Primary cardiac lymphoma (PCL) is a rare disease entity that can present with severe cardiac and cardioembolic symptoms. We present a 79-year-old male with history of polymalgia rheumatica on chronic prednisone who presented with a two-week history of progressively worsening dyspnea, cough, and a 10 pound weight loss. Transthoracic echocardiogram (TTE) and computed tomography (CT) of the chest showed a large mediastinal mass with invasion of the pericardium. A biopsy of an abdominal soft-tissue mass confirmed the diagnosis of PCL. The patient was treated with two cycles of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) which was complicated by progressive heart failure requiring substitution of liposomal doxorubicin. The epidemiology, presentation, diagnosis, and treatment options of PCL are discussed. We present a 79-year-old male with history of polymyalgia rheumatica on chronic prednisone who presented with a two-week history of progressively worsening dyspnea, cough, and a 10 pound weight loss. He initially presented to an urgent care and had been prescribed antibiotics without improvement in his symptoms. He returned to the urgent care and underwent a chest X-ray, which was remarkable for cardiomegaly. Given this finding in conjunction with his respiratory symptoms, he was referred to the emergency department (ED).In the ED, a CT of the chest showed a large mediastinal mass with invasion of the pericardium ; a soft-The patient was admitted to the intensive care unit (ICU) where a TTE showed a left ventricular ejection fracture of 55% along with a large, homogenous adherent mass infiltrating the right atrium and ventricle, abnormal thickening of the interatrial and interventricular septum of the right heart, severe right ventricular dysfunction, severe basal to apical hypokinesis to akinesis, and a pulmonary artery pressure of 21.8 mmHg Figures .Initial differential included primary lymphoma, cardiac sarcoma, or metastatic involvement of the heart. The patient underwent a biopsy of an abdominal soft-tissue mass. Pathology showed diffuse large B-cell lymphoma (DLBCL), nongerminal center type, with BCL2 and MYC.The patient received intravenous (IV) methylprednisolone 250 mg daily for five days for debulking. He was initially treated with two cycles of R-CHOP; however, given his persistently reduced ejection fraction, the patient was changed to liposomal doxorubicin (R-CDOP) for the third and fourth cycle. Furthermore, his first cycle was also complicated by new onset first-degree atrioventricular block and a right bundle branch block. A positron emission tomography (PET)/CT scan and TTE were scheduled prior to the next cycle of R-CDOP. In addition to his systemic chemotherapy, the patient received three cycles of central nervous system prophylaxis with high-dose methotrexate.Malignancy of the heart is most often secondary to metastatic disease or direct invasion. Lymphoma, leukemia, and melanoma are the most frequent primaries that metastasize to the heart . PrimaryThe World Health Organization (WHO) defines PCL as a lymphoma involving only the heart/pericardium or a lymphoma with the bulk of the tumor in the heart in the setting of clinical cardiac symptoms . PCL accPrimary cardiac tumors including PCL do not have a pathognomonic presentation, rather they present based on their location in the heart. Right-sided tumors present with signs and symptoms of right-sided heart failure if they are obstructing blood flow, or they can present with symptoms of pulmonary emboli from embolic tumor fragments into the lungs. Left-sided tumors can present with signs and symptoms of left-sided heart failure if they are obstructing blood flow, or they can present as an ischemic stroke from embolic tumor into the CNS. Lastly, left ventricular tumors that are intramural can present with arrhythmias or conduction defects. An institutional study at the Mayo Clinic found the most common patient complaint on presentation to be dyspnea on exertion (79%) followed by nonspecific chest pain (38%) and cough (21%) .The diagnosis of primary cardiac tumors is based upon imaging and biopsy findings. TTE is often the initial image modality but is limited by operator-expertise and body habitus. CT of the chest can be used but is limited in soft-tissue contrast. Cardiac magnetic resonance imaging (MRI) is the preferred imaging modality, as it provides an unrestricted view, high temporal resolution, and good soft-tissue contrast to help characterize a cardiac mass. In PCL, the tumor often appears as a large nodular mass that is isoattenuating to myocardium on CT, isointense to myocardium on T1, and hyperintense to myocardium on T2 .Imaging often greatly helps characterize the type of primary cardiac tumor. Often the differentiation between benign and malignant or even the specific disease diagnosis can be made based upon imaging alone. Depending on the imaging and suspected etiology of malignancy, tissue sampling may or may not be warranted. If imaging cannot characterize the mass, a discussion of the risks and benefits of an invasive biopsy must take place. Methods of obtaining a tissue diagnosis include myocardial biopsy during exploratory thoracotomy, pericardiocentesis if pericardial effusion present, TEE-guided biopsy, mediastinoscopy, and endomyocardial transvenous biopsy . In our Primary cardiac tumors are treated differently based on the specific pathologic disease. There is no gold standard of treatment for PCL. In reported cases, anthracycline-based chemotherapy and rituximab was associated with prolonged survival , 10. TheIn conclusion, PCL is a rare disease that accounts for 1.3% of primary cardiac tumors and 0.5% of extranodal lymphomas. The disease does not have a pathognomonic presentation, rather it presents based on its location in the heart with signs of heart failure or cardioembolic phenomena. PCL is diagnosed based on imaging and tissue biopsy. If there is a high suspicion for PCL based on imaging, it is important to obtain a tissue biopsy. Definitive tissue diagnosis of PCL can then be treated with anthracycline-based chemotherapy plus rituximab, which has been associated with prolonged survival.


Juxta-cortical chondroma is a rare cartilaginous tumor originating from the periosteum. On conventional radiography, the lesion typically causes saucerization of the adjacent cortex with well-delineated sclerotic margins. Projection radiography may be less accurate than cross-sectional imaging to demonstrate the precise extent of pressure erosion and bone and soft tissue extent. Although magnetic resonance imaging (MRI) is the imaging technique of choice for further preoperative evaluation, cone-beam computed tomography (CT) may be of additional value. Due to its high spatial resolution, cone-beam CT may detect very tiny matrix calcifications and allows a more precise evaluation of the saucerized cortex at a low radiation dose. Juxta-cortical chondroma (JCC) is a rare benign cartilaginous tumor originating from the periosteum . ImagingA nine-year-old boy presented with a slowly progressive swelling at the dorso-ulnar aspect of the proximal phalanx of the fifth finger of the left hand. CR performed five years previously revealed a well-delineated cortical lesion, originally interpreted as a non-ossifying fibroma Figure . CR at aThe lesion was resected followed by bone grafting. Histopathological examination revealed a benign cartilaginous lesion Figure .A 59-year-old woman presented with a slowly progressive swelling for 12 months at the middle phalanx of the right third finger Figure .MRI confirmed a juxta-cortical lesion with associated intramedullary extension. The lesion was isointense to muscle on T1-WI Figure  and hypeThe lesion was resected and filled-up with bone grafts. Histopathology demonstrated a benign cartilaginous tumor Figure .Juxta-cortical chondroma, previously designated as periosteal chondroma, is a rare benign chondroid tumor, accounting for 1.3% of cartilaginous tumors 14. Clinic5On CR, the endophytic part of the lesion has a well-defined, sharp sclerotic margin. The exophytic part of the lesion typically causes pressure erosion of the cortex also known as saucerization . These fOn MRI, the lesion is hypo- or isointense to muscle on T1-WI, is hyperintense on T2-WI, and demonstrates a peripheral or ring-and-arc enhancement pattern, in keeping with chondroid tissue. The juxta-cortical soft tissue component causes pressure erosion with cortex remodelling. Due to its higher spatial resolution than MRI, CT is superior for demonstrating characteristic overhanging bone spicules. Although saucerization is usually visible on CR, superimposition of bone may hamper evaluation of the precise extent. Whereas the shape of overhanging borders may resemble a “Codman’s triangle” on CR, seen in aggressive intramedullary lesions, CT more clearly depicts the well-delineated overhanging shelf of bone around the proximal and/or distal ends of the exophytic component of a benign juxta-cortical lesion [The main drawback of CT is its high radiation dose. Generally, CBCT has a lower radiation dose in comparison to most multi-detector computed tomography (MDCT) equipment. However, with the use of specialized dose modification, radiation dosage of state-of-the art MDCT may be lowered. Another advantage of CBCT is its compact design and lower cost for installation and maintenance. CBCT was particularly useful in case 2, in which cortical breakthrough and extent of the overhanging bony edges were underestimated on CR.Histopathologically, JCC is characterized by a cellular hyaline to myxoid cartilage lacking nuclear hyperplasia. Nuclei are small and round with condensed chromatin. Slightly larger nuclei with open chromatin and small nucleoli are common. The cells can be evenly distributed or arranged in small clusters. More than one cell per lacuna as well as binucleated cells can be present but those features are rather occasional. Mitotic activity is absent . Focal cThe differential diagnosis of JCC include other juxta-cortical lesions such as non-ossifying fibroma (NOF), desmoid, cortical neurofibroma, Nora’s lesion, periosteal chondrosarcoma and periosteal osteosarcoma 8. NOF ra11Treatment includes complete tumor excision with subsequent bone graft filling 9.CBCT may be of additional value to CR and MRI in preoperative characterization and staging of a JCC at a low radiation. Due to the lack of superimposing structures, CBCT may allow more precise interpretation of characteristic radiological features than CR.


