A 67-year old man with a history of papillary thyroid cancer (PTC) presented with metastatic disease to the left colon in the form of a 6.1x1.0 cm bleeding, ulcerated mass. Radiopaque surgical clips were used as fiducial markers to localize the gross tumor volume (GTV) as well as the corresponding clinical target volume (CTV) and planning target volume (PTV). Daily cone beam computed tomography (CBCT) image guidance was utilized to verify the tumor position. Inter- and intrafraction movement of the tumor mass was assessed. Gastrointestinal bleeding was controlled using palliative image-guided radiation therapy (IGRT). Papillary thyroid cancer (PTC) is the most common form of well differentiated thyroid cancer and is usually associated with good prognosis . RecurreRadiation treatment of tumors of the colon is not typically performed as it presents a challenge to visualize and consistently target mobile abdominal organs. Treatment planning without adequate visualization of the target area can result in wide treatment margins and excessive toxicity. Movement of the intra-abdominal organs can result in underdosing of the target or overdosing of the surrounding organs. Furthermore, conventional CBCT techniques rely on bony matching, which does not necessarily correlate with internal organ motion. Use of fiducial markers, such as surgical clips, can narrow the treatment volume and improve radiation targeting. Previous studies demonstrate that use of surgical clips for targeting prostate cancer, results in a reduction in total setup error, smaller planning target volume (PTV) margin and rectal sparing . DespiteHere, we present a unique case of multi-site metastasis of papillary thyroid carcinoma including an ulcerated, bleeding colonic mass. Furthermore, we describe our experience with image-guided radiation therapy (IGRT) for palliative treatment to control bleeding of metastatic disease to the colon.A 67-year old man presented with an ulcerated, bleeding mass in the colon, shown by biopsy to be metastatic PTC in May 2014.He initially presented with a locally advanced thyroid tumour in 2005 treated with total thyroidectomy and bilateral neck dissection. Post-surgical pathology revealed the primary lesion to be classic papillary thyroid carcinoma with areas of follicular and focal oncocytic variants. There was evidence of capsular invasion, extra-thyroidal extension involving muscle, adherence to the trachea and involved neck nodes; tumour stage was pT4N2M0. The patient underwent adjuvant radioactive iodine, and external beam radiation to a dose of 60Gy over 30 fractions to the thyroid bed and neck.In June 2007, the patient developed locoregional recurrence which was treated with left neck dissection and radioactive iodine of 150 millicuries. In December 2009, chest imaging revealed pulmonary and mediastinal metastases which were treated with a third course of radioactive iodine of 157 millicuries. In January 2013, the patient presented with spinal metastases involving the T9, T11, and L2 vertebrae encroaching on the spinal canal. This was treated with radiation dose of 20Gy in 5 fractions to control neuropathy and bony pain.In June 2014, the patient presented with abdominal fullness and lower gastrointestinal bleeding. Abdominal and pelvic CT with contrast showed metastatic deposits throughout the abdomen and pelvis, with lymphadenopathy involving the gastrohepatic ligament, celiac axis, aortocaval and left external iliac regions. The patient was found to have a hemoglobin (HgB) level of 65 mg/L and was transfused a total of 4 units of packed red blood cells.Colonoscopy was performed; an actively bleeding 6.1 × 1.0 cm ulcerated lesion was dominant in the left colon. Biopsy of the colon mass was performed and the immunohistochemical profile was positive for TTF-1 and CK7, negative for CDX2, CK20 and HBME1. Immunohistochemistry and morphology were consistent with metastatic papillary thyroid cancer. Further surgical management of the mass was not deemed appropriate given the patient’s extent of disease and comorbidities. Additional radioactive iodine treatment was not recommended after considering the three previous doses and the low likelihood of targeting the colonic lesion. Therefore, the patient was planned for radiotherapy with the intent to stop bleeding from the colonic mass. A palliative dose of 20 Gy in 5 fractions was chosen to try to alleviate symptoms while limiting toxicity to the adjacent, unaffected organs at risk.3 to 251.5 cm3 while shifting the geometric center of the PTV by 13.7 cm  to ensure the PTV encompassed the three clips and CTV contour. Bony registration was first confirmed on preliminary CBCT and then, if required, any additional shifts were applied to ensure that all three clips were encompassed by the PTV volume. The three clips were well visualized by radiation therapy staff and centered within the PTV volume on each of the five treatment days.A review of each daily CBCT image and treatment shifts was performed to determine tumour motion using the surgical clips as a surrogate to define the GTV. The clips were contoured in the initial CT simulation scan and all subsequent CBCT images.Clip displacement  was assessed using Image Fusion from the Pinnacle 9.2 TPS. Cone beam CT images from each treatment day were fused with the initial planning CT for bony registration. Displacement was calculated by comparing the coordinates of the geometric centers of the contoured clip volume (i.e. the GTV) of the initial planning CT versus daily CBCTs. The average interfraction movement of the clips was 0.69 cm (ranging 0.51-0.98 cm) from initial planning  of the patient produced a 0.24 cm and 0.41 cm displacement of the target on days 4 and 5, respectively (Table The patient tolerated the palliative course of radiation treatment well with no gastrointestinal or genitourinary toxicity observed. Lower gastrointestinal