four months after surgery. The ES family is a group of poorly differentiated tumors made up of small round blue cells and it recognizes the rearrangement of the ESWR1 gene as a pathognomonic sign [ES is the second most common pediatric bone tumor, with a peak of incidence between the first and second decade and 80% of cases arise from the skeleton . In adulHerein, we report a case of an adult woman affected by an EES treated with surgery and chemotherapy. Secondary aim was to perform a review of the literature about EES and its therapeutic management.2A 45-year-old female with persistent chest pain underwent chest-abdomen CT scan for suspicion of pulmonary thromboembolism. The imaging revealed a heterogeneous retroperitoneal mass, sized as 17 cm in diameter, extending from the superior left renal pole and infiltrating posterior abdominal wall and partially the diaphragm . The tumThe patient underwent a laparotomic left radical nephrectomy with adrenalectomy and partial resection of psoas muscle and diaphragm, which were macroscopically involved by tumor, in order to achieve a radical surgery. Hilar and para-aortic lymphadenectomy were also carried out. Operative time was 200 min. Estimated blood loss was 550 cc. There were not any perioperative complications. No blood transfusion was needed. The patient was dismissed on post-operatory day 13.Macroscopic examination showed a 200 × 150 × 90 mm grey-yellow, friable mass with capsular interruption and containing necrotic and hemorrhagic areas. Surgical margins were unclear at level of psoas and diaphragm resection. Histologically, the tumor was made up of nodules separated by sclero-hyaline septa and it was composed of small to medium-sized cells with oval nuclei, small nucleoli, granular chromatin, and scanty cytoplasm. The mitotic activity was high. On immunohistochemistry, the neoplastic cell resulted to be diffusely positive for CD99, focally positive for CD56, and focally and weakly positive for synaptophysin. The neoplastic cells were negative for cytokeratin (CKAE1/AE3), epithelial membrane antigen (EMA), S-100, leucocyte common antigen (LCA), CD2, CD20, CD79 alpha, CD117, LAT, CD10, MPO, TdT, CD68PGM1, NPM1, CD38, PAX5, CD31, CD34, and desmin. The proliferative index (KI67/MIB-1) was about 70%. The genetic analysis revealed the presence of EWSR1 rearrangement. The immuno-morphological analysis highlighted a malignant aggressive neoplasm with EWSR1 rearrangement, in keeping with EES. Lymph nodes were free of tumor. Four weeks after surgery, the patient underwent ESFT 2001 chemotherapy protocol, including 5 cycles of injectable vincristine (1.6 mg), ifosfamide (2.2 g), mesna (440 mg), and etoposide (110 mg). The protocol, the most suitable in this case, comprehensive of adriamycin in addition to vincristine et ifosfamide, was not administered due to the poor heart ejection fraction.After the first cycle of chemotherapy, the patient required emergency care for dyspnea. An X-ray exam showed left pleural effusion and a chest drainage was placed. A CT scan disclosed a huge recurrence in the surgical site and a peritoneal carcinomatosis as shown in Informed consent: Written informed consent was obtained from the patient for publication of relevant medical information and all of accompanying images within the manuscript.3ES was first described by Stout in 1918 and later on in 1921 by James Ewing who characterized this tumor describing it in the diaphysis of long bones. ES in children and adolescents is defined as a bone tumor, which may occur at any site within the skeleton, but preferentially affects the trunk and the diaphysis of long bones . Howevert  chromosomal translocation, which fuses EWSR1 to the FLI1 ETS family gene to generate EWSR1-FLI1 fusion transcripts which induce mitotic defects leading to genomic instability and subsequent malignant transformation [About 85–90% of EES cases present a somatic reciprocal ormation ,8,9.A first challenge for this kind of lesion is the diagnosis. Clinical symptoms are not characteristic when present.As in other pathologic conditions, radiological findings are usually not typical and present a wide spectrum of imaging features and metastatic patterns. The imaging features of primary EES on contrast-enhanced CT or magnetic resonance imaging (MRI) are bulky heterogeneous masses with frequent local invasion or with compression of adjacent organs. Retroperitoneal masses are usually large at diagnosis with 50% of lesions bigger than 20 cm . Most caIn recent literature, more and more cases of extra-skeletal sites in adults are reported. Probably, this increase is related to the availability of molecular diagnostic techniques and not to a true increase in its incidence .ES of the kidney is a rare tumor in adults and it was described for the first time in 1975 by Seemayer and colleagues. Some authors suggested that the origin of ES in the kidney could be neural cells invaginated into the kidney during their development or embryonic neural crest cells migrating into the kidney and undergoing tumorigenesis . The occAt diagnosis, the most common symptom is pain (in 63%), followed by hematuria in 41% and a palpable mass in 25% . In our Regarding renal ES , 57% of In our case, the huge mass showed imaging features of malignant tumor, and for this reason, we performed a surgical treatment without biopsy. Due to bulky size and wide blood supply of the tumor, a preoperative renal angio-embolization was carried out in order to reduce the risk of intraoperative bleeding and to facilitate surgical procedure, thus decreasing operative time and perioperative morbidity. Other important benefits of preoperative embolization included the potential role of an early ligation of the renal vein before the renal artery has been fully controlled, according to the indications given by Robson. In literature, the real usefulness of preoperative renal embolization is still debated. However, a recent prospective, randomized study showed preoperative renal embolization to be a safe and well-tolerated procedure that allowed to reduce median blood loss in patients with huge kidney cancer who underwent embolization before nephrectomy compared to patients who did not undergo preoperative embolization .Prognosis is poor in metastatic disease ,27,28,29Multimodal approach including surgery associated to the adjuvant chemotherapy is the standard therapeutic approach, with radiotherapy (RT) playing an optional role in localized and nonsurgical tumors. However, 32.5% of