Primary bladder leiomyosarcoma was diagnosed in a four-year-old, mixed-breed, spayed female cat that presented with lethargy, stranguria, polyuria, hematuria, urinary incontinence and abdominal sensitivity. On abdominal ultrasound, the urinary bladder was observed to have a preserved anatomical position and a hyperechoic mass. The mass measured approximately 1.5 cm, was irregular, and arose from the mucosa of the bladder wall. Due to the evidence of a primary tumor in the urinary bladder, we conducted a partial cystectomy with a 1.0 cm surgical margin and performed histopathology and immunohistochemistry. The histopathology revealed a poorly differentiated malignant neoplasm, characterized by the proliferation of spindle cells with moderate nuclear pleomorphism, suggestive of leiomyosarcoma. Immunohistochemistry confirmed the histopathological diagnosis, showing positive staining for vimentin, desmin and alpha-smooth muscle actin and negative staining for S100, pan-cytokeratin and MyoD1. We also assessed the proliferative index by Ki67 staining and found that 57% of the neoplastic cells were positive for Ki67. We conducted clinical follow-ups every three months in the first year and every six months thereafter. The patient showed no signs of recurrence after 48 months. The surgery was sufficient to treat the leiomyosarcoma, and adjuvant chemotherapy was not necessary in this case. Feline bladder tumors are very rare in veterinary medicine. Primary transitional cell carcinoma ,2,3 are Due to the limited information regarding the diagnosis and outcomes of urinary leiomyosarcomas in cats, we describe herein a case of primary bladder leiomyosarcoma in a female cat.®, Aimargues, France) for recurrent urinary disorders. However, after cessation of treatment, there was a recurrence of the clinical signs. Due to the history of chronic lower urinary tract disease, a complete blood count (CBC) was performed, a dosage of creatinine was administered, and urea, urinalysis, and uroculture tests were performed with an antibiogram and abdominal ultrasonography. The CBC showed increased hemoglobin (18.0 g/dL), hematocrit (50.0%), eosinophils (2000/µL), lymphopenia (900/µL) and total protein (8.1). There were no alterations in creatinine (1.1 mg/dL) or urea (30 mg/dL) values. Urinalysis revealed hyposthenuria, proteinuria (500 mg/dL), hematuria, and bacteriuria. The uroculture evidenced the growth of Bacillus sp. , which was sensitive to enrofloxacin. Abdominal ultrasonography revealed a poorly filled urinary bladder, with a preserved anatomic shape, showing an irregular mass measuring 1.5 cm in diameter that was adhered to the wall, and exhibited areas of calcification  and ketoprofen (1 mg/kg SID for 5 days) was performed. Then, a partial cystectomy was performed, with the whole tumor mass removed with a 1.0 cm surgical margin. The gross morphology of the tumor mass revealed a brownish nodule with a lobed and ulcerated surface measuring 2.2 × 1.3 × 0.8 cm. The histological evaluation revealed a poorly differentiated malignant neoplasm ulcerating the bladder mucosa . The neoThe immunohistochemical evaluation was performed using citrate buffer (pH 6.0) and a pressure cooker . The slides were placed in an automatic immunohistochemical processing unit, in an Autostainer Classic® platform . The antibody information is described in The tumor cells stained red with Masson’s trichrome, and only the infiltrating fibroblasts were found in the collagen matrix . The association between FLUTD and bladder neoplasm has been described in the literature ,4,5,6,7.The ultrasound evaluation is useful to identified tumor patterns and infiltration into bladder wall ,4,5,6. URegarding bladder sarcomas, there is little information regarding the association between inflammation and tumor development. Three previous reports in the literature ,8,9 descVimentin staining indicated a tumor from mesenchymal origin, whereas desmin demonstrated a tumor originating from muscle. The alpha-actin is protein from smooth muscle fibers, and MyoD1 from skeletal muscle fibers . The posThere was described in the previous literature, a two bladder fibrosarcoma treated with surgery ,13. In oEven with a high proliferative index, a surgical approach was sufficient to provide long-term survival for the cat studied in this report. Feline leiomyosarcoma should be included in the differential diagnosis of patients with chronic lower urinary tract disease and histochemistry. Immunohistochemistry can be used for the definitive diagnosis.


Disc herniation is one of most common causes of spine surgery. Because of the presence of posterior longitudinal ligaments, disc fragments often migrate into the ventral epidural space. A posterior epidural herniation of a disc fragment is a rare occurrence. We report two cases of posterior migrated disc fragments, with, radiological and clinical findings. Because of the rarity of a posterior migration of the intervertebral disc fragments, a differential diagnosis can be challenging. This painful syndrome associated with neurological lower limb deficits can be confused initially, with other posterior epidural space-occupying lesions such as tumours, abscess or hematomas. A gadolinium-enhanced MRI scan is the gold standard for a correct diagnosis. Early surgical decompression of the spine with a posterior approach remains the optimal technique in ensuring the best possible outcome for the patient. We report two cases of posterior migrated disc fragments, in whom neurologic symptoms and deficits were present at the time of admission.Disc herniation is very common, representing one of the major reasons for spine surgery. Literature reviews estimate that 35 to 72% of all lumbar disc herniation cause fragment migration, usually in the anterior and anterolateral epidural spaceA 53-year old man was admitted to our emergency unit with low back pain , bilateral leg pain (VAS 9) and acute paraparesis with leg extension and bilateral hip flexion deficit without trauma. A neurological examination found that the strength in the right hip flexion and the right knee flexion was grade 2 with bilateral hypoesthesia in the L4 and L5 region. No other neurologic abnormalities were found. Blood tests revealed no alterations. CT scan of lumbar spine did not reveal any significant findings. Therefore, an MRI of the lumbar spine was performed. MRI images showed a mass in the dorsal epidural space with compression of the epidural sac at the L3-L4 level . FifteenA 49-year old man was admitted to our emergency unit for acute dorsal pain experienced during driving. He had no trauma. The patient had bilateral hypoesthesia in the L2-L3-L4-L5 region and VAS for back pain was 9. A neurologic examination revealed a condition of paraparesis. The strength of knee flexion and extension was grade 3 bilaterally, the hip flexion was grade 3 bilaterally and the ankle flexion and extension was grade 4. The blood test revealed no alterations. MRI of the thoracic spine revealed a wide lesion in the dorsal epidural space with compression of the epidural sac at the T6-T7 level . Twelve 5. The midline septum extends from the vertebral body to the posterior longitudinal ligament and prevents the lateral migration of disc fragments.Disc fragment migration in the spinal canal is a common condition although it generally occurs in the anterior epidural space. Posterior disc fragments migration in the epidural space is a rare condition but when it does occur, it becomes a diagnostic challenge that requires prompt diagnosis and decompression surgery. When a disc fragment is pushed back violently and crosses the posterior longitudinal ligament (PLL), posterior migration should be prevented by the presence of certain anatomic structures such as the "midline septum", the "peridural" membrane, the nerve roots and the Hoffman ligamentsThe peridural membrane extends from one side to the other, spanning the width of the vertebral body. Anterior dural (Hoffman) ligaments, are subtle attachments between the dura mater and the deep layer of the PLL, with two ligaments (right and left) usually present at each level. Finally, nerve roots, epidural fat and epidural venous plexus also are structures that can prevent posterior epidural migration. However, the actual role of all these structures in preventing disc fragment migration remains unknown. In this aspect, the occurrence of posterior epidural migration of disc fragments can be related to strong expulsion forces that push back the fragment over all anatomic barriers. Anatomic variations like an abnormal position of the disc, cranial or caudal to the intervertebral foramen, could be associated with a different of the position of the nerve roots and may allow for posterior migration.1. Disc fragments, in 80% of cases, are generally hypointense in T1-weighted images and hyperintense on T2-weighted images. Sometimes, diagnosis is only possible during surgery where the real nature of the compression appears clearly. The treatment of choice is the removal of the extruded fragments with decompression of neurologic structures.The clinical presentation of these patients is characterised by significant neurological symptoms due to the compression of the nerve roots, such as cauda equina syndrome. In some cases, posterior migration is characterised by chronic back pain, while in other cases an acute onset may be observed. Diagnosis, on the other hand, can be challenging because of a variety of diseases caused by posterior epidural space-occupying lesions such as tumours, abscess or hematoma. The only way to achieve the correct differential diagnosis is a gadolinium-enhanced MRI that, in the case of extruded disc fragments due to vascularisation of the epidural fat surrounding the fragment, shows "ring enhancement” after the injection of the contrast mediumIt should be remembered that the approach to thoracic disc herniation is usually anterior. In the second case described, a posterior approach was performed to allow a prompt spine decompression; no discectomy was performed. Early surgery had to be carried out to prevent severe neurologic deterioration and to allow neurologic recovery after surgery.The authors declare no conflicts of interest.