bleeding stopped within 4 days of completing radiation therapy and post-treatment HgB level was 95 mg/L; no additional transfusions were required. At six-week follow-up, the patient did not have any recurrent lower GI bleeding and HgB had increased to 103 mg/L. At a later date, the patient was admitted to hospital with thromboembolic stroke and died from complications.Given the complicated and unfortunate course of disease for this patient, palliative management was recommended to control lower GI bleeding, avoid further transfusions, and improve quality of life. Rare abdominal and pelvic metastasis of papillary thyroid cancer presented a challenge for management, and only a handful of reports have been published -6. One rAbdominal organ and tumor movements are influenced by respiration and bladder filling status, leading to irregular changes in conformity . Abbas e3 is associated with >10% risk of grade 3 or higher acute toxicity [3 which would have exposed the patient to a high risk of severe acute small bowel toxicity[3, suggesting a potential decrease in the risk of severe toxicity.Irradiation of the abdomen and pelvis may cause acute toxicities including nausea, vomiting and diarrhea. For palliative dose regimens such as 20 Gy in 5 fractions the reported rates of nausea and/or vomiting range between 30-50% -12. Bioltoxicity -15. We r toxicity. The plaInter- and intrafractional tumor movement have been assessed with regards to lung, pancreatic, prostatic, vaginal and hepatic cancers, but not lesions in the colon -20. ReceHere we present a rare case of papillary thyroid cancer metastasis to the colon treated with a palliative dose of IGRT to control lower GI bleeding. Surgical clips were used as fiducial markers with a volumetric expansion of 1 cm for the CTV and 1 cm for the PTV. The main benefit of the fiducial markers in this case was accurate delineation of the GTV which allowed for smaller target volumes. Using daily CBCT, the interfraction and intrafraction clip movements were measured to be less than 1 cm which was within the PTV margin chosen for this case.This study demonstrates the clinical efficacy of palliative IGRT for a colon tumor using surgical clips as fiducial markers to enhance target localization and verify daily treatment position. The radiopaque surgical clips provide a reliable marker for visual verification of a mobile and otherwise imperceptible lesion.Informed consent was obtained from the patient and next of kin for publication of this case report and any accompanying images.


Ewing sarcoma of bone is classically a permeative lesion in the diaphysis of long bones in children. While they occur primarily in children and adolescents, they can be seen in young adults in their 20s, but these are typically seen in flat bones. The permeative nature of the lesion can elicit new bone formation creating a partially sclerotic appearance, cortical expansion presenting as a “Codman triangle,” or have an “onion-skin” type of aggressive periosteal reaction/periostitis. Ewing sarcoma is rarely seen without an associated soft-tissue mass and is even rarer to just have benign-appearing periostitis . We present such a case of Ewing sarcoma in a young adult confined to just the medullary metadiaphysis without cortical erosion or soft-tissue mass. To the best of our knowledge, this is the first case to be reported in the radiology literature. We present a 29-year-old postpartum female with a four-month history of right groin pain that occasionally radiated into the hip and anterior right thigh. The pain was intermittent but worsening. She decided on physical therapy but with no improvement. Her medication was limited to ibuprofen as she was still breastfeeding and the pain worsened with standing and lifting her baby. She denied history of trauma, fever, chills, night sweats, weight loss, or night pain. Plain radiographs of the right hip and femur were normal .She subsequently received an MRI of the right hip which revealed a large marrow-replacing lesion extending from the intertrochanteric region into the proximal diaphysis with surrounding edema in the bone and soft tissues . The patWith a working diagnosis of lymphoma, the fine-needle biopsy of the lesion instead revealed viable small round blue cells surrounding a central focus of necrosis  and smalThe patient underwent chemotherapy followed by surgical wide resection and prosthetic reconstruction. The resected gross specimen showed no residual viable tumor by microscopic examination .Ewing sarcoma (ES) is one entity of what is now considered to be a group termed Ewing sarcoma family tumors (ESFT), which includes peripheral primitive neuroectodermal tumors (PNETs) and Askin tumors . ES accoThe characteristic radiologic appearance of ES is a permeative osteolytic lesion involving the diaphysis or less commonly the metaphysis of a long bone but also occurs in the flat bones. The clinical course of the tumor when it is metaphyseal begins as an intramedullary lesion that causes marrow replacement and permeates through the cortical bone and extends into the adjacent soft tissues. Interestingly, the diaphyseal course also begins in the intramedullary canal but then permeates the cortex via haversian canals and vascular channels and extends into the surrounding soft tissue leaving behind the more characteristic periosteal, multilayered reactive bone formation commonly described as an “onion-skin” periosteal reaction . Our casOsseous lymphoma can be confused with Ewing sarcoma not only clinically but also pathologically. Ewing sarcomas microscopically appear as sheets of small blue round monomorphic cells with little intercellular stroma . SeveralIn summary, Ewing sarcoma is a very aggressive tumor with variable prognosis depending on stage, site, size, microscopic feature, and biologic features. Prognosis has improved thanks to modern chemotherapy advancements.