patients received only surgical treatment, while 13% received only chemotherapy. In the 87% of the patients who received surgical treatment, a nephrectomy was performed. About 5.5% of surgical patients received neoadjuvant chemotherapy, 47% adjuvant chemotherapy, and 15% adjuvant chemotherapy and radiotherapy. The mean overall survival (OS) in the group of patients treated by multimodal approach was 20.8 months, whereas that in the patients who received only surgery was 10.3 months ,30,31,32The key point in the management of these tumors is to obtain a complete surgical debridement with clear negative margins, but this is affected by the disease stage at diagnosis ,36. In o4Reporting this case, we would point out that in presence of a renal mass, especially if huge, it has to be borne in mind that EES can occur primarily in the kidney. The local invasion should be well-evaluated before the surgery because, in case of EES, only a complete surgical ablation of the tumor could improve cancer-specific survival.In adult, advanced ES is a dramatic condition with inauspicious outcome. Often, successful rate of surgical treatment may be affected by complexity to obtain negative surgical margins due to disease extension. Due to this, surgery in advanced disease may be considered as an important step of multimodal treatment.


Hepatic hemangiomas are the most common benign hepatic tumor. Current guidelines recommend surveillance imaging and reserving surgical intervention for symptomatic patients with giant liver hemangiomas (>5 cm). We present the case of a patient with a rapidly enlarging giant hepatic hemangioma initially managed by surveillance. During her observation period, she developed weight loss, constipation, and pancytopenia concerning for Kasabach-Merritt Syndrome. Resection of the hemangioma was complicated by its large size (28.0 × 18.0 × 11.4 cm). Patients with rapidly growing giant liver hemangiomas, even when asymptomatic, should be promptly referred to specialized surgical centers for evaluation and management. Liver hemangiomas are the most common, benign mesenchymal hepatic lesions occurring in up to 20% of the population. Seldom, giant hemangiomas may cause abdominal pain, congestive heart failure, or even Kasabach-Merritt Syndrome (KMS), a rare life-threatening consumptive coagulopathy. We present the case of a young patient with a rapidly enlarging giant hepatic hemangioma that leads to multiple medical comorbidities. We emphasize the need for recognizing early signs to abandon standard surveillance therapy in patients with rapidly enlarging giant liver hemangiomas and urge for timely referral to specialized hepatobiliary surgical units and surgical resection.A 37-year-old female with a known history of giant hepatic hemangioma was referred with one-year history of increasing abdominal pain associated with progressive nausea, 60 lbs weight loss and constipation. The hemangioma was initially diagnosed during a workup for lower back pain two years before the onset of abdominal pain. General surgical consultations were sought, and expectant management was advised. The patient had subsequently been treated conservatively for lumbar disk herniation and newly diagnosed irritable bowel syndrome. In our clinic, she appeared pale and malnourished with lumbar lordosis. The abdominal exam revealed generalized distention with a palpable liver edge below the right subcostal margin.Magnetic resonance imaging (MRI) of the abdomen revealed 18.5 (AP) × 19.2 (TV) × 25.3 (SI) cm mass located in the right posterior hepatic lobe consistent with giant hemangioma . A revieConsent for surgical resection was obtained. A surgical set-up has been described earlier.  A midlinThe patient was discharged home on postsurgical day five. At her six-month follow-up, she had resolution of her abdominal pain and nausea and reported improved bowel function. Her weight loss stabilized. She continued to have mild lower back pain from her lumbar disk herniation. There was interval improvement of her pancytopenia . ComputeHemangiomas are the most common benign hepatic lesion and occur in up to 20% of the population. The classification of hemangiomas based on size varies widely, with giant hemangiomas being described as >5 or even >10 cm –5. The lCurrent guidelines advocate for the conservative management of asymptomatic giant liver hemangiomas via serial imaging to monitor growth , 7. ReseWhen her hemangioma was initially diagnosed, our patient did not have typical indications for resection such as abdominal pain or extrinsic visceral compression. Over the next three years, surveillance MRIs demonstrated the hemangioma nearly doubling in diameter from 13 cm to 25 cm, with an approximate eight-time increase in absolute volume. During this observation phase, she developed typical compressive symptoms attributable to her hemangioma including nausea, constipation, 60 lbs (27 kg) weight loss, and abdominal pain and was beginning to exhibit early signs concerning for Kasabach-Merritt Syndrome. Surgical resection required large open procedure and, although without complication, was technically difficult given anatomic distortion from tumor size and inability to secure the retrohepatic inferior vena cava. This raises the question of whether surgical resection should be preemptively performed in similar patients who demonstrate persistent hemangioma growth.While hemangiomas may regress in size over time, more than half of all hemangiomas grow on follow-up imaging. In addition, a larger hemangioma size on initial diagnosis correlated with a higher subsequent rate of growth, with younger patients more likely to have the highest growth rate . Liu et As they grow in size, giant liver hemangiomas are associated with increasing perioperative risk. In a retrospective review, Wahab et al. found that patients undergoing resection for hemangiomas larger than 10 cm had significantly increased intraoperative blood loss with need for blood transfusions and longer operative times . Our unpWe conclude that progressively enlarging hepatic hemangiomas may benefit from surgical resection prior to presentation of typical symptoms. We propose that incidentally discovered giant hemangiomas should be referred to specialized surgical centers for continued follow-up. Early surgical resection (or enucleation) should be considered for giant hemangiomas before anatomic distortion of liver parenchyma or the onset of medical comorbidities make surgical intervention prohibitively risky.