Primary testicular lymphoma is a rare testicular neoplasm that mainly affects elderly patients, with Human Immunodeficiency Virus (HIV) being a known risk factor in the younger population. Approximately 20% of patients will have disseminated disease with extra-nodal involvement at clinical presentation. Rarely, direct spread along the spermatic cord and gonadal vessels can occur and has been described in the literature. We present two cases of this phenomenon where the primary testicular tumour has spread along the gonadal vein to its origin at the inferior vena cava. Primary Testicular Lymphoma (PTL) is an uncommon neoplasm representing less than 5% of all testicular tumours and between 1-2% of all cases of non-Hodgkin's Lymphoma (NHL) , 4. AlonPTL typically presents as a unilateral painless testicular mass or scrotal swelling and is difficult to differentiate clinically and on imaging from germ cell tumours. Rarely pain may occur, while constitutional symptoms are indicative of systemic disease, which is present in 20% of patients at the time of diagnosis.NHL of the testis may be associated with extra-nodal involvement of the central nervous system (CNS), Waldeyer's ring, lungs, pleura, skin, and soft tissues as well as the contralateral testis. A small number of case reports however describe direct spread along the spermatic cord and gonadal vessels into the retroperitoneum . In the A previously well 77-year-old gentleman presented with a 6-week history of right-sided testicular swelling and gradual onset of pain with no preceding history of trauma or known malignancy. He was initially treated in primary care for suspected orchitis but due to persisting symptoms he was referred for a scrotal ultrasound.The ultrasound study  demonstrCT demonstrated an enhancing right-sided testicular mass  with sofFollowing a multidisciplinary team (MDT) discussion and with lymphoma being the main differential due to the distribution of the lesions, the testicular mass was biopsied under ultrasound guidance and histology results demonstrated diffuse large B-cell lymphoma . Lymphomatous tonsillar involvement was confirmed on biopsy and gastroscopy following an episode of haematemesis showed gastric infiltration, not evident on imaging. Imaging investigations were completed with whole spine and brain Magnetic Resonance Imaging (MRI) to assess for CNS involvement. The patient was subsequently commenced on chemotherapy for stage VI Diffuse large B-cell lymphoma, with follow-up 3-month imaging showing very good partial response.An 82-year-old patient with a background of monoclonal gammopathy of undetermined significance (MGUS) and previous prostate cancer treated with external beam radiation therapy presented with constitutional symptoms. On clinical examination an enlarged right testicle was noted and serum biochemistry revealed hypercalcaemia.A CT of the chest, abdomen, and pelvis was performed to assess for a new underlying malignancy or prostate cancer recurrence. The study demonstrated a large right-sided scrotal mass Figures  with sofA subsequent ultrasound  was perfAfter discussion at the urology cancer MDT a differential diagnosis of sarcoma and lymphoma was suggested and a decision for ultrasound guided biopsy of the testicular lesion was made, rather than orchiectomy due to epididymal and spermatic cord involvement. Histology was consistent with diffuse large B-cell lymphoma, germinal centre subtype. The patient was subsequently referred to haematology and following 3-cycles of chemotherapy demonstrated complete radiological response.As PTL carries a worse prognosis  than either germ cell tumours or NHL involving lymph-node groups or other extra-nodal sites, timely differentiation from other scrotal masses is required . TreatmeThe main differential diagnoses for PTL include germ cell tumours, acute and chronic epididymo-orchitis and leukaemia. Unlike lymphoma, germ cell tumours are frequently associated with raised serum Human chorionic gonadotropin (HCG) or alpha-fetoprotein (AFP) and metastasise in a predictable fashion cranially through successive retroperitoneal lymph-node groups . AlthougAt CT and ultrasound, sporadic nodal, and extra-nodal deposits around the body increase the probability of lymphoma over germ cell tumours or infection. As in the two cases presented here, cord-like soft tissue extending in a contiguous fashion from the testicular mass onto spermatic cord and gonadal vessels in the retroperitoneum is a very specific feature that has only ever been described in PTL.


We report a rare case of giant esophageal lipoma treated with thoracoscopic enucleation successfully.A 69-year-old woman was referred to our department with dysphagia. Computed tomography examination revealed a large hypoattenuating submucosal mass with dense fat in the middle and lower esophagus. Upper gastrointestinal endoscopy revealed a submucosal mass with normal mucosa on the middle and lower esophageal wall. On a diagnosis of esophageal lipoma, we performed a video-assisted thoracoscopic operation and the 14.0 × 6.5 × 3.0 cm in size submucosal tumor was completely enucleated. We could successfully avoid a subtotal esophagectomy with high invasiveness. The patient was discharged on the 36th day after operation, and no symptoms had been noted.Video-assisted thoracoscopic enucleation with minimal invasiveness may be an appropriate treatment option even for such a huge benign esophageal submucosal tumor. Benign tumors of the esophagus are very rare. Esophageal lipomas, in particular, account for only 0.4% of all digestive tract benign tumors . BecauseA 69-year-old woman was admitted to our hospital with dysphagia during the previous 1 month. The clinical interview disclosed a history of uterine myoma and bronchial asthma. Physical examination on admission revealed normal findings. A chest computed tomography (CT) scan revealed a 10 × 7 cm submucosal mass in size on the middle and lower esophagus. Upper border of the mass was not close to the tracheal bifurcation. The tumor was hypoattenuating and characteristic of adipose tissue  at the anterior axillary line for assistant and 7th ICS at the posterior axillary line for scope, and two 5 mm ports were inserted at 5th and 9th ICS at the midaxillary line in the right lateral chest wall respectively for operator. First of all, the mediastinal pleura was opened, and a large bulge was visualized in the middle and lower esophagus. The esophagus was mobilized circumferentially, and two 3-0 monocryls were placed around the middle and lower esophagus to retract the esophagus for the remainder of the procedure. A longitudinal incision of the right esophageal wall was performed to expose the lumen of esophagus (Fig. Upper gastrointestinal endoscopy 8 days after the operation showed anastomotic ulcer and reflux esophagitis Fig. a. These Esophageal submucosal tumors represent less than 1% of all esophageal neoplasms . Of thesThere are few case reports of esophageal lipoma treated with thoracoscopic enucleation. Though Chien-Ying Wang et al.  reportedThe knack of this operation is to develop the appropriate submucosal layer along the tumor surface to preserve the muscle layer, after incision of mucosa of left esophageal wall. Since the tumor was elastic soft, the enucleation was possible with a mucosal incision shorter than the actual tumor size. In addition, after a longitudinal incision of the right esophageal wall, by four retraction sutures placed on the esophageal wall from the outside of the thorax to secure the view of esophageal lumen, we could easily separate the submucosal layer or reapproximate split mucosa layer Fig. d. If theHowever, video-assisted thoracoscopic enucleation and suture can be performed safely only by well-trained surgeons. Watanabe et al. reported the difficulty of thoracoscopic enucleation for an esophageal schwannoma larger than 5 cm and the conversion to subtotal esophagectomy . RecentlIn addition, there are other suggested surgical plans for enucleation of esophageal lipoma. Tsalis et al.  reportedWe could remove an exceptionally large esophageal lipoma with thoracoscopic enucleation. Although esophageal lipomas are extremely rare, thoracoscopic enucleation is considered a safe and useful treatment.


Kaposi's sarcoma involving the digestive tract in isolated form before the appearance of diffuse skin lesions is very rare in HIV‐negative patients and is a condition requiring watchful waiting. We describe a case of a 61‐year‐old woman undergoing an excision of a solitary nodule in the colon, with diagnosis of classic Kaposi's sarcoma. Cutaneous lesions appeared on the legs after three months from colon manifestation. Kaposi's sarcoma involving the digestive tract before skin lesions is very rare in HIV‐negative patients.1What is the percentage of Kaposi's sarcoma involving the digestive tract in HIV‐negative patients?22 every two weeks yielded partial response and was effectively continued until 12 courses.A 61‐year‐old woman was admitted to our hospital due to Stevens‐Johnson's reaction of uncertain etiology and mild anemia. Diffuse cutaneous macule‐papules were observed in thorax and lower limbs and empirically treated with antibiotics and steroids. Endoscopic examinations of stomach were negative, while colonoscopy allowed to evidence and remove a solitary purple nodule in the transverse colon, with diagnosis of classic Kaposi's sarcoma (Figure None declared.GC: participated in the management of patient care and wrote the paper. LT: analyzed the colon tissue specimens and made pathological diagnosis of tumor. SM: guided treatment decisions and contributed to the management of patient care.