Struma ovarii is an uncommon ovarian teratoma comprised predominantly of mature thyroid tissue. The combination of pseudo-Meigs’ syndrome, and elevation of CA 125 to the struma ovarii is a rare condition that can mimic ovarian malignancy. We reported a case of benign struma ovarii, presenting with the clinical features of advanced ovarian carcinoma: complex pelvic mass, gross ascites, bilateral pleural effusion and markedly elevated serum CA 125 levels. The patient underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. Ascites and pleural effusion were not evident and the CA 125 levels returned to normal following surgical excision. A systematic review of reported cases of coexistent benign struma ovarii, pseudo-Meigs’ syndrome and elevated serum CA 125 was performed. Struma ovarii accompanied by pseudo-Meigs’ syndrome and elevated serum CA 125 should be considered in the differential diagnosis of ovarian epithelial cancer. Struma ovarii is a rare ovarian neoplasm consisting almost exclusively of mature thyroid tissue (>50%) derived from germ cells in a mature teratoma . Few of On April 3, 2014, a 52-year-old, Chinese female, premenopausal, gravida 3, para 1, was admitted to the United Hospital of Dezhou , complaining of oppression in chest and shortness of breath for 5 days. The patient’s previous menstrual period was March 31, 2014. The patient did not complain of any pain or changes in micturition or bowel movements. The patient’s medical history included surgery for an ovarian tumor 26 years previously and surgery for a broad ligament tumor 10 years previously. Non-enhanced CT imaging of the chest showed bilateral pleural effusions, particularly the right thoracic cavity. Marked ascites, and a large solid and cystic mass (65×56×69 mm) in the right ovary were detected by pelvic ultrasound. On April 8, 2014, the patient was subsequently transferred to the Department of Gynecology of Qilu Hospital, Shandong University .On admission, the patient was found to have ascites and bilateral pleural effusion. The gynecological examination revealed a mass in the right adnexal region with a normal-sized mobile uterus. Abdominal and pelvic ultrasound confirmed the presence of ascites and a large irregular, cyst-solid-mixed mass in the right ovary, ~75×56 mm in size. CT scan of the chest, abdomen, and pelvis revealed bilateral lung basal atelectasis with a large right pleural effusion, gross ascites, and a large loculated complex cystic pelvic mass. There was no evidence of enlarged lymph nodes. To alleviate symptoms and aid in the diagnosis, thoracentesis was performed to yield straw-colored fluid (800 ml) consistent with an exudative process. There was approximately 2,000 ml pleural effusion. Paracentesis yielded an exudate , found to be negative for malignant cells and mycobacterium tuberculosis. Cytological examination of the fluid revealed benign mesothelial cells and a few lymphocytes without malignant cells. The serum CA 125 level was 1,289 U/ml . The AFP and CEA levels were within normal range. Liver function tests were also within normal limits.The patient was arranged for an exploratory laparotomy through a vertical supraumbilical midline excision for diagnostic and therapeutic purposes. The patient was found to have ascites and 1,000 ml of straw-colored fluid was drained upon entrance to the peritoneal cavity. Extensive adhesions between posterior/left wall of uterus, left ovary, oviduct and intestinal canal as well as its surrounding tissues were identified. A 7×5 cm mixed cystic-solid neoplasm was found to arise from the right ovary. The left ovary, the two fallopian tubes, the uterus, diaphragm, bowel, and omentum appeared to be free of disease. There was no evidence of enlarged lymph nodes or metastatic lesions. The right tube and ovary were removed for frozen section and it was suggestive of cystic mature teratoma with a large component of thyroid. The patient and family member insisted on a hysterectomy and left salpingo-oophorectomy, which were performed.Thyroid function tests were not performed prior to surgery as the struma ovarii was not taken into consideration. Following the diagnosis, thyroid function tests were obtained on the third postoperative day. The results of the tests revealed normal thyroid function with serum FT3 levels at 2.05 pg/ml (1.8–4.6 pg/ml), FT4 levels at 17.79 pmol/l (12–22 pmol/l) and TSH levels at 2.9 uIU/ml (0.27–4.2 uIU/ml). Mild hypoalbuminemia was observed. On the sixth day post-operation the level of CA 125 was decreased to 609.6 U/ml. On the seventh day post-operation another chest CT was taken, examined and compared with the pre-operative one . The finStruma ovarii is an uncommon benign neoplasm of ovary that usually presents with asymptomatic mass and is difficult to diagnose prior to surgery. Ascitic fluid is identified in 20% of cases of struma ovarii . Struma et al  and a significantly elevated CA 125 level were highly suspicious for an ovarian malignancy. Struma ovarii accompanied by pseudo-Meigs’ syndrome and elevated serum CA 125 should be considered in the differential diagnosis of ovarian epithelial cancer.