Klinefelter syndrome is a rare chromosomal disorder with at least one extra X chromosome in males resulting in male hypogonadism, androgen deficiency and impaired spermatogenesis. It is associated with an increased risk of certain malignancies; including leukemia, breast cancer, non-Hodgkin's lymphoma and mediastinal germ cell tumors, however, testicular tumors are rare in men with Klinefelter syndrome. Testicular epidermoid cysts are rare benign tumors affecting the testes. We report a case of bilateral testicular epidermoid cysts in a 30-year-old man known to have Klinefelter syndrome. He had an incidental finding of bilateral hard irregular-surfaced testes during routine assessment for testosterone replacement therapy. Biochemical investigation confirmed primary hypogonadism and ultrasound imaging demonstrated bilateral solid testicular masses with no blood flow seen within the lesions. The patient went on to have a right-sided radical orchiectomy with left-side sparing. The histology revealed features in keeping with that of a testicular epidermoid cyst with no evidence of malignancy. The patient was commenced on testosterone replacement therapy. This case emphasizes the importance of routine physical examination of the male external and internal genitalia when considering testosterone replacement therapy. Klinefelter syndrome is a state of hypergonadotropic hypogonadism in male individuals due to having at least one extra X chromosome. The 47, XXY karyotype is the commonest human sex chromosome disorder having a prevalence of one in 500-600 males . KlinefeTesticular epidermoid cysts (TEC) are benign tumors and although the most common benign tumors of the testes, they account for only 1-2% of all testicular tumors. They usually measure 1-3 cm in size and affect men in their second to fourth decade of life ,6. Most Testicular tumors occurring in patients with Klinefelter syndrome are rare. Bilateral TEC occurring in patients with Klinefelter syndrome are even rarer. We present a case of bilateral TEC occurring in a young man with Klinefelter syndrome.Medical history and demographicsA 30-year-old man was referred to the endocrine outpatient clinic to initiate testosterone replacement in view of worsening symptoms of tiredness, lethargy and erectile dysfunction. He was diagnosed with Klinefelter syndrome during childhood but had not had any testosterone replacement for up to 10 years due to poor adherence to treatment. He had a medical history of mild asthma. He did not have cryptorchidism in childhood. He was a smoker and occasional drinker. He had not fathered a child before. He was not married but had a partner and both expressed their desire to have children in the future. He had no family history of testicular pathology.On physical examination, he had gynecomastia and sparse body hair over his arms and legs. However, he had normal pubic hair and normal penile size. His both testes were very hard with irregular surfaces: the right testes measuring 25 ml in size and the left testes measuring 8-10 ml in size. He weighed 83.6 kg with a height of 1.93 m (BMI 22.4).InvestigationsHis laboratory tests showed very low serum testosterone levels, elevated luteinizing hormone and follicle stimulating hormone levels, which were consistent with primary gonadal failure (hypergonadotropic hypogonadism). Testicular tumor markers such as alpha-fetoprotein and serum beta human chorionic gonadotropin (beta HCG) were normal. Thyroid function test, renal function test, liver function test, prostate specific antigen, vitamin D, calcium and prolactin levels were all within normal limits Table .An ultrasound scan of his testes demonstrated bilateral solid testicular masses with no blood flow seen within the lesions. There was very little identifiable normal testicular tissue seen within the right testis, which was replaced by a large mass measuring 3.4 cm x 2.7 cm x 2.3 cm. The left testis contained an echogenic, well defined mass measuring 0.9 cm x 0.65 cm x 0.5 cm  but normal in the hip . A computerized tomography (CT) scan of his chest, abdomen and pelvis revealed no abnormalities.A bone density scan revealed slightly low bone mineral density (BMD) in the spine  as opposed to enucleation because of the size of the lesion and the fact that malignant changes could not be confidently ruled out. After receiving the histology report a decision was made to spare the left testis and keep the left testicular mass under ultrasound surveillance. Sperm retrieval and cryopreservation was discussed with the patient. His post-op serum testosterone level had fallen to 2.7 nmol/L. Because the patient had not been on testosterone replacement for a long time and had become very symptomatic, he was not keen on waiting to see if he would get funding approval for sperm retrieval and cryopreservation for future assisted fertility treatment. He also understood that the chances of success would be low. The patient was then put on testosterone replacement therapy in the form of a topical gel (40-60 mg) to apply daily.Outcome and follow-up The patient remained well and was being followed up with regular ultrasound scanning of the spared left testis. The appearance of the left-sided epidermoid cyst remained unchanged over five years of ultrasound surveillance.TEC are benign tumors classified under "teratoma of prepubertal type". They are the most common benign tumors of the testes, but account for only 1-2% of all testicular tumors and commonly affect men in their second to fourth decade of life ,6. HowevThe biochemical and ultrasound findings are usually enough for a confident pre-operative diagnosis, such that testis-sparing surgery  can be performed, with concomitant use of intra-operative