A 65-year-old woman presented with right lower quadrant (RLQ) abdominal pain of three days duration. During her hospitalization, she underwent computed tomography (CT) of the abdomen, duplex ultrasound of the abdomen, esophagogastroduodenoscopy (EGD), and colonoscopy as part of a diagnostic workup. The workup identified high-grade obstructions of the celiac artery (CA), superior mesenteric artery (SMA), atypical appearing gastric ulcers, and a diffusely ulcerated cecum, which created a mass-like appearance. The patient developed cecal perforation despite mesenteric vessel stenting and ultimately required right hemicolectomy for definitive management. This case report represents a rare presentation of simultaneous gastric ischemia and cecal ischemia with necrosis in a patient with underlying peripheral vascular disease. Isolated gastric ischemia and isolated cecal ischemia are uncommon due to an abundance of collateral circulation in the stomach and the colon. Furthermore, such cases are not commonly reported in the literature -2. IsolaA 65-year-old female presented to the emergency department with complaints of abdominal pain, nausea, and vomiting of three days duration. In the week prior to admission, she had a poor appetite and a 4 lb weight loss. Previous medical history was significant for gastroesophageal reflux disease (GERD), hypertension, peripheral vascular disease, and chronic constipation. Social history was notable for cigarette smoking for several decades. The patient's abdominal pain was predominantly in the right lower quadrant (RLQ). One year prior, she had undergone esophagogastroduodenoscopy (EGD) for further evaluation of abdominal pain; it revealed significant inflammation in the stomach and was labeled as “hemorrhagic gastritis.” Gastric biopsies showed no evidence of infection or malignancy. At that time, colonoscopy to evaluate her abdominal pain and constipation revealed small areas of ulceration in the cecum that on biopsy revealed a fibrinous exudate without necrosis. Random colon biopsies from normal-appearing mucosa were unremarkable. There was no histologic evidence or history of inflammatory bowel disease (IBD). At the time of this most recent hospitalization, her vital signs were within normal limits. Physical exam disclosed a slender and frail-appearing woman in no distress. Abdominal examination revealed a mildly tense abdomen with exquisite tenderness to even gentle palpation in the RLQ. There were no peritoneal signs. Routine laboratory tests revealed leukocytosis with white blood cell (WBC) 19.4x10^3/uL and normal hemoglobin and platelet counts. The basic metabolic panel was normal except for a potassium level of 2.5 mEq/L. The liver chemistry panel, lipase, and troponins were within normal limits. Lactic acid was minimally raised at 2.1 mmol/L and urinalysis revealed no indicators of infection.Computed tomography (CT) abdomen and pelvis, with intravenous (IV) contrast at the time of admission, disclosed a 2.9 cm infra-renal abdominal aortic aneurysm (AAA) with mural thrombus as well as high-grade stenosis of the left common iliac artery (LCIA). The celiac artery (CA), superior mesenteric artery (SMA), and superior mesenteric vein (SMV) were reported to be patent. On Day 2 of admission, a right upper quadrant ultrasound (RUQ US) revealed a small amount of gallbladder sludge but no evidence of cholecystitis or choledocholithiasis. The patient was empirically started on metronidazole and ciprofloxacin for possible infectious and/or ischemic colitis. On Day 2 of admission, she developed rectal bleeding and her WBC count increased to 22.3 x10^3/uL. On Day 3 of admission, a repeat CT abdomen and pelvis with IV contrast was unremarkable. She underwent EGD and colonoscopy on Day 5 of admission. The EGD revealed scattered, atypical appearing ulcers in the fundus, in the body along the lesser curvature, and in the antrum (Figure Biopsies of the cecum revealed inflammatory exudates as well as significant gangrenous necrosis consistent with ischemia. The colon was otherwise normal aside from mild sigmoid diverticulosis. Also obtained on Day 5 of admission was a duplex US of the abdomen, which was remarkable for a peak systolic velocity of 543 cm/s at the origin of the celiac artery, indicating high-grade stenosis. A peak systolic velocity of 805 cm/s at the origin of the SMA was seen, also indicating high-grade stenosis. The origin of the IMA was occluded but evidence of collateral vessel formation was identified distally. The peak systolic velocity at non-occluded portions of the IMA was 52 cm/s. Two days later, the patient underwent a mesenteric angiogram and stenting of the CA and SMA. Dual antiplatelet therapy with aspirin and clopidogrel was initiated and the patient was also started on a statin. On post-procedure Day 1, the patient reported an improvement in her abdominal pain. On post-procedure Day 2, she had a recurrence of her RLQ abdominal pain and developed tachycardia, worsening leukocytosis, and an acute kidney injury. CT abdomen and pelvis without contrast revealed the development of moderate ascites but did not show any evidence of acute bowel injury. On hospital Day 9, the patient underwent exploratory laparotomy. Upon opening the abdomen, 1.5 liters of ascitic fluid was drained. The right colon and cecum were adherent to the anterior pelvic sidewall. Significant swelling of the cecum was seen and a contained perforation was found. The patient underwent a right hemicolectomy with primary anastomosis. The patient tolerated the procedure well and had no complications. The patient was discharged in good condition on hospital Day 19.Gastric ischemia and cecal ischemia are significant causes of morbidity and mortality, especially in the elderly population . The etiIsolated cecal ischemia most commonly presents as either chronic or acute right lower quadrant abdominal pain . In seveGastric and cecal ischemia can be detected by endoscopic evaluation, cross-sectional imaging, ultrasound examination, and diagnostic angiography ,5,11-14.The treatment of gastric ischemia includes fluid resuscitation, low-intermittent nasogastric suction to combat gastric distension, intravenous proton pump inhibitor therapy, and the select usage of broad-spectrum antibiotics if sepsis or gastric pneumatosis is present. Angiographic interventions may be indicated in cases of splanchnic vascular obstruction. Surgical intervention is indicated in cases of gastric perforation, gastric volvulus, or cases of severe gastric ischemia, which fail to improve with medical therapy ,20. CurrGastric ischemia is rare due to the rich vascular supply of the stomach. However, gastric ischemia is likely underdiagnosed. The endoscopic findings of scattered or localized gastric mucosal disruptions, obliteration of the vascular pattern in the mucosa, and the presence of atypical-appearing ulcers should raise the suspicion for gastric ischemia and prompt treatment should be initiated.Clinicians should consider isolated cecal ischemia with necrosis in patients presenting with significant RLQ abdominal pain, especially those with underlying vascular disease. While isolated cecal ischemia with necrosis has high morbidity and mortality, with early diagnosis and prompt treatment, it can be successfully managed


Cystic lymphangioma is an uncommon benign tumor that occurs primarily in children in the cervical region. We report the first case of a pleural-based cystic lymphangioma in an infant. The patient was admitted for RUL pneumonia. Because of the persistence of the radiographic findings despite clinical improvement, a computed tomography (CT) and a magnetic resonance imaging (MRI) scan were performed. They showed a multiloculated cystic lesion in the superior posterior right hemithorax. A surgical procedure was performed with complete resection of the tumor. Histopathological examination showed a pleural-based intrathoracic multicystic lymphangioma. One year after the surgery, the patient feels well without any sign of recurrence. Cystic lymphangioma is a rare congenital malformation first described in 1843 . These cμl with 61% PMN) and elevated C-reactive protein level ). Chest X-ray demonstrated a large infiltration in the right upper lobe , bronchogenic cysts, and the cystic form of neuroblastoma . Recurre success . In our In summary, we have presented the first case of pleural-based intrathoracic multicystic lymphangioma in an infant presenting as pneumonia with complete cure after surgical resection.


Primary osseous tumors of the spinal column account for approximately 1% of the total number of spinal tumors found in the pediatric patient population. The authors present a case of a C1 benign giant cell lesion that was incidentally found in a 15-year-old patient. A transoral biopsy was performed followed by treatment with denosumab, with definitive management in the form of transoral tumor resection with subsequent occiput-cervical three posterior instrumented fusion. The patient tolerated all of the procedures well, as there were no post-operative complications, discharged home neurologically intact and was eager to return to school when assessed during a follow-up visit in clinic. Osteolytic lesions affecting the cervical spine are rare in the pediatric population. It is of utmost importance to have sufficient background knowledge in order to formulate a differential diagnosis, as well as an understanding of principles underlying surgical techniques required to prevent occipital-cervical instability in this patient population. The information presented will guide surgical decision-making by identifying the patient population that would benefit from neurosurgical interventions to stabilize the atlantoaxial junction, in the context of rare osteolytic conditions affecting the cervical spine. Spinal tumors comprise approximately 5–10% of all pediatric central nervous system (CNS) tumors ,3. PrimaA 15-year-old female with no significant past medical history presented after being struck in the face by a ball while playing water polo. The patient felt pain in her jaw, which was the chief complaint when she presented to the emergency department. Upon neurological assessment, the patient complained of midline tenderness from the skull base to midline cervical spine over C3; denied headaches, changes in vision, speech or swallowing, extremity weakness or paresthesias. A maxillofacial computed tomography (CT) scan did not show evidence of an acute facial fracture. However, the CT scan did reveal a radiolucent, ovoid-shaped lytic lesion arising in the left lateral mass of C1, between the anterior tubercle and the transverse process. Magnetic resonance imaging (MRI) studies further confirmed an enhancing osseous lesion at the left lateral mass of C1, with cortical breach and extension into the left lateral atlantodental joint space . Of noteDifferential diagnoses underlying this vertebral cortical erosion included those of infectious etiology, as well as oncologic lesions, such as giant cell tumor of bone, aneurysmal bone cyst, osteoblastoma, osteosarcoma or even Langerhans histiocytosis (LCH). Oncology recommended that the cervical spine lesion be biopsied for tissue diagnosis. Due to the unusual location of the lesion and risk of locally aggressive pathology, or possible tumor seeding along the biopsy track, interventional radiology was unable to perform a CT guided needle biopsy. It was therefore decided that the patient would require open neurosurgical biopsy for diagnosis. Due to the anterior and lateral location of the vertebral lesion, an anterior transoral approach to the C1 lesion was performed, in order to obtain a sufficient amount of the contrast enhancing component of the mass for pathologic diagnosis. The transoral approach was performed in a multidisciplinary fashion, during which the otolaryngology team used direct visualization, as well as stereotactic navigation, to expose the C1 anterior tubercle on the left side. Once exposure was completed, neurosurgery team utilized a matchstick burr to then drill the anterior outer cortex of C1. Multiple specimens from the fibrous tumor were taken, with curettes and pituitary forceps. The sampled tissue did not show features of osteoblastoma or osteosarcoma, nor were there features of LCH or signs of infection. In the sampled region, the lesion consisted of a proliferation of nondescript stromal cells with intermixed multinucleated giant cells, and occasional clusters of foamy histiocytes . SpecialThe patient was re-assessed three months postoperatively and MRI studies revealed that there was no interval decrease in the size of the tumor. In fact, there was a slight progression of the lesion anteriorly, despite treatment with Denosumab. After presenting the patient’s case at our institution’s multidisciplinary tumor board, it was decided to offer the patient a gross total resection of the offending lesion. This would inherently lead to significant atlantoaxial instability, therefore a posterior occiput to cervical three instrumented fusion was also warranted.The transoral approach was performed in a multidisciplinary fashion, during which the otolaryngology team used direct visualization as well as stereotactic navigation, to expose the cervical vertebrae through the posterior pharynx. Fibrous tumor was identified and dissected until superior, inferior, and lateral margins of tumor resection were confirmed grossly, with fluoroscopy, and neuronavigation. Additional C1 anterior tubercle eccentric towards the right side was also taken, to include a normal bony margin. A small rim of tumor adherent to the vertebral artery was left behind. After the otolaryngology team closed the posterior pharynx, the patient was carefully turned prone, maintaining spinal precautions. Base of the occiput to cervical three was then exposed. C2 pedicle screws were placed. C3 lateral mass screws were placed. An occipital plate was sized. Screws into the occiput were placed. Fluoroscopy confirmed excellent position and spinal alignment. There were no post-operative complications and the patient was discharged home in good condition. Pathologic examination of the resected material at this time showed complete disappearance of the giant cells, due to Denosumab therapy, with the remaining lesional tissue resembling benign fibrous histiocytoma . Post-opThe differential diagnosis for a spinal column tumor in a pediatric patient is broad, ranging from primary osseous lesions like osteochondromas and osteosarcomas, to tumors of soft tissue origin like rhabdomyosarcoma or neuroblastoma. If the patient’s age is taken into consideration, the most common primary osseous tumors in the pediatric spinal column are as follows: 0–5 years of age: eosinophilic granuloma; 5–10 years of age: aneurysmal bone cyst, eosinophilic granuloma, osteoblastoma, osteoid osteoma, osteosarcoma, and Ewing sarcoma; lastly 10–20 years of age: aneurysmal bone cyst, osteochondroma, osteoid osteoma, osteosarcoma, Ewing sarcoma [The most common primary osseous tumor in children is osteochondroma, which makes up about 4% of solitary spinal column tumors. They are benign lesions that consist of an abnormal outgrowth of bone with cartilaginous cap ,7,8. Of The most common malignant primary bone tumor seen in the spinal column of pediatric patients is Ewing sarcoma. Interestingly, these lesions can either arise as primary spinal lesions, or metastasize from other locations to the spine, a characteristic not usually seen in other primary osseous lesions. Ewing sarcoma has a predilection for the sacrum and are found less commonly in the lumbar, thoracic spine, and cervical spine, with decreasing frequency . OsteoblAnother benign primary osseous lesion that can arise in the spine is the aneurysmal bone cyst. These lesions typically present in patients that are 20 years of age or younger and usually affect the lumbar spine ,15. Of nManagement of most osseous spinal lesions involves en bloc resection with clear margins to avoid tumor recurrence and can be followed by adjuvant therapy, if indicated by pathologic analysis ,20. HoweTo prevent occipitocervical instability, it is often necessary to reconstruct elements of the cervical vertebral column after tumor extirpation. Instability is usually due to a combination of destruction of local anatomy by the tumor and correlates to extent of bony and ligamentous removal during surgery . In thesHere we report a case of an osteolytic lesion that was incidentally found in the cervical spine of a pediatric patient. The relevant differential diagnosis, as well as surgical techniques involved in treating this patient, have been thoroughly discussed. Anatomy of the atlantooccipital and atlantoaxial joint spaces is complex and medical management of osteolytic lesions discovered in this area should be considered as first-line treatment. However, when the offending lesion is locally aggressive and does not respond to medical management, one must consider surgical intervention for attempted gross total resection, with stabilization of the cranio-vertebral junction. Stabilization of the aforementioned junction using instrumented fusion is key to prevention of patient morbidity and mortality. It is our hope that the information presented will guide surgical decision-making. It is critical to be able to correctly identify the patient population that would most likely benefit from neurosurgical interventions, in the context of rare osteolytic conditions affecting the cervical spine.


Primary sarcomas of the gallbladder (GB) are a rare disease that were first described by Griffon and Segall in 1897. Leiomyosarcomas (LMS), as described in the case report at hand, are considered a major subgroup.A 62-year-old female was referred to our hospital with intermittent right upper quadrant pain. A 45 mm mass arising from the neck of the GB was diagnosed by ultrasound. No distant metastases were revealed. We successfully removed the GB and the surrounding liver tissue. We also performed a lymphadenectomy of the hepatoduodenal ligament. The histopathological and immunohistochemical examination revealed an R0 resected epithelioid LMS of the GB.Our review of literature shows only 20 publications of LMS of the GB. The majority of the patients are female with an average age of 65.95 years. In a non-metastasized stage, a cholecystectomy with a wedge resection of the surrounding liver tissue, accompanied by a lymphadenectomy of the hepatoduodenal ligament, is described as a successful surgical approach.The LMS should be taken under consideration when diagnosing a tumor of the GB. LeiomThe work has been reported in accordance with the SCARE criteria .2A 62-year-old female was referred to our hospital due to intermittent right upper quadrant and epigastric pain for 7 days in 2015. Her medical history consisted of hypothyroidism and pyelonephritis. Her surgical history included an appendectomy. During the clinical examination a positive murphy sign was found. The laboratory tests demonstrated an elevated bilirubin, y-glutamyl transferase, alkaline phosphatase, and lipase. Additionally, the inflammatory markers were elevated. The patient underwent an abdominal ultrasound. An inflamed GB with multiple gallstones was detected. Moreover, a 45 mm mass arising from the neck of GB was found . Therefo2.1The histopathological examination diagnosed an R0 resected 40 mm smooth bounded spindle-shaped epithelioid tumor in a chronic inflamed GB. The liver tissue and lymph nodes did not contain malignant cells : Contribution to the paper: author, data collection, data analysis and interpretation, writing the paper.Dr. med. Muharrem Salak (co-author): Contribution to the paper: surgical treatment of the patient.PD Dr. med. Thomas Mairinger (co-author): Contribution to the paper: data analysis.PD Dr. med. Franz Theissig (co-author): Contribution to the paper: Histopathological examination, interpretation of the histological pictures, immunohistochemically examination.The case report at hand is not a first-in-man case report of a novel technology or surgical technique, therefore a registration of these case reports according to Declaration of Helsinki 2013 is not required.Dr. med. Christoph Paasch.Helios Klinikum Berlin-Buch.Department of General, Visceral and Cancer Surgery, Schwanebecker Chaussee 50, Berlin.Not commissioned, externally peer-reviewed.None.


Juvenile polyposis syndrome (JPS) is a non-cancerous benign growth predominant in a young population with an estimated incidence of one in 1, 00,000 to 1, 60,000 per year. It is a rare genetic presentation, which can occur sporadically as well. There is a 39% evident risk of developing colorectal carcinoma. Herein, we present an unusual case of a 13-year-old girl from a rural area with a negative family history of juvenile polyposis, who had complaints of rectal prolapse and rectal bleeding which were more pronounced after defecation. Her contrast computed tomography (CT) scan revealed a distended large bowel studded with multiple juvenile polyps throughout, the largest of which was detected on the mid rectum. Colo-colic intussusception was also found due to a large polyp at the hepatic flexure, inferiorly extending up to ascending colon and caecum. Pan proctocolectomy with ileoanal J pouch anastomosis was performed, bearing in mind the risk for colorectal cancer and her general state of health. Juvenile polyposis syndrome (JPS) is characterized as a non-cancerous benign growth, which occurs predominantly in the young population. It commonly presents with anemia, abdominal pain, diarrhea and bleeding per rectum; however, in few case presentations, it may be accompanied by intussusception, intestinal obstruction or polyp prolapse . Numerous juvenile polyps constitute an increased risk of intestinal cancer if they are more than five in number .The colon is the most common site but it can be located anywhere along the gastrointestinal tract. JPS is typically recognized by the age of 30 years, while the average presenting age is 9.5 years . It is aA 13-year-old girl from a rural area was admitted to the Surgery Department of Civil Hospital, Karachi, Pakistan with a history of painful rectal bleeding and protrusion of a red mass through anus after defecation for the last two years. Initially, the mass was small in size although it progressively increased in size, which protruded on defecation and had to be reduced manually. It was associated with fresh bleeding and pain after passing stool. She developed pallor and generalized body weakness over time. However, the patient had no significant history of nausea, vomiting, diarrhea, constipation, blood transfusion or any surgery. Her family history was also negative for colonic polyps and cancer. She was taken to a local general physician in her area but her condition did not improve. Then, she was referred to tertiary care hospital for further management.On general physical examination, she was ill-looking and lethargic. Her pulse was 90 beats/minute; blood pressure 90/60 mmHg; respiratory rate 20 breaths/minute; afebrile. There were no signs of jaundice, cyanosis, clubbing or koilonychia. On abdominal examination, her abdomen was soft, mildly tender with audible gut sounds. The digital rectal examination was performed which revealed friable, easily prolapsed and bloodstained multiple pedunculated polypoid masses with normal anal sphincter tone and anal canal Figure . All othThe full blood count showed hypochromic microcytic anemia with hemoglobin of 10 g/dl, packed cell volume of 28.6% and a mean corpuscular volume of 66.8 fl. The white blood cells count indicated neutrophilic leukocytosis of total leukocytes 16.7x109/L and neutrophils 79%. Other laboratory tests were within normal ranges. The chest X-ray and ultrasound scan of the abdomen were normal. Small multiple erosions in fundus were found on upper gastrointestinal tract endoscopy while the rest was unremarkable. Colonoscopy illustrated hundreds of pedunculated polyps throughout the colon. Double contrast barium enema was performed which demonstrated various filling defects, scattered diffusely in the large bowel up to the rectum Figures .Contrast computed tomography (CT) scan of abdomen depicted distended large bowel which was studded with numerous, pedunculated and broad-based soft tissue density filling defects of variable sizes throughout the transverse colon, descending colon, sigmoid colon, and rectum; largest of them found in the mid rectum was measured 2.3x2.07cm. A large, irregular, heterogeneously enhancing polypoidal like mass lesion was seen at the hepatic flexure, inferiorly extending up to the ascending colon and caecum, measuring up to 3.9x4.9x3.0 cm (CCxTVxAP dimensions), also causing colo-colic intussusception. Multiple enlarged solid enhancing lymph nodes were found and the largest of them measured 1.3x1.0 cm at the ileocolic region. Histopathology report confirmed benign juvenile polyposis, showing a number of polyps with cystically dilated, hyperplastic glands, lined by tall columnar epithelium with basally placed nuclei. Some glands were filled with fibrin and intervening stroma was inflamed and showed congested blood vessels. Most of the polyps were covered by granulation tissue but indicated no evidence of dysplasia or malignancy.Genetic testing was unavailable at our hospital. A pan-proctocolectomy with ileoanal J pouch anastomosis was planned. After consent and counseling on long-term risk of colorectal cancer associated with polyposis; exploratory laparotomy was performed and numerous polyps in colon and rectum were found. The entire colon and enlarged lymph nodes were resected Figure  and ileoJPS is one of the hamartomatous polyposis syndromes usually associated with mutations in either of the two genes namely BMPR1A and SMAD4, linked to the TGF-B/BMP signaling pathway . It may JPS is diagnosed when a patient has any one of these findings (Jass criteria): polyps in colon or rectum greater than five in number, juvenile polyps throughout the gastrointestinal tract or any number of juvenile polyps found in one or more family members -6. In thIn this case, the largest polyp was measured about 2.3x2.07 cm in the mid-rectal region. In similar studies, it was proposed that polyps within the size of 1-2.9 cm have mildly dilated glands and moderately dysplastic epithelium with further sequential changes may progress into an adenoma leading to the formation of adenocarcinoma . On radiOur patient belonged to the rural side of the country where the likelihood of survival and treatment from any rare or complicated disease is minimal. It is a case originating from an ineffectual and resource-limited setting. Due to these constraints and delayed presentation; the cases subsequently culminate poorly for the patients with low chances of survival or compromised quality of life. A case of a 7-year-old boy reported in India, with progressive rectal prolapse and bleeding for five months and was treated with total proctocolectomy with ileoanal anastomosis. However, due to the presentation in a well-established and efficient environment, the patient was found to be doing well on a two-year follow-up after his surgery .The treatment of choice in the symptomatic patients includes surgical colectomy and ileorectal anastomosis including surveillance endoscopy of the remaining rectum . HoweverDespite digital rectal examination being of great value to assess such cases, the unavailability of important diagnostic tools like colonoscopy, CT scan in rural areas delays the whole process of management. Hence, measures need to be taken in order to provide better chances of survival to this part of the population. With a better educational background, awareness, and resources, polyps and early stage cancer can be identified and removed.


Indolent B cell lymphomas are a group of lymphoid malignancies characterized by their potential to undergo histologic transformation to aggressive lymphomas. While different subtypes of indolent B cell lymphomas demonstrate specific clinical and imaging features, histologic transformation can be suspected on cross-sectional imaging when disproportionate lymph node enlargement or new focal lesions in extranodal organs are seen. On PET/CT, transformed indolent lymphoma may show new or increased nodal FDG avidity or new FDG-avid lesions in different organs. In this article, we will (1) review the imaging features of different subtypes of indolent B cell lymphomas, (2) discuss the imaging features of histologic transformation, and (3) propose a diagnostic algorithm for transformed indolent lymphoma. The purpose of this review is to familiarize radiologists with the spectrum of clinical and imaging features of indolent B cell lymphomas and to define the role of imaging in raising concern for transformation and in guiding biopsy for confirmation. Indolent lymphomas can undergo histologic transformation to aggressive lymphomasImaging findings of indolent and transformed lymphomas are presentedImaging can raise concern for transformation and guide biopsy for confirmationAn algorithm for management of transformed indolent lymphoma is proposedLymphoma encompasses a heterogeneous group of lymphoid malignancies accounting for 4% of all cancers diagnosed in USA, with around 20,000 estimated deaths in 2016 . The terIndolent lymphomas may undergo histologic transformation (HT), which is defined as evolution from indolent, low-grade lymphoma to aggressive, high-grade lymphoma, by means of genetic mutations with corresponding modifications in histologic architecture, clinical behavior, and prognosis of the disease , 10. CliIn this paper, after a brief introduction on indolent lymphomas, we will present the clinical and imaging features of most common subtypes of indolent lymphomas, we will discuss the imaging features of HT for the different subtypes of indolent lymphoma and its differential diagnosis, and eventually we will propose a imaging algorithm for diagnosis and management of TIL, so to provide the radiologist with the appropriate clinical tools to recognize indolent lymphoma from diagnosis to transformation.Indolent lymphomas are characterized by a long course of disease, with death occurring years after diagnosis . DiagnosAfter a variable number of years of follow-up, generally with transient and incomplete response to therapy, death may occur due to disease progression, histologic transformation, or for unrelated causes , 18, 22.Management and treatment of the first two events, progression and HT, is challenging, as these events are associated with high death rates . GeneralThe most common subtypes of indolent lymphomas undergoing HT are FL, chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), marginal zone lymphomas (MZL), Waldenstrom macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL), and nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) . These sMycosis fungoides, a rare indolent T cell lymphoma which can transform to large T cell lymphoma, presents mostly with cutaneous lesions, will not be reviewed in this paper , 26.Follicular lymphoma is the most common type of indolent B cell non-Hodgkin lymphoma (NHL), originating from centroblasts and centrocytes of germinal centers of the lymph nodes, the spleen, or the bone marrow and is characterized by several genetic mutations including the BCL2 translocation , 27, 28.On cross-sectional imaging, FL presents with multiple, deep, non-contiguous enlarged lymph nodes, homogeneously enhancing on CT or MR Fig.  29]. In. In29]. On PET/CT, FL is reported as FDG avid in 91–100% of cases, in general with low avidity, depending on the histologic grade of FL , 32–34. Chronic lymphocytic leukemia/small lymphocytic lymphoma represent a spectrum of disease ranging from a pure bone marrow and blood disease (CLL) to pure extramedullary disease (SLL), in which small mutated small lymphocytes undergo uncontrolled proliferation. When HT occurs in CLL, it is termed Richter transformation. This occurs with a 0.5–1%-year rate, with 16% probability of transformation at 10 years .Imaging findings of CLL/SLL include adenopathy  splenomegaly, hepatomegaly, and various degrees of bone marrow infiltration Fig.  36–39].–39.36–39Marginal zone lymphoma represents a subset of lymphoma arising from the marginal zone of the secondary follicle . Accordiadnexa oculi) shows homogeneously enhancing T2-hyperintense or hypoattenuating soft tissue infiltrating the adnexa on CT or MRI. MALT lymphoma of the lung shows nodules, reticulations, consolidations, and peribronchial infiltrates [Regarding imaging features, these depend on the location and subtype of MZL. MALT lymphoma of the ocular appendages  is a B cell neoplasm in which malignant lymphocytes share morphologic characteristics with mature plasma cells . When anCross-sectional imaging findings include bone marrow involvement, adenopathy, extranodal involvement, or splenic lesions or splenomegaly Fig.  10, 51,,51, 52. Nodular lymphocyte-predominant Hodgkin lymphoma is a rare subtype of HL characterized by the presence of scattered large hystiocytic and lymphocytic cells often referred as “popcorn” cells due to the multi-lobated or folded appearance of the nucleus . Its cliCT reveals adenopathy, more commonly supradiaphragmatic, in particular axillary or cervical, and spleen involvement , 63. MRIHistologic transformation of lymphoma can occur in lymph nodes, the spleen, or in extranodal locations , 65–67. Extranodal transformation may manifest with new focal lesions in various organs, which can present as hypodense homogeneous lesions in solid organs on CT, or in case of hollow viscera, new or increased wall thickening, such as bowel or ureteral wall thickening Fig. . MRI canIn addition, it is worth mentioning that since indolent lymphomas are composed of multiple subpopulations with distinct mutations, multiple transformations can occur in the same patient with low-grade disease, sometimes either simultaneously or sequentially .Regarding different types of TIL, most studies focus on FL, showing that in patients with FL and clinical signs of HT, PET/CT has proven useful to identify transformed lymph nodes in patients with clinical signs and symptoms of HT, with SUVmax of transformed lymph nodes significantly higher than for nontransformed lymph nodes , 66. DueDifferentiating progression of indolent lymphoma from TIL can be challenging, especially in the case of progression from low- to high-grade indolent lymphoma, as there is wide overlap between clinical, histologic, and molecular characteristics of progressed and transformed lymphoma –74. On iDue to the high recurrence rate of indolent lymphomas, the possibility of recurrence of disease after initial response should be always considered when patients present with increased adenopathy or increased extranodal disease on restaging scans. To differentiate recurrence from HT, the clinical presentation can be helpful: elevated LDH, new B or systemic symptoms are most likely associated with HT . On imagPatients with lymphoma are inherently at risk of secondary tumors due to the status of immunosuppression, and patients with history of cancer can develop lymphomas. Differentiating HT from tumor progression can be difficult, especially in cases of tumors which tend to metastasize to lymph nodes. Specific imaging features of the primary cancer, as well as concordance between primary tumor growth and nodal progression, or presence of new sites of metastasis, can be helpful to differentiate tumor progression from HT Fig. .Fig. 8A An imaging algorithm for management of suspected histologic transformation is presented in Fig. Patients with HT could present to the radiologist in two ways: as asymptomatic patients undergoing surveillance for lymphoma or CLL with evidence of HT on imaging, or patients with clinical signs or symptoms suspicious for HT, including systemic signs, such as fatigue, infections or bleeding, “B” symptoms , adenopathy or organomegaly, and increased LDH or hypercalcemia.Asymptomatic patients are generally imaged with CT or PET/CT. Symptomatic patients can undergo PET/CT if the primary lymphoma is known to be FDG avid; CT if the disease is not FDG avid, to evaluate imaging characteristic of nodal disease or extranodal involvement of disease or in cases of suspected emergent conditions . MRI should be reserved in cases of potential extranodal involvement, such as bone marrow, liver, kidney, central nervous system, or soft tissue involvement. Ultrasound may be useful to study morphology and sonographic characteristics of superficial lymph nodes, as a first line imaging method to study focal extranodal involvement or to guide biopsy Fig. . Once PEIn every case and for every imaging modality, recurrent disease and development of secondary tumors should be ruled out, carefully evaluating the presence of concordant increase in nodal size or FDG avidity, extranodal involvement such as breast lesions and lung nodules. In addition, if HT is suspected on imaging, potential site of HT and approach for biopsy should be described when appropriate. Other potential complications should also be looked for and reported, including splenomegaly with impending splenic rupture, bowel obstruction, hydronephrosis, fractures, and bile duct dilation. It should be noted, however, that the likelihood of TIL in asymptomatic patients is low. Therefore, caution is necessary when raising concern of HT in these cases, as it may expose patients to unnecessary interventions and excess risks . FinallyHistologic transformation represents a critical point in the natural history of indolent lymphoma, with dramatic changes in patient prognosis and treatment. Knowing the most common types of indolent lymphoma and the natural history, biology, clinical, and imaging presentation of HT will help radiologists understand their role in patient management. Radiologists should be able to recognize signs of transformation, to identify a site for potential biopsy and to recognize mimickers or complications of HT, with an impact for patient prognosis.


Most of the colonic submucosal mesenchymal polyps are benign tumors. They are formed by more than one type of mesenchymal tissue. The diagnosis of angiolipofibroma depends on the histological findings with the proliferation of vascular, fatty, and fibrous connective tissues. Majority of angiolipofibromas are arising in the kidney and have rare presentation as extra renal region. Here we report a case of 58-year-old female patient with angiolipofibroma of sigmoid colon (8.5 cm x 4 cm size) which was treated successfully with surgical resection. The clinical presentation, operative procedure, pathological features, and medical literature review are presented. Most of the colonic polyps are originated in the mucosa and a small subset of them originate from the submucosal mesenchymal. The most common types of submucosal polyp are composed of single type tissue include lipoma, leiomyoma, neurofibroma or mucosal Schwann cell hamartomas, vascular lesion, and gastrointestinal stromal tumor (GISTs) . The mixThe aim of this report is to present an unusual case of colonic polyp originating from submucosal with mixed mesenchymal composed of vascular, mature fatty, and fibrous connective tissues.A 58-year-old female patient presented with one episode of rounded mass protruded from anus during defecation and reduced manually by herself. She was previously healthy person and had normal bowel motion. She had no history of abdominal pain or mass, bleeding per rectum, and weight loss. There was no family history of colonic polyps or colon cancer. On physical examination, there was no significant anemia, no palpable abdominal mass nor tenderness. Stool examination was positive for occult blood. Her hemoglobin was mildly reduced 11.6 gm/dl with hypochromic microcystic type of anemia and a serum carcinoembryonic antigen level (0.32 ng/ml) was normal. Other laboratory tests like blood sugar, renal profiles, and liver profiles were within normal ranges.A full colonoscopy was performed. A large polyp about 8 cm with rather smooth surface but highly vascular was found at the sigmoid colon, 14 cm from anal verge. The mass was occupied two third circumference of whole colonic lumen . The oriIn laparotomy, a large polypoid lesion about 8 x 3 cm with short stalk was found in sigmoid colon. The serosal surface appeared normal and no adjacent intra-abdominal lymph nodes were enlarged. Affected portion of sigmoid colon was resected and sent for histology examination . No renaMacroscopic examination of the excised specimen showed a polypoid growth measured 14 x 9 x 5 cm, of soft consistency, covered by grayish white fleshy mucosa. The luminal surface of polypoidal growth was measured 8.5 x 4 cm. The mucosa surface appeared normal. No hemorrhagic or necrosis was seen .Histological finding was large pedunculated polyp arising from the wall and composed of mature adipocytes and proliferating large and small vessels of varying nature  lined by a thin wall endothelial lining. No vasculitis or thrombosis was noted. There also proliferated smooth muscle spindle cells with eosinophilic cytoplasm embedded in loose myxoid stroma. Histological diagnosis was benign angiolipofibroma polyp, sigmoid colon .Colonoscopy is the gold standard procedure for colonic screening of colorectal cancer or polyps . In thisThe majority of angiolipofibromas are benign tumors derived from mesenchymal tissue and majorities are arising in the kidney. Extra renal presentations are very rare and reported to be found in the liver, nasal cavity, vagina, spermatic cord, skin, mediastinum, and GI tract. Among GI tract involvement, colonic involvement is rather rare condition .Angiolipofibroma may be a challenging in diagnosis when it arises outside its typical renal location because of its rarity and the histological variation. Typically, angiolipofibroma shows a mixture of adipose tissue, fat, and abnormal vessels, but it may show a wide range of histological appearances. These include tumors where the muscle component is dominant, including epithelioid variants band showing considerable nuclear atypia. Preoperative diagnosis of angiolipofibroma is difficult .Demir et al. described a similar lesion originating in the mesocolon of the sigmoid, composed of vascular proliferation in a fibrofatty tissue background; it was published in the name of “angio lipomatous mesenchymal hamartoma” . GroismaOnly few cases of colonic angiolipofibroma have been reported in the literature –11. MostAs the recurrence of colonic polyps, it is known that adenoma recurrence rates are estimated to be around 30-40%, 3 to 4 years after the initial colonoscopy , 13. TheThis submucosal mesenchymal, angiolipofibroma polyp case was reported on left side of the colon as the common location but differed from others, which was larger polyp (8.5 x 4 cm) with short or no stalk not favored to be removed endoscopically. Surgical excision is the treatment of choice for large lesion.


T1 and T2 weightedimages with thin rim enhancement, resembling an atypical meningioma. CT angiogram showed no vascular connection. Following worsening diplopia and a slight increase in lesion size on follow-up MRI, the patient was re-reviewed in our regional skull base multidisciplinary team meeting, where a decision for excision was made. Pre-operatively, the absence of a vascular connection was confirmed on catheter angiogram. Histopathological examination demonstrated features typical of calcified pseudoneoplasm of the neuraxis, with extensive metaplastic calcification with stroma containing variable fibrovascular tissue and focal inflammatory cell infiltrates, spindle and epithelioid cells, and psammoma bodies at the rim of the lesion. Following surgery, the patient had persisting diplopia. He remains under clinical review. As surgical resection is considered curative, no further imaging follow-up is planned.An 18-year-old part-time teacher presented with headache and diplopia. Physical examination showed partial left oculomotor palsy. Neurology examination was otherwise unremarkable. Cross-sectional imaging was arranged for investigation of third nerve palsy. On CT scan, the lesion was calcified, and on MRI, hypointense on  An 18-year-old part-time teacher presented with headache and diplopia. Headache was intermittent and side-locked, affecting the left temporal region, lasting up to 4 h at a time over a 2-year period, with a gradual worsening in frequency and severity and accompanied by new intermittent diplopia. The headache was refractory to analgesics. There were no other cranial nerve symptoms. Physical examination showed a partial left oculomotor palsy with partial limitation of downgaze, adduction of the eye and sluggish left pupillary response. There was no ptosis. Neurological examination was otherwise unremarkable.T1 and T2 hypointense mass in the expected location of the proximal left third nerve, between the P1 segment of the left posterior cerebral and the left superior cerebellar arteries  was obtained from the patient for publication of this case report.


Encrusted cystitis (EC) was first described as chronic cystitis with mucosal calcification in 1914 -4. Etiol We report a case of a 40-years old female with a history of transurethral bladder tumor resection (TUR-BT) in 2012. She was admitted to an outpatient clinic elsewhere in January 2015 with pelvic pain and recurrent urinary tract infection for the past 4 years. Diagnostic cystoscopy elsewhere revealed stone like particles covering the bladder mucosa. Later on, several TUR-BTs had been performed to remove these lesions in various hospitals elsewhere and histopathology reports revealed non-specific chronic cystitis without tumor. Patient had a re-TUR-BT in March 2015 again elsewhere for suspicious tumor, macroscopic hematuria and voiding stone like particles in urine. A necrotic bladder mucosa containing calcified encrustations with underlying inflammatory polymorphonuclear cell infiltration with abundant blood vessels was observed. Pathology result for this TUR-BT was encrusted cystitis (EC) . Patientth month of follow-up. At 18th month follow-up she was free from any complaint and infection with a remarkably increased functional bladder capacity of 340 mL.Patient was admitted to our clinic with severe pain, gross hematuria, and voiding stone like particles, and she had lower urinary tract symptoms (LUTS) reminiscent of interstitial cystitis. Urinalysis showed alkaline urine with struvite crystals in the sediment. Her functional bladder capacity was 75 mL. Urine culture was sterile. An irregularity on the right bladder wall, and a moderate hydro-nephrosis at the right side was revealed with ultrasound. Filling defect at the dome and right bladder wall was also seen in magnetic resonance study . A diagnUrea splitting bacteria play a role in the EC etiology. A bacterial culture is essential for the bacterial confirmation of the condition. Though sterile urine culture should not rule out the diagnosis of EC, prolonged culture or tissue cultures should be performed. A prolonged 48 to 90 hours 5% carbon dioxide or sheep blood agar urine culture may be helpful when EC is suspected in the presence of sterile pyuria , 7.Risk factors such as intravesical chemotherapy or BCG installations, urinary trauma, or bacterial urease activity results in ammonia release, which in turn damages the glycosaminoglycan layer of the bladder mucosa . As a coHaving a positive urine culture for C urealyticum does not necessarily means that patient has calcified encrusted particles in the urothelial mucosa. EC incidence was reported as 15.6% n=18/115) for patients with positive C urealyticum urine culture (/115 for In our present case, the patient had a TUR-BT history, and she presented with LUTS, gross hematuria and micturition of sandy grits. Urine culture was sterile, and ultrasound revealed stone like particles. Patient had a re-TUR-BT and part of the material and urine from bladder barbotage was sent for C urealyticum culture, which was positive. Treatment with teicoplanin 400 mg/day, and weekly intravesical DMSO was started.C urealyticum is the most commonly reported cause of EC, and it has multiple antibiotic resistance . TeicoplTo the best of our knowledge, DMSO has never been reported as treatment agent for encrusted cystitis. DMSO does not act as an acid or base with a pKa=35. It is an important polar aprotic solvent that dissolves both polar and non-polar compounds. It is miscible in a wide range of organic solvents as well as water. DMSO is a FDA approved drug mainly used in interstitial cystitis/painful bladder syndrome. DMSO plays a role in replenishing the damaged glycosaminoglycan layer and it has anti-inflammatory activity on injured bladder urothelium . The preIn conclusion, rare pathologies such as EC are limited to case reports and case series. Although urine acidification has been reported to be a component of the treatment, bladder irrigation with an aprotic solvent was effective in our case, and has not been reported previously. Further studies are needed to confirm our treatment effect and results.


Pseudomyxoma extraperitonei (PE) is a rare finding. The most common cause is the rupture of a mucocele of the appendix into the retroperitoneum.Here we report a case of a 52 years old female patient with a mass in the right abdomen and vague lower abdominal pain underwent resection of a extraperitoneal encapsulated mass. The histopathological examination revealed a mucinous pseudomyxoma with a low grade of differentiation.We report a case of pseudomyxoma extraperitonei with a review of literature.The treatment of pseudomyxoma differs substantially depending on whether it is intraperitoneal or extraperitoneal. The risk of recurrence is such that follow-up, based on a physical examination, CT scan and serum markers, is essential. The only remarkable event in her past history was a right breast fibroid neoplasm that had been removed 10 years before.2An abdominal examination revealed a large, fixed mass in the right subcostal region. There were no cervical, axillary or inguinal lymphadenopathies, nor any signs of ascites. Other tests were normal with the exception of a slight increase in the CEA level . Computed tomography (CT) of the chest and abdomen revealed a roundish, 100 × 70 × 80-mm mass in the right flank, with regular margins, a partial capsule and a 19-mm internal calcification with mural calcification and hyperdense central striae . The US-3The case was discussed at a multidisciplinary team meeting. As it proved difficult to make a preoperative diagnosis, the patient underwent surgery, during which a peritoneal encapsulated mass that was free of all surrounding structures, including the pancreas, kidneys and bowel, was found. This mass was resected completely without breaking the capsule . During 4Werth  first deThe treatment of pseudomyxoma differs substantially depending on whether it is intraperitoneal or extraperitoneal. For intraperitoneal pseudomyxoma, Sugarbaker et al.  recommenThe risk of recurrence is such that follow-up, based on a physical examination, CT scan and serum markers, is essential. Combined treatment in pseudomyxoma retroperitonei is associated with a 20-year survival rate in up to 70% of patients , whereasThe authors disclose no conflicts.The Authors disclose no sources of funding for research.This is a case report and review of literature. It’s exempt from ethical approval.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 requestG.T. Capolupo: study design, data collections, data analysis, and writing.G. Mascianà: study design, data collections, data analysis, and writing.F. Carannante: study design, data collections, data analysis, and writing.M. Caricato: reviewer.This is not a human study.Prof. Marco Caricato.Not commissioned, externally peer-reviewed.


Accessory spleen (AS) is a condition found in about 20% of patients and is most commonly located in the hilar region of the spleen. It is more often asymptomatic, single, and smaller than 2 cm. In the present study, we report the rare case of a 13-year-old boy with giant accessory spleen underwent spontaneous intrasplenic hemorrhage who presented with recurrent abdominal pain. Contrast-enhanced MRI was mandatory for the diagnosis before surgical procedure. An accessory spleen (AS) or splenunculus is a congenital anomaly consisting of the ectopic normal splenic tissue separated from the main body of the spleen  and mostA 13-year-old boy presented to pediatric surgery unit due to abdominal pain in the left abdomen without fever, nausea, vomiting, or genitourinary symptoms. He had no history of trauma or injury or operative treatment. His family history revealed no significant disease. On admission, the patient presented blood pressure 120/80 mmHg and 80 bpm, and all laboratory data were within normal limits. Examination of the abdomen showed pain in the left abdomen but negative McBurney or Bloomberg signs. Plain Rx of the abdomen did not show signs of intestinal obstruction or perforation. Abdominal US showed a mass 7 cm in diameter, located anteriorly and inferiorly to the spleen with similar structure. Abdominal MRI with contrast confirmed previous finding and, in addition, showed an area of intracapsular hemorrhage in the mass (Figures 1b-value DWI accessory, the splenic tissue shows marked hyperintensity [An accessory spleen, in other words supernumerary spleens, splenunculi, or splenules, is a congenitally duplicated splenic tissue that is separated from the main body of the spleen and is observed in about 10%–30% of patients in autopsy series and 16% of patients undergoing contrast-enhanced abdominal CT . The mosntensity . Scintigntensity . Howeverntensity . Treatmentensity . In our ntensity . The ope


Recurrences after radical esophagectomy are common. The prognosis for recurrent esophageal cancer is generally poor. Recurrences usually occur between 1 and 3 years of surgery, with the duration of median survival after recurrence ranging from 5 to 10 months. The number of sites and involved organs vary among patients. Consequently, a standard therapeutic strategy has not been established, and the role of surgery in the management of recurrence is unclear.A 67-year-old man presented with dysphagia 6 months previously and was diagnosed with esophageal squamous cell carcinoma (ESCC) in the upper thoracic region , for which he underwent thoracoscopy-assisted esophagectomy and lymphadenectomy. Adjuvant chemotherapy was not prescribed. Three years after the operation, he developed a solitary metastasis in the left lung, requiring segmentectomy followed by chemotherapy with combined cisplatin (CDDP) and 5-fluorouracil (5-FU). The following year, a metastatic lesion was recognized in the right lung, invading the chest wall, for which he underwent partial lobectomy with local chest wall resection. Multiple mediastinal and abdominal lymph node (LN) metastases were detected in the right lung a year later, which necessitated chemoradiation to a dose of 50.4 Gy with concomitant CDDP and 5-FU. Post-treatment computed tomography (CT) showed a good response. Positron emission tomography (PET)-CT revealed a reduction in the metastatic LNs with no fluoro-deoxy-glucose (FDG) uptake. The following year, metastases were detected in the left cervical LNs. Owing to the limited extent of metastases, resection was followed by chemoradiation to a dose of 50 Gy with CDDP and 5-FU. The following year, metastases were detected in the mediastinal LNs; chemotherapy was administered with nedaplatin and docetaxel. The follow-up CT and PET-CT demonstrated complete disappearance of the tumor, and the patient is currently surviving without recurrence for 11 years from the first curative operation.This case demonstrates that aggressive multidisciplinary treatment including surgery and radiation to achieve local control could be a meaningful treatment strategy in cases with limited and slowly occurring recurrences. Esophageal carcinoma is the sixth leading cause of cancer-related deaths in Japan . AdvanceWe had previously reported that the recurrence rate at 1 and 2 years after surgery was 71% and 84%, respectively –13. In pHere, we report a rare case surviving for 11 years after successful control of limited and slowly repetitive recurrences of esophageal squamous cell carcinoma. This was achieved with multidisciplinary treatment including repeated metastasectomies and chemoradiation.A 67-year-old man was admitted to our hospital with a history of dysphagia for 6 months. Upper gastrointestinal fiber endoscopy revealed a thoracic esophageal lesion. On histopathology, the biopsy specimen of the esophageal lesion revealed squamous cell carcinoma (SCC). Esophagography showed a localized lesion in the upper middle thoracic esophagus Fig. , and PETAt 3 years after surgery, a solitary metastatic lesion was detected by CT and PET-CT in the upper lobe of the left lung Fig. . A segmeThe year after the lung surgery, a metastatic lesion was detected on CT and PET-CT in the upper lobe of the right lung, invading the chest wall Fig. . This waThe year after right lobectomy, follow-up CT revealed multiple LN metastases in the mediastinum, right supraclavicular region, right axilla, and abdomen Fig. ; PET-CT Locoregional LN metastases in the left cervical region were detected in the year after chemoradiation Fig. . As the The fifth recurrence occurred in the LNs of the mediastinum and right hilum, a year after the cervical nodes were treated Fig. . ChemothAs shown in Fig. Here, we reported a case where long-term survival was accomplished despite multiple recurrences of ESCC. Various clinical patterns of recurrence are observed in ESCC. However, only a few trials have studied post-recurrence treatments on a large scale. There are therefore no recommended or standardized guidelines for treatment in these clinical situations .In the present case, the resected specimen of primary ESCC revealed well-to-moderately differentiated squamous cell carcinoma on histopathology. According to the Japanese Classification of esophageal cancer, the tumor had an infiltrative growth pattern of type b, with lymphatic (ly) 2, and venous invasion (v) 1. Tumor diameter (> 4 cm), poor differentiation, and pN3-4 have been reported as prognostic factors in ESCC . Other rAs explained, the first recurrence was observed in the periphery of the left lung at 3 years after the first operation. This progression was relatively slower than that reported in previous reports , 9, 20. Unlike in lung metastases, patients with metastases to the locoregional LNs have been reported to achieve longer survival with multidisciplinary treatment, which included resection of the involved nodes , 22, 23.Past reports have discussed the efficacy of multidisciplinary treatment for recurrences after curative surgery in esophageal cancer. Iitaka et al. reported on the efficacy of multimodal treatment in a case of ESCC with multiple recurrent lesions . Their pThe patient outcomes of the cases in which intensive or local treatments are particularly effective is a matter of interest. A report by Ghaly et al. described a case where definitive therapy was offered for an isolated recurrence after esophagectomy . They stThe interval between each recurrence plays an important role in deciding the therapeutic strategy. To date, only a few studies have investigated the interval between each recurrence, as long-term survival after repeated surgeries or other forms of definitive therapy in cases of recurrent esophageal cancer, is rare. In the present case, the first recurrence was recognized in the third year after primary resection. However, the interval between each of the treated recurrences was within 1 year. Recurrences occurring within 1 year after surgery are common in clinical practice, but in this case, each recurrent lesion was solitary or locoregional, which allowed intensive multidisciplinary treatment.In summary, we speculate that intensive and multimodality treatment is likely to be particularly effective if the recurrent lesion is solitary or locoregional, and slowly progressive; in cases selected for resection, surgery is particularly effective if the tumor is easy to approach surgically .We present a case of long-term survival with multidisciplinary treatment for multiple sequential recurrences in ESCC after curative esophagectomy. Despite the poor prognosis of recurrent esophageal cancer, this case demonstrates that multidisciplinary management, including aggressive local therapies, can be particularly effective in cases with localized recurrences, appearing gradually. Cumulative accounts of similar cases, with detailed analyses, are necessary to establish the optimal treatment strategy for recurrences in ESCC.