Pancreatic tumors in pregnancy are rare but clinically challenging. Careful diagnostic workup, including appropriate imaging examinations, should be performed to evaluate surgery indications and timing . In the present case a diagnosis of an adult pancreatic hemangioma was made. We were not able to identify a similar case in the very sparse literature on this rare disease.A 30-year-old woman at 12 weeks of gestation was diagnosed with a large pancreatic tumor having a cystic pattern based on imaging. Although the preoperative diagnosis was uncertain, patient preference and clinical symptoms and signs suggested surgery. Open distal pancreatic resection including splenectomy was performed, and complete resection of the large cystic tumor was successfully achieved, with no postoperative complications. Although a solid pseudopapillary epithelial neoplasm (SPEN) was suspected, specimen morphology, including immunohistochemistry, supported the diagnosis of an adult benign pancreatic hemangioma.Although mucinous cystic neoplasm (MCN) and adenocarcinoma are the most common pancreatic tumors during pregnancy, various other malignant and benign lesions can be encountered. This report adds to the very small number of pancreatic hemangiomas reported in the literature and involves the first patient diagnosed with this rare condition during pregnancy. Careful clinical considerations regarding diagnostic workup and treatments are required to ensure that mother and child receive the best possible care. In pregnant women, the acute abdomen is often a challenge. Careful clinical evaluation, close cooperation between the surgeon and the gynecologist as needed, and the application of appropriate diagnostic tools and educated judgment remain the cornerstones of standard care –6. AlthoIn this report, we discuss clinical considerations related to the unexpected finding of a large cystic lesion that appears to be related to the pancreas in a pregnant woman with acute abdomen.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.th postoperative day without complications.An otherwise healthy, non-obese 30-year-old woman, para 0, at 12 weeks gestation was admitted to our hospital’s Department of Gastrointestinal Surgery with a history of 3 weeks of varying but increasing pain in the left upper quadrant of her abdomen and nausea without vomiting. A palpable left subcostal mass was detected by clinical examination, which revealed no other notable findings except for her pregnant status. Ultrasound (US) examination (Fig. The multicystic tumor Fig.  measuredAfter surgery, the patient’s pregnancy proceeded uneventfully, and she spontaneously delivered a healthy child at the calculated gestational time. During follow-up of at least 12 postoperative months, the patient has expressed no complaints or concerns related to her treatment.While hemangiomas are rarely found in the pancreas, they are very common in the liver. Mundinger et al. reviewed the literature and found only 9 cases with confirmed adult pancreatic hemangiomas reported between 1939 and 2009 . The lesOur patient was definitely in the younger range of age, and importantly, we report on the first pregnant patient diagnosed with an adult pancreas hemangioma. Although the final morphologic diagnosis of this rare condition can be challenging, CD 31 and CD 34 immunohistochemical labeling adds valuable support to the diagnosis of a neoplasm of vascular origin .Both benign and malignant pancreatic neoplasms are rarely diagnosed during pregnancy; in particular, pancreatic cancer during pregnancy is extremely rare, with fewer than 10 described cases to date , 16. HowDel Chiaro et al.  recentlyAlthough reports have described a number of anecdotes involving worrisome histories of pregnant women with malignant tumors, including carcinomas of the pancreas , 30, 31,


Primary squamous cell carcinoma (SCC) of the gallbladder is a rare malignancy of the gallbladder, accounting for less than 5% of gallbladder pathology. Initial presentation is often similar to adenocarcinoma of the gallbladder. SCC tends to be more locally aggressive, however, and possesses a worse prognosis than adenocarcinoma. We report a case of locally advanced SCC of the gallbladder. She underwent hematology/oncology evaluation to exclude a possible bone marrow dysplastic process. This did not demonstrate an underlying cause for the leukocytosis. She presented to a local emergency room in mid-June 2012 with complaints of chills, weakness, and malaise. She was admitted with Gram-negative rod bacteremia. A computed tomography (CT) scan was obtained which demonstrated an 8.6 cm mass of the right lobe of the liver with associated abscess .For those deemed unresectable or unable to undergo complete resection, the outcome is poor. Those with SCC of the gallbladder appear to have an even worse outcome than those with adenocarcinoma. Roa et al. reviewed 34 cases of squamous and adenosquamous carcinomas of the gallbladder . The surIn conclusion, SCC of the gallbladder is a rare disease process associated with a poor overall outcome. Patients often present with large, bulky tumors with involvement of adjacent organs. Lymph node involvement and distant metastasis are rare. Surgery is the mainstay of therapy and offers the only possibility for long-term survival. Unfortunately, many patients present with advanced stage disease, such as the one described, with limited treatment options.


Hepatic lymphangiomas, malformations of the liver lymphatic system, are extremely rare conditions in adults. A 41-year-old man presented with right upper abdominal pain for 6 months was introduced in this report. Ultrasound (US) and computed tomography (CT) scan demonstrated a giant cystictumor with a pedunculatedextrahepatic growth pattern. Due to diagnostic uncertainty, a partial hepatectomy was performed and pathological results confirmed the diagnosis of solitary hepatic lymphangioma. In this article, we reviewed the clinical and pathology features, preoperative diagnostic challenges, and treatments of hepaticlymphangiomas. Lymphangiomas are benign neoplasms regarded as congenital malformations of the lymphatic system  and Hepatitis B core antibody (HBcAb) were positive. Results of kidney function, electrolytes, and all tumor markers related to the liver were negative. Brain computed tomography (CT) scan and chest X-ray did not find other lesions in the patient.Abdominal ultrasound (US) showed a giant mixed-echoic mass (15 cm × 10 cm) with a pedunculatedextrahepatic growth pattern. An enhanced abdominal CT scan confirmed a giant cystic hepatic lesion with no capsule. The lesion was demonstrated with a heterogeneous enhancement in the arterial phase (Figure At laparotomy, a huge, smooth tumor was found in segment II with pedunculated extrahepatic growth, with no other pathology found in the abdomen. The tumor was completely removed bylocal resectionof segment II of the liver using an ultrasonic scalpel and cavitron ultrasonic surgical aspirator after occlusion of the left hemihepatic vascular.Histology of the resected specimen revealed a giant cystic mass about 16.2 cm × 13 cm × 4.3cmm in size. On macroscopic examination, the tumor was cystic and multilocular. The cyst had a thick, gray-white wall and was filled with serous fluid containing a small amount of blood. Microscopically, the tumor was composed of irregular and expanded lymphatic vessels, lined by flattened endothelial cells (Figure The postoperative course was uneventful and the patient has been followed up for 30 months. Now, he is symptom-free with no evidence of recurrence on subsequent abdominal imaging.Lymphangiomas are a group of benign tumors which are composed of lymphatic spaces lined by attenuated endothelium. Histologically, lymphangiomascan be divided into three groups: capillary, cavernous, and cystic lymphangioma (Asch et al. Hepatic lymphangiomas usually have non-specific clinical signs or symptoms (Matsumoto et al. In conclusion, we report a rare case of huge hepatic lymphangioma with pedunculatedextrahepatic growth in a 41-year-old male. Preoperative examinations were not sufficient to differentiate the nature of the tumor. The total resection of the tumor resolved all his symptoms, and the pathological finding revealed the true nature of the mass. The long-term prognosis following surgery is quite satisfactory.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.The corresponding author is the deputy head of the Society of hepatobiliary surgery, Branch Association of surgery, China Medical Association. Eastern Hepatobiliary Surgery Hospital is the biggest therapeutic centre of hepatobiliary surgery in China.