frozen section histologic analysis, to rule out occult malignancy. However, total orchiectomy must be done if in doubt . TesticuA large cohort study demonstrated that men with Klinefelter syndrome may be at increased risk for developing lung cancer, non-Hodgkin lymphoma, breast cancer and mediastinal germ cell tumors . The incidence of testicular cancer was not high and there were no cases of mortality from testicular cancer found in this study . The comUp until 2018 there had been only eight cases of TEC found in individuals with Klinefelter syndrome reported in the literature. Of these cases only one had bilateral involvement. Their ages ranged from six to 38 years and all cases underwent orchiectomy . ReasonsThe patient in our case report had a lesion that satisfied both the ultrasonic and histological criteria for the diagnosis of a TEC. However, as there was very little testicular tissue detectable and any malignant changes could not be ruled out with 100% confidence on ultrasound, an orchiectomy was deemed safer. The fact that the left testicular mass remains the same size on yearly ultrasound scanning over five years confirms it is a similar but smaller epidermoid cyst.The patient in this case report did not complain of any testicular mass or discomfort. The testicular masses were coincidental findings during examination of the male genitalia before testosterone replacement therapy. This patient had right-sided orchiectomy as opposed to enucleation because malignancy could not be confidently ruled out as in the previous case reports. The left-sided testicular mass was left alone after the right-sided mass was confirmed benign. Sparing the left side also helped the psychological well-being of the patient.We have presented a rare case of bilateral testicular epidermoid cysts occurring in a young man with Klinefelter syndrome. This case was found by chance during routine examination before initiation of testosterone replacement therapy. This case emphasizes the importance of routine physical examination of the male external and internal genitalia when considering testosterone replacement therapy. We hope that this case report not only adds to the existing literature but also contributes to gaining insight into this rare benign tumor of the testis.


Odontogenic keratocyst (OKC) is the third most common odontogenic cyst which arises from cell rests of dental lamina, and usually observed in the jaws. Because OKC is noted for its high rate of recurrence, there are various treatment strategies. Here, we present a rare case of OKC which occupied the entire maxillary sinus and pterygoid process of the sphenoid bone extending nearly to the skull base.The patient was a 21-year-old male and underwent surgical removal of the cyst using the Caldwell-Luc procedure which in this case extended the surgical approach to the pterygoid process of the sphenoid bone via the pterygomaxillary junction. However, we found a recurrent lesion in the posterior wall of the maxillary sinus 20 months after the surgery and subsequently performed a secondary cystectomy. Surgical specimens showed positive bcl-2 staining of OKC and negative cytokeratin-10 on immunohistochemistry for both primary and recurrent lesions.OKC rarely occurs in the maxillary sinus and extends to the deep maxillary structure and the skull base. In order to prevent recurrence, it is necessary to recognize the exact location of the entire lesion. Careful examination of preoperative CT images is needed to make a complete surgical planning and to perform a reliable surgical procedure. This lesion used to be classified as an odontogenic tumor by the World Health Organization but was reclassified as a cyst in 2017. Because OKC is noted for its high rate of recurrence, there are various treatment strategies for the lesion. Previous studies have reported that enucleation had a recurrence rate of 56%, whereas resection had a rate of 0% but with increased morbidity for the patient . Other tThe most frequently involved site of OKC is the mandibular bone occurring at a rate of around 73% compared with 27% for the maxillary bone . PreviouHere, we report on a rare case of OKC which entirely involved the maxillary sinus extending into the pterygoid process of the sphenoid bone nearly to the skull base. This work has been reported in line with the SCARE criteria .2A 21-year-old male patient was referred to our university hospital to examine his upper right third molar which was displaced into the maxillary sinus based on panoramic radiography. He had no symptoms and no previous medical history. Extraoral examination showed no evidence of facial swelling or asymmetry. On intraoral examination, no abnormal findings were observed. Panoramic radiography showed an ill-defined and radiopaque lesion in the right maxillary sinus involving the third molar that had been displaced to the roof of the sinus A. ComputThe patient underwent biopsy and histopathological examination diagnosed the specimen to be OKC. We performed enucleation of the cyst and removal of the displaced third molar using the Caldwell-Luc procedure which in this case extended the surgical approach to the right pterygoid process of the sphenoid bone via the pterygomaxillary junction A to 2C. 3In the study of odontogenic cysts patients, OKC is reportedly diagnosed at 19.1% . PreviouThere have been many arguments made regarding the best treatment for OKC, and it is still controversial because the most significant features of OKC is its potentially aggressive behavior, tendency to recur, and neoplastic potential compared with other jaw cysts. Treatment options for OKC include aggressive curettage, enucleation with or without Carnoy’s solution or 5-FU, marsupialization, and resection ,3. One oRegarding immunohistochemistry, the present case showed negative staining of cytokeratin-10 and positive of bcl-2. Previous reports have indicated that bcl-2 protein was consistently expressed in the basal cells of OKC but was not expressed in other odontogenic cysts or normal oral mucosa ,19. BecaThe current case had a recurrence in the posterior wall of the maxillary sinus 20 months after initial surgery. A systematic review of the literature has identified that enucleation with adjunctive therapy, such as Carnoy’s solution application or decompression before enucleation, had a recurrence rate of 1%–8.7%, whereas simple enucleation had a rate of 17%–56% . Also, p4In conclusion, OKC rarely occurs in the maxillary sinus and extends to the deep maxillary structure and the skull base. In order to prevent recurrence, it is necessary to recognize the exact location of the entire lesion. Careful follow-up is also needed to detect recurrence especially in the deep maxillary structure.This case report did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Ethical approval is exempted as this is a case report.Written informed consent was obtained from the patient for publication of this case report and accompanying images.Mitsuo Goto: case report design, writing the manuscript.Sei Ueda: data collection, manuscript preparation.Satoru Miyabe: followed up the patient, contributed to the pathology section.Satoshi Watanabe: followed up the patient, reviewed the manuscript.Yoshihiko Sugita: contributed to the pathology section, literature search.Toru Nagao: reviewed the manuscript, revised the manuscript.Not applicable.Name of the registry: Not applicable.Unique identifying number or registration ID: Not applicable.Hyperlink to your specific registration (must be publicly accessible and will be checked): Not applicable.Mitsuo Goto.Not commissioned, externally peer-reviewed.The authors declare no conflicts of interest.


Müllerianosis is a very rare neoplasm composed of two or three Müllerian derived tissues . We report the first case of concurrent müllerianosis of the urinary bladder and the umbilicus presenting with umbilical bleeding.A 43-year-old Asian premesopausal female, gravida 1, para 1, presented with intermittent umbilical bleeding. An umbilical nodule and a bladder tumor on the posterior wall of the urinary bladder were identified. She underwent transurethral resection of the bladder tumor and excision of the umbilical nodule successively. Diagnosis of müllerianosis was confirmed by the histological and immunological features. No tumor recurrence was noted at 6 months of follow-up.Müllerianosis is extremely rare and mainly reported in the urinary bladder, and generally affects women of reproductive age. Despite the common presentations of müllerianosis of the urinary bladder including irritative voiding symptoms, abdominal/pelvic pain and gross hematuria, our rare case had no symptom except umbilical bleeding. The possibility of concurrent bladder müllerianosis should be considered when müllerianosis is found at other location. We suggest a surgical intervention to establish the correct pathological diagnosis because it is essential to exclude malignant neoplasms of the urinary bladder. The majority of patients have a favorable prognosis. Müllerianosis is a very rare neoplasm composed of at least two of müllerian-derived tissues, namely endosalpinx, endometrium and endocervix,  and has 2). She had no other symptoms including abdominal/pelvic pain, gross hematuria, urinary frequency, micturition pain, dysmenorrhea or dyspareunia. On pelvic examination, she had no lifting tenderness. She visited our Obstetrics and Gynecology clinic, where a bladder lesion was incidentally identified on transabdominal ultrasound. A firm, irregular and unmovable nodule was also noted in the umbilicus with a diameter of 2 cm . Of the total 22 patients with müllerianosis of the urinary bladder, five were postmenopausal females. The most common symptoms have been reported to be irritative voiding symptoms (54.5%) including frequency, urgency, urge incontinence and dysuria, abdominal/pelvic pain (45.5%) and gross hematuria (36.4%). These symptoms occurred cyclically in six cases (27.3%), mostly during the menstrual period. One patient with a tumor on the ureterovesical junction presented with renal colic . Our casGrossly, müllerianosis of the urinary bladder has been described as “sessile polypoid”,  “submucoBoth medical and surgical treatments can be used for müllerianosis of the urinary bladder. Regardless of the lack of consensus on the choice of therapy, most physicians and patients chose the surgical intervention in the reported cases, and most of the patients had a favorable prognosis. Sixteen patients underwent TURBT, only five of whom required subsequent medical treatment, , 13 or pMüllerianosis of the urinary bladder mimics several bladder neoplasms such as cystitis glandularis and nephrogenic adenoma . It is mIn conclusion, müllerianosis is a rare neoplasm which mainly affects women of reproductive age. The common presentations of müllerianosis of the urinary bladder include irritative voiding symptoms, abdominal/pelvic pain and gross hematuria. In our case, multifocal müllerianosis were separately located on the urinary bladder and the umbilicus, which implies that the possibility of concurrent bladder müllerianosis should be considered when müllerianosis is found at other location. Despite a favorable prognosis in the majority of patients, in view of the importance of establishing the correct pathological diagnosis, we suggest treatment with a surgical intervention consisting of TURBT and partial nephrectomy if recurrence occurs with persistent symptoms.


Soft tissue tumors of Schwann cells in either the peripheral or cranial nerves are known as schwannomas or also as neurilemmomas. Limited data exists regarding psoas muscle schwannoma in the literature. Therefore, we have done an extensive literature review and found a total number of 46 cases of intra-psoas schwannoma including our case, which are summarized in this article along with reporting our new case.We report a very rare case of primary left intra-psoas muscle schwannoma in a 39-year-old Saudi gentleman, smoker with an unremarkable medical and surgical history. The patient presented to the emergency department with exacerbated chronic lower abdominal pain since almost 2 years. After conforming the diagnosis, the patient was admitted electively and underwent uneventful laparoscopic hand-assisted resection.Schwannomas are neurogenic tumors which known to be solitary, benign, well circumscribed, encapsulated, and slow-growing tumors. Multiple imaging modalities are used to detect such a tumor. Although there is no consensus on the best surgical approach, endoscopic mini-laparotomy, laparoscopy , and robotic resection were reported with good outcomes.Retroperitoneal schwannoma and intra-psoas muscle variant are a rare entity, with slow growing rate and risk of malignant transformation. Therefore, high index of suspicion, good follow-up, and large-cohort studies are encouraged. They predominantly affect female patients from the third to the fifth decade of life with a highly nonspecific presentation ,2. They In this research article, we present a summarized extensive literature review as well as reporting a very rare case of primary left intra-psoas muscle schwannoma treated by anterior laparoscopic hand-assisted resection.2A 39-year-old Saudi gentleman, smoker with an unremarkable medical, surgical and Family history, presented to the emergency department with exacerbated chronic lower abdominal pain since almost 2 years. The pain was mainly in the left lower quadrant, intermittent, pressure-like, and aggravated by sudden movement or lifting objects, which was improved but not relieved by analgesia. No urinary symptoms, signs of lower GI bleeding, obstruction, malignancy, history of irritable bowel syndrome or inflammation were found.On examination, the patient looked uncomfortable, in pain, but not in distress and vitally stable. The abdomen was nondistended, and there were significant tenders on deep palpation on the left lower quadrant without rebound tenderness. Routine lab and urine analyses were unremarkable. Unenhanced CT abdomen was done to rule out renal stones which showed left psoas muscle well-defined heterogeneous soft tissue mass : a–c. HeThe patient was placed in a semi-lateral decubitus position, the surgeon on the right side of the patient with his two assistants. The abdomen was accessed through the supraumbilical area by a 5 mm port. We proceed with an anterior intraperitoneal approach to the retroperitoneum through lateral colonic reflection. Initially, the patient underwent diagnostic laparoscopy then four more ports were placed after that we started with mobilization of the left colon and splenic flexure. Both left ureter and kidney (The anterior-inferior surface of the Gerota's fascia was exposed) were identified and preserved followed by visualization of the swollen left psoas muscle. Using Ligasure, blunt , and cutting dissection were done to dissect the encapsulated mass from psoas muscle and spine was done till a plane was developed all around the mass. The tumor was gently eased out of its area and removed via an extension of the umbilical port. Surgicel was applied and hemostasis secured. After placement of closed suction drainage of the area, the left colon was repositioned on to the left side of the abdomen. The operative time was 167 min, and blood loss was about 43 mL.The patient was discharged on day two postoperatively after showing good bowel motion, taking a regular diet with a significant improvement in pain. The postoperative histopathological examination showed the same diagnosis : a–d. Du3Schwannomas are neurogenic tumors which are known to be solitary, benign (about 5% of benign soft-tissue neoplasms), well-circumscribed, encapsulated, and slow-growing tumors. Only trunks of cranial nerves I and II are not vulnerable to schwannoma if compared to other parts of the body ,6. The mIn 1952, Syred D. R. reported the first case of intra-psoas schwannoma, which was followed by a limited number of cases till today . There wMost often, and like in our patient, incidental and delayed diagnosis of retroperitoneal mass is made which is most probably related to vague symptoms and large and flexible retroperitoneal space ,3. The dA long fusiform structure located on both sides of the vertebral column and pelvis known as psoas muscle plays a significant role in human life. It consists of two origins: the deeper part comes from the first four lumbar vertebrae, while the superficial part comes from the lateral surface of the lower thoracic vertebrae and from adjacent intervertebral discs. The lumbar plexus is located in this area. Distally, it forms iliopsoas muscle by combining to iliacus muscle. During both movement and static states, the psoas major muscle is known to have a biomechanical and postural function. It is also involved in mood and stress disorders ,10.Multiple imaging modalities are used to detect such a tumor, like abdominal ultrasonography, CT abdomen, and MRI . If retroperitoneal tumors are suspected, MRI is highly recommended which offers a better delineation of the origin, extent, and internal composition of these lesions ,6. MRI iBased on the findings of pathology, histology, and immunohistochemistry, a definitive diagnosis is made. On histopathological examination, schwannoma has two types of Antoni areas, including Antoni A  and Antoni B (loosely textured pleomorphic cells), which can present separately or together. In our case, both are present. Immunohistochemistry stain for S-100 protein is a confirmatory test which is strongly expressed ,6.Schwannoma is known to be nonsensitive both to radiation and chemotherapy. Therefore, complete surgical excision with negative margins  is the cornerstone in the management with good prognosis and low recurrence rate ,5,6. In 4Up to date, the total number of reported intra-psoas schwannomas including our case is 46 cases. Generally, retroperitoneal schwannomas are rare, nonsensitive to chemotherapy or radiotherapy, and showing risk of malignant transformation. There is limited data about the incidence, signs and symptoms, and recurrence rate of intra-psoas muscle variant in the literature. Therefore, high index of suspicion, individualization of management, good follow-up, and large-cohort studies are required.All authors have nothing to disclose.Awadh AlQahtani, Mohammed N Alali, Safaa Allehiani, Suliman AlShammari, Hussein Al-Sakkaf, and Maria A Arafah.All authors listed below have no source of funding to disclose.There is no ethical approval was obtained as it’s a case report but a written consent was taken from the family as the patient passed away.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.Awadh AlQahtani: study concept, design, writing the paper.Mohammed alali: data collection, data analysis, interpretation, writing the paper.Safaa Allehiani: data collection, writing the paper.Suliman Alshammari: data collection, writing the paper.Hussein Al-Sakkaf: data interpretation (radiology part).Maria A Arafah: data analysis interpretation (pathology part).Our paper is a case report, no registration was done for it.Awadh AlQahtani: dr_aq1@yahoo.com.Mohammed alali: drmo7ammed2@gmail.com.Not commissioned, externally peer-reviewed.


Primary lymphoma of the cavernous sinus is a rare form of extranodal non-Hodgkin lymphoma, of which very few cases have been reported in the published literature. This report presents the MRI findings with apparent diffusion coefficient (ADC) value in an exceedingly rare primary marginal zone B-cell lymphoma (MZBCL) of the cavernous sinus.–3 mm2/s . Subtotal resection of the tumor was performed, and improvement of clinical symptoms were observed. The pathologic diagnosis of MZBCL was established by immunohistochemical examinations.The case in this study is a 59-year-old immunocompetent male patient with a 2-month history of right ptosis and blurred vision. Right third cranial nerve palsy and binocular diplopia were observed upon neurological examination. Preoperative brain CT showed an extra-axial enhancing mass lesion in the right cavernous sinus. On MRI, ipsilateral internal carotid arterial encasement was noted without causing stenosis of the vessel. Isointense signal on T1-weighted and T2-weighted images, homogeneous contrast enhancement, and diffusion restriction were also observed. The mean ADC value of the tumor is 0.64 × 10Primary MZBCL of the cavernous sinus is exceedingly rare, and preoperative confirmation poses a major challenge with CT and conventional MRI only. In this case, preoperative quantitative ADC value is shown to offer valuable additional information in the diagnostic process. Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma (NHL) typically limited to the neuroaxis without systemic involvement. It represents approximately 1% of NHLs and 4% of all intracranial neoplasms . The bra–3 mm2/s   was thus designated according to the 2014 Lugano classification [The case in this study is a 59-year-old male patient with unremarkable medical history and a 2-month duration of right ptosis and blurred vision. No headache, fever, weight loss or nocturnal sweating is documented. Neurological examination revealed palsy of the right third cranial nerve (CN) and binocular diplopia. Pupillary light reflex and corneal reflex were intact. Routine laboratory data including complete blood count and urinalysis are within normal limits. Brain CT revealed an enhancing mass lesion in the right cavernous sinus Fig. a withoutfication . After sPCNSL arising from the cavernous sinus is a rare occurrence with only 21 known cases published in available literature in the English language. Amongst these cases, 8 are histologically confirmed MZBCL  of the gastrointestinal tract associated with chronic infection of Helicobacter pylori . In addi–3 and 0.71 × 10–3 mm2/s have been reported [–3 mm2/s in six dural MZBCL cases. Although the final diagnosis of MZBCL of the cavernous sinus needs to be confirmed by immunohistochemistry, the preoperative quantitative low ADC value (0.64 × 10–3 mm2/s) in our case offers valuable additional information in the diagnostic process. On conventional MRI, ICA encasement with vascular stenosis is characteristically present in the case of cavernous sinus meningiomas [–3 mm2/s to 0.8 × 10–3 mm2/s had been reported in these high-grade meningiomas as compared with our case [Common differential diagnoses of cavernous tumors include meningioma, schwannoma, metastasis, Tolosa–Hunt syndrome, and hemangioma. From a radiological point of view, differentiation amongst these lesions can be challenging with conventional MRI only, since all may exhibit similar signal intensities and enhancement patterns. However, ADC values may offer additional diagnostic value in differentiating these lesions. In PCNSLs, diffusion restriction with low ADC values between 0.63 × 10reported , 32. De reported  have repingiomas . In contingiomas . Althougour case . Furtherour case . Metastaour case . Tolosa–our case . Hemangiour case . In our our case . TherefoTo the best of our knowledge, this report is the first to mention and discuss preoperative ADC values of the primary cavernous sinus MZBCL. This case suggests that the combination of preoperative MRI and ADC values may offer helpful additional information regarding to the diagnosis and treatment planning for lymphoma of the cavernous sinus.


Unexpectedly, liver biopsy findings were consistent with tuberculous infection. Following the intensive phase of antituberculous treatment, repeat CT revealed significant resolution of the mediastinal lymph nodes making the lung cancer Stage 1 (T1 lesion). She underwent left lower lobectomy as a definitive surgical treatment. Positron emissiontomography/CT scan in this patient was considered to be a hepatic superscan since it revealed a hot liver.A 56-year-old female presented with intermittent hemoptysis and was diagnosed with lung cancer.  A 56-year-old female presented with intermittent hemoptysis. Elevated erythrocyte sedimentation rate (ESR) of 90 mm/h was the only positive laboratory workup finding.18F-FDG PET/CT (F18-fluorodeoxyglucose positron emission tomography/CT) for staging revealed diffuse and extensive uptake in the liver (hypermetabolic liver or rather hot liver) and also by the mediastinal lymph nodes . CT also revealed few necrotic mediastinal lymph nodes, without any calcification in the left paratracheal location and mild hepatomegaly without any focal lesions. ph nodes . The uptThe patient required either EBUS-TBNA  or mediastinoscopic biopsy of lymph nodes or liver biopsy to confirm metastases in the respective sites . We performed ultrasound-guided liver biopsy in this patient, because of the ready availability and ease of this procedure compared to the others.Ultrasound performed showed coarsening of liver parenchyma , which iAfter a discussion with MDT (multidisciplinary tumor board), the patient was started on antituberculous treatment. Repeat CT  after 2 months of the intensive phase of antituberculous drug treatment, revealed significant resolution of mediastinal lymph nodes which confirms the false positivity of mediastinal lymphadenopathy. The lung cancer was thus staged as T1 lesion, instead of Stage IV disease (AJCC TNM staging eighth edition).She underwent left lower lobectomy as a definite surgical treatment. Histopathological examination of the left lower lobectomy specimen showed moderately differentiated adenocarcinoma of the lung with a predominant lepidic pattern. Adjacent lung parenchyma showed multiple granulomas . ImmunohShe was declared cured of her tuberculosis on completing antituberculous treatment. Follow-up imaging after 2 years showed disease-free status.1 A similar term is also employed in 18F-FDG PET/CT imaging.Superscan is classically a ‘super bone scan’ of skeletal scintigraphy.2 in 18F-FDG PET/CT is described as sequestration of the radiopharmaceutical in an organ or an organ system with reduction or complete absence in the activity detected in those organs which are normally associated with significant physiologic uptake of 18F-FDG. The organs showing normal physiological uptake are kidneys, collecting systems, brain, and heart. Hepatic superscan/hot liver is characterized by intense uptake in the liver with suppression of the background physiological activity.Superscan18F-FDG PET/CT is usually associated with malignancy, like massive liver metastases (breast primary), primary hepatic lymphoma, diffuse angiosarcoma of the liver, secondary lymphomatous or leukemic involvement of liver etc. Usually infective etiologies like hepatic tuberculosis,3 Q fever hepatitis will cause increased uptake in the liver without significant suppression of the background physiological activity.2 Metastases from lung cancer rarely cause superscan appearance.4 Very rarely, even infective etiologies can cause superscan appearance, which is the situation in our case.Superscan appearance in the liver in There is an increasing incidence of typical and atypical forms of tuberculosis, especially in developing countries, partly due to the development of drug resistance and partly due to increasing HIV incidence. In a developing country like India, where tuberculosis is endemic, the possibility of the same should be born in mind while considering the differentials of diffuse hepatic involvement. Biopsy proven lung cancer misleads us to believe that hepatic involvement is probably metastatic. However, decision to go ahead with liver biopsy helped us to understand the false positivity of hepatic superscan, detect and treat a potentially fatal infection and perform definitive and curative surgery for lung cancer.It is always safe to consider the possibility of miliary hepatic tuberculosis, whenever we are dealing with a case of hypermetabolic liver, especially in a tuberculosis endemic area. The same had helped us to offer definitive and curative treatment to our patient.Even in the background of proven malignancy, the infective processes like tuberculosis (Koch’s) should be considered as a differential diagnosis for hypermetabolic PET/CT foci, especially in endemic areas like South Asia.In cases of diffuse hepatic involvement, liver biopsy when performed under ultrasound guidance from a relatively heterogeneous appearing area gives a good yield.Hepatic superscan can have a false-positive interpretation for metastases.Tuberculosis in our case was either subclinical/initial presentation of intermittent hemoptysis was due to pulmonary tuberculosis rather than due to malignancy.