Round ligament of uterus leiomyoma is a rare, benign tumor of the vulva. Its incidence is not known exactly, and the mean age ranges from 13 to 70. Although clinical properties of benign and malignant diseases in the vulvar area are frequently similar, early diagnosis and treatment are essential. Local excision is recommended as definitive therapy. We present an 28-year-old female without any birth with a mass in anterior vaginal wall diagnosed as vulvar leiomyoma. In conclusion, a brief review of relevant literature emphasizes that leiomyomas are quite rare outside of the uterus but they might occur in any tissue or organ containing smooth muscle, spontaneously or parasitically after the spreading effect of an accident or surgical trauma. Clinicians should be alert especially for the diagnosis in a tissue with smooth muscle content. Tumors of the round ligament of uterus leiomyomas are benign, solid tumors of vulva which are seen rarely and usually they originate from smooth muscle fibers in ligamentum rotundum that extends to labia majora . There aA 28-year-old, nulligravid patient presented to our clinic with complaints of dyspareunia, dysuria, and a palpable vaginal mass. A solid, tender, irreducible mass protrudes from the anterior vaginal wall extending to left vaginal wall until the level of hymen was detected Figures . The solLeiomyoma of the uterus is common, but leiomyoma of round ligament is rare. Fifty percent of round ligament leiomyomas present with uterine fibroids . They arA brief review of relevant literature emphasizes that leiomyomas are quite rare outside of the uterus but they might occur in any tissue or organ containing smooth muscle, spontaneously or parasitically after the spreading effect of an accident or surgical trauma. Clinicians should be alert especially for the diagnosis in a tissue with smooth muscle content.


Non-Hodgkin lymphoma (NHL) is found in the adrenal gland secondarily at a rate of 25% . PrimaryA 62-year-old male admitted to our hospital with abdominal pain in the left lumbar region persisting for 4 months without B-symptoms. Abdominal and thorax computed tomography (CT) scanning was performed and revealed a mass of 93x60 mm on the left adrenal gland and a 58-mm mass on the right adrenal gland. The mass was nonfunctional according to hormone test results. The patient underwent left adrenalectomy. The pathology specimen revealed NHL, DLBCL, leukocyte common antigen (+), CD20 (+), CD3 (-) . In posiPAL are rare, generally occurring among patients of advanced age mean: 68 years) and dominantly in males M/F: 2.2/1) . C8 years aNonspecific clinical presentation and imaging results make it very hard to diagnose before surgery . In CT aPrognosis is usually poor. Poor prognostic factors are advanced age, large tumor size, bilateral involvement, high LDH levels, involvement of other organs, and adrenal insufficiency at admission ,3,5,7,8.Treatment includes surgery, combination chemotherapy and radiotherapy, but bilateral adrenalectomy with adjuvant radiotherapy is still controversial . CommonlIn conclusion, this rare disease should be kept in mind in patients with adrenal masses even in the absence of other malignancies, without nodal or extranodal involvement, or in patients with adrenal insufficiency.The authors of this paper have no conflicts of interest, including specific financial interests, relationships, and/ or affiliations relevant to the subject matter or materials included.


Metachronous bilateral testicular masses in adults should prompt consideration of adult presentation of CAH. Since all untreated CAH patients are expected to have elevated serum ACTH, formal exclusion of CAH prior to surgical resection of a testicular Leydig-like proliferation could be accomplished by screening for elevated serum ACTH.A 33-year-old male with a history of left testis Leydig cell tumor (LCT), 3-month status after left radical orchiectomy, presented with a rapidly enlarging (0.6 cm to 3.7 cm) right testicular mass. He underwent a right radical orchiectomy, sections interpreted as showing a similar Leydig cell-like oncocytic proliferation, with a differential diagnosis including metachronous bilateral LCT and metachronous bilateral testicular tumors associated with congenital adrenal hyperplasia  and “testicular tumors of the adrenogenital syndrome” (TTAGS)). Additional workup demonstrated a markedly elevated serum adrenocorticotropic hormone (ACTH) and elevated adrenal precursor steroid levels. He was diagnosed with congenital adrenal hyperplasia, 3 Testicular tumors in children and adults may be due to neoplasms or reactive hyperplasias. This paper considers the differential diagnosis of metachronous bilateral Leydig-like testicular tumors in an adult.Primary testicular neoplasms are diverse, broadly categorized into germ cell tumors  and sex cord/stromal tumors (Leydig cell tumors (LCT), Sertoli cell tumor, granulosa cell tumor, fibroma/thecoma, and undifferentiated tumors) . Sex cor CYP21 gene that encodes the 21α-hydroxylase enzyme, and 5–8% are associated with loss-of-function mutations in the CYP11B1 gene that encodes the 11β-hydroxylase enzyme [ CYP17A1 gene that encodes the 17α-hydroxylase enzyme and loss-of-function mutations in the HSD3β2 gene that encodes the 3β-hydroxysteroid dehydrogenase enzyme. Loss of function of these enzymes leads to decreased production of cortisol and other steroid hormones. Cortisol deficiency leads to increased hypothalamic secretion of corticotropin-releasing hormone (CRH) and increased anterior pituitary secretion of adrenocorticotropic hormone (ACTH).Congenital adrenal hyperplasia (CAH) is a set of autosomal recessive genetic disorders characterized by loss-of-function defects in enzymes involved in the production of cortisol, aldosterone, and sex hormones , 11. Roue enzyme , 12. LesIncreased serum ACTH in undertreated CAH causes hyperplasia of normal adrenal cortex bilaterally , hyperplTesticular hilar steroid cell hyperplastic nodules occur in up to 94% of CAH patients , 16. TreDifferent terms have been used to describe these testicular steroid cell hyperplasias, including “testicular tumors of the adrenogenital syndrome (TTAGS)” , 5, “tesComparison and contrast of the clinical, gross, micro, IHC, and endocrine features of LCTs and TARTs are summarized in β-hydroxysteroid dehydrogenase deficiency.Because TARTs frequently mimic Leydig cells cytologically, a large unifocal unilateral TART that displaces testicular parenchyma could easily be confused with an LCT in an adult in whom CAH is unrecognized. This diagnostic puzzle is less likely in a child, because precocious puberty would usually prompt an endocrine workup that would distinguish LCT from TART. This paper describes a case in which an adult presented with metachronous bilateral Leydig cell-like testicular tumors, prompting a workup for congenital adrenal hyperplasia (CAH), and was ultimately found to have CAH due to 3A 33-year-old male presented with a 2-month history of left testicular pain and swelling. He reported a history of hypospadias repair and bilateral cryptorchidism status after orchiopexy as a child. A scrotal ultrasound (US) revealed a heterogeneous 2.5 cm left testis mass and an incidental markedly heterogeneous 0.6 cm right testis nodule. A computed tomography scan of the chest and abdomen revealed no significant lymphadenopathy. He underwent left radical orchiectomy at an outside hospital, demonstrating a unifocal 3.5 × 3.0 × 2.2 cm LCT, without extratesticular invasion or vascular invasion. Nuclear atypia was seen, but no mitotic activity, coagulative tumor cell necrosis, vascular invasion, or Reinke crystals were noted. The Leydig-like proliferation was interpreted as a Leydig cell tumor, likely benign, staged as pT1.Two weeks after orchiectomy, he was referred to this institution for evaluation of a right testicular mass. He reported an unintentional 11-pound weight loss, night sweats, decreased libido, and erectile dysfunction. A repeat ultrasound of the right testis approximately 3.5 months after initial ultrasound revealed an increase in the size of the right testis nodule from 0.6 cm to 3.7 cm. No palpable mass was appreciated on initial or follow-up physical exams. The working clinical diagnosis at this juncture was a metachronous primary testicular malignancy. A metastatic workup was negative, and he underwent a right radical inguinal orchiectomy. Gross examination of the right testicular mass revealed a testicular hilar mass. Tissue sections showed nodules of oncocytic cells with low N : C ratios, prominent nucleoli, and focal lipofuscin, without Reinke crystals and without mitotic activity or coagulative necrosis . Immunosβ-hydroxysteroid dehydrogenase deficiency (3BHSD). He also had elevated renin activity supporting the salt-wasting variant of 3BHSD. His spontaneous puberty and late presentation point to a mild 3BHSD deficiency. He was referred to genetic testing but unfortunately was lost to follow-up. The findings of metachronous bilateral Leydig-like tumors associated with depressed cortisol and elevated serum ACTH are most consistent with bilateral TARTs in a patient with unrecognized CAH.He was referred to endocrinology for concern for congenital adrenal hyperplasia, and additional history revealed facial hair as an adolescent, an early growth spurt, frequent asthma flairs that responded to steroid dosing, and salt craving without a history of hypertension. In retrospect, he suffered nausea and vomiting after both surgical procedures. The first episode was associated with fever and resolved after administration of antibiotics. The episode after the second surgery resolved after changing to a different pain medication. He also reported a grandfather with a similar early puberty. After his first orchiectomy, he attempted sperm banking but was azoospermic. More directed physical exam revealed tan skin , pale striae on the abdomen, mild acanthosis nigricans of the neck, and multiple hyperpigmented chicken pox scars. Further testing demonstrated normal sodium levels, although there were no postoperative measurements, cortisol of 0.3 mcg/dL , ACTH of 3050 pg/mL , and DHEA-S of 145 mcg/dL . He had an elevated 17-OH pregnenolone to 17-OH progesterone ratio, consistent with 3The first TART case was reported in 1940 in a 3-year-old boy with marked salt cravings, an enlarged penis and scrotum, and pubic hair who died in the hospital just days after admission . LaboratTART prevalence as high as 94% is reported in patients with CAH , 22–24 aAccurate distinction between TART and LCT in adults without known CAH can be problematic. This is particularly true if the TART is unilateral or bilateral with a metachronous presentation (as in the current case). Even with foreknowledge of CAH in a patient with bilateral testicular masses, morphologic features may not allow distinction between TART and LCT . HoweverClinical and radiographic features can be used to distinguish TART from LCT. TART presentation varies with age. In boys, the lesions are often unilateral  and the Endocrine features can be used to distinguish TART from LCT. TARTs represent reactive hyperplasias that develop in response to supraphysiologic ACTH, whereas LCTs are not ACTH-responsive and are not associated with supraphysiologic ACTH. Thus, preoperative serum ACTH can be used to screen for background CAH in patients with uni- or bilateral testicular tumors. Both LCTs and TARTs can be associated with alteration in serum testosterone. Most CAH is due to 21-hydroxylase deficiency, leading to high testosterone levels secreted by both the adrenals and the testes, until the TARTs get so big as to cause peritubular fibrosis leading to low testosterone. Thus, the serum testosterone in CAH is a function of the extent of testicular TART expansion , 34. HowGross and microscopic pathologic features can be used to distinguish TART from LCT. Bilaterality, multinodularity, dark brown color, hilar/paratesticular location, coarse fibrous stroma, and/or strong synaptophysin immunoreactivity favor TART over LCT , 8, 9. UThis patient's mild clinical symptoms of CAH likely contributed to the lack of clinical detection or treatment of his CAH, prolonging his exposure to high ACTH and further stimulating the ACTH-responsive steroid cells in the hilum of the residual testis. It has long been considered that growth of these lesions is due to chronic elevations of ACTH. TARTs have been shown to have ACTH receptors and to secrete steroids when stimulated with ACTH in vivo . It can β-HSD deficiency. This case highlights the challenges that urologists, radiologists, pathologists, and endocrinologists face when dealing with Leydig-like tumors in adults. We recommend a detailed history and physical examination as the first step in the evaluation of all patients with testicular masses, in addition to serum tumor markers and imaging. In this patient, the evolution of bilateral testicular masses over a short time interval could have prompted a more thorough discussion of past medical and family history, which would have revealed bilateral cryptorchidism, hypospadias, and a grandfather with early puberty. Taken together, this piece of information could have led to additional endocrine evaluation prior to surgical intervention. Because unilateral LCT and unilateral TART will share similar Leydig-like morphology and because both can involve the hilum and parenchyma, we recommend checking ACTH levels in all patients with a diagnosis of LCT with hilar involvement, as this may prompt formal preoperative workup for CAH.We present a rare case in which pathology from a second orchiectomy led to a diagnosis of CAH due to (rare) 3


Leiomyoma of vulva is rare, and usually misdiagnosed clinically as Bartholin cyst. It usually presents spindle-shaped tumor cells, but some rare cases consisted mainly of atypical epithelioid tumor cells. We report here a case of 30-year-old woman consulting with a vulvar mass of 7 cm in the Bartholin glands area. The lesion was surgically excised with its capsule completely. Pathological examination and immunochemistry showed characteristic of epithelioid leiomyoma with myxoid stroma with both estrogen receptor (ER) and progesterone receptor (PR) staining negative, which was really rare as only 2 cases of vulvar leiomyoma with both ER and PR were reported before. Smooth muscle tumors of the vulva are rare and are usually misdiagnosed clinically as Bartholin cysts. These tumors are considered to originate from smooth muscle within erectile tissue, blood vessel walls, the round ligament, the dartos muscle, or the arrector pili muscle  and fromA 30-year-old Chinese G2P1 female had found a mass in her left labium major for more than 7 years. The mass was about 1 cm when first discovered and asymptomatic in the next 6 years and then showed obvious enlargement in the last year. The patient did not feel any discomfort and never went to hospital until this visit. Her general physical examination was normal except for a palpable soft mass of about 7 cm involving the lower half of left labium major. During the 7 years the patient had a history of full-term pregnancy of cesarean delivery and an induced abortion of the first trimester. The family and patient's past medical history were not significant.The mass was considered clinically to be a Bartholin gland cyst before surgery. When mucous between hymen and left labium minor was incised, soft fragile tissue of raw fish appearance was found but well circumscribed with a capsule. The mass with its wall was carefully excised. The patient had a good postoperative recovery with no complications. Recurrence was not found in the next 14 months.The gross specimen consisted of a soft mass measuring 7 cm in diameter. The cut surfaces were pink to white gray in color with a rubbery and fleshy quality. Microscopically, the neoplasm consisted of epithelioid cells arranged in nests filled with mucous material. The nuclear morphology was uniform throughout and consisted of round, regularly shaped nuclei with a single prominent nucleolus. Mitotic figure was rare, and no atypical mitoses were found. The tumor was sharply demarcated peripherally and was covered throughout by a thin layer of delicate fibrous tissue. The immunohistochemical staining pattern of our case, strong positivity for SMA and caldesmon, and focal positivity for desmin  are indiVulvar leiomyoma is rare. Reidel found only one leiomyoma after reviewing 144 vulvar tumors . There aAs a rare variant, only a few cases of vulvar epithelioid leiomyoma have been reported in the English literature. We reviewed the literature in PubMed and summarized the reports of vulvar epithelioid leiomyoma in For our case, we diagnosed the mass as Bartholin gland cyst for the first impression. After surgery we encountered difficulty in making a definite diagnosis as the myxoid appearance was extremely rare. Supportive immunohistochemistry is important for diagnosis and differentiation besides hematoxylin and eosin-stained sides when neoplasms arise at unusual sites. We examined several indicators and found strong positivity for SMA and caldesmon and focal positivity for desmin, which is indicative of smooth muscle differentiation. ER and PR staining were negative which was quite different from leiomyoma from uterine.It was suggested that vulvar leiomyoma was an estrogen dependent neoplasm , 9. EstrER and PR are highly expressed in uterine leiomyoma. It can be interpreted that leiomyoma originates from ER/PR expressing myometrial smooth muscle cells. But smooth muscle tumors of vulva were thought to originate from smooth muscle within erectile tissue or blood vessel walls, the round ligament, the dartos muscle, or the arrector pili muscle, which may lack intrinsic ER/PR expression. As Malcolm reported, there was a significant decline of ER staining in epidermis and fibroblast from vagina towards labia minora, labia majora, and the suprapubic skin. In their report ER staining was not seen in skin appendages, blood vessels, and lymphatics except for occasional sweat glands; for PR, no staining was seen in vulvar skin appendages or vessels .The myxoid material of epithelioid leiomyoma of vulva can be digested by hyaluronic acid, indicating that it was composed of hyaluronic acid . The myxThe most confusing problem with vulvar smooth muscle tumor is to distinguish benign from malignant forms. There are significant differences between the criteria used for smooth muscle tumors of uterine and soft tissues. Significant mitotic activity, focal degenerative cellular atypia, and/or hyaline necrosis can be present in benign uterine leiomyoma, which may be thought of as features of leiomyosarcoma of other places . TavassoLocal excision of tumor with its capsule and a surrounding rim of normal tissue was recommended for the patient . In Niel


We present a 13-year-old male patient with congenital melanocytic nevi and a yearlong history of enlarging right preauricular mass. Examination revealed 2 congenital melanocytic nevi overlying a large, firm, mobile 2.7 × 2.5-cm subcutaneous mass with irregular borders. Pathology confirmed intradermal melanocytic nevi; the underlying mass was a calcified and ossified pilomatrixoma.What is a pilomatrixoma/pilomatricoma?What are the causes and syndromic associations of pilomatrixomas?What are congenital melanocytic nevi, and what is the risk of malignant transformation?What is the association between pilomatrixomas and congenital melanocytic nevi?,2-4Pilomatrixomas are cutaneous adnexal tumors, also referred to as calcifying epithelioma of Malherbe, as it was first described by Malherbe and Chenantais more than 100 years ago.1,4,1,1,4Pilomatrixoma has been reported to arise in a site of trauma or previous infection.1Congenital melanocytic nevi are benign proliferations of the nevus cell type of melanocytes that present at birth or within the first few months of life. They can extend into the dermis and subcutaneous structures and are usually small (<1.5 cm) or medium sized (1.5-20 cm).6,6Although many case series have been published on the topic of pilomatrixoma, the association between pilomatrixoma and intradermal melanocytic nevus has not yet been reported. There have been studies detailing a pigmented type of pilomatrixoma, in which the presence of melanin within the pilomatricoma itself was the distinguishing feature.7We present a unique case of an otherwise healthy 13-year-old male patient with 2 melanocytic nevi  overlyin


The rare phenomenon of tumor-to-tumor metastasis was first described in 1930. The donor neoplasm is most frequently lung or breast carcinoma, whereas intracranial meningiomas are reportedly the commonest recipient neoplasm. Here we report a case of metastasis from a primary gastric cancer to a uterine lipoleiomyoma. A 65-year-old woman presented with locally advanced gastric cancer with computed tomography (CT) evidence of peritoneal dissemination and a 9 cm pelvic mass. She underwent 16 courses of TS-1/cisplatin chemotherapy, which achieved significant tumor reduction. However, repeat CT and magnetic resonance imaging revealed a 9 cm diameter pelvic mass adjacent to the uterus. The mass was heterogeneously hyperintense on T1- and T2-weighted images with some low signal spots on fat-suppressed T1-weighted images, suggesting a benign ovarian tumor such as a mature cystic teratoma. After 3 months, pelvic CT revealed a 10 cm multilocular cystic mass that exhibited heterogeneous enhancement after intravenous contrast administration. A diagnostic laparotomy revealed a subserosal uterine tumor extending into the right broad ligament; total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed. The uterine tumor showed histological features of lipoleiomyoma infiltrated by well- to moderately differentiated carcinoma cells that were similar to those of the gastric biopsy, supporting a diagnosis of metastatic gastric adenocarcinoma. Other than by direct invasion from adjacent pelvic organs, metastasis of extragenital cancers to the uterus is uncommon. In addition, metastases to uterine lipoleiomyomas, a rare variant of leiomyoma, are extremely rare. The rare phenomenon of metastases to histologically distinct tumors was first described in 1930 . As for A 62-year-old woman, gravida 2 para 2, presented with abdominal pain. Gastrointestinal endoscopy showed superficial gastric erosions and histopathological examination of endoscopic biopsy specimens revealed poorly differentiated adenocarcinoma. Staging computed tomography (CT) scan demonstrated thickening of the gastric sinus wall, peritoneal dissemination, and 9 cm diameter solid pelvic mass. Based on these findings, the patient was diagnosed as having advanced (stage IV) gastric cancer and treated with TS-1/cisplatin (CDDP) chemotherapy. After 16 courses of TS-1/CDDP chemotherapy, gastroscopy showed complete resolution of the ulcerous lesion and CT demonstrated significant reduction in peritoneal dissemination. However, the pelvic tumor had not changed in size and had developed a cystic component. The patient was referred to our hospital for evaluation of the pelvic tumor.At the first visit to our hospital, the patient's general condition was good and her medical and family histories unremarkable. On pelvic examination, a firm, nontender, approximately 10 cm diameter mass was palpated in the right lower abdomen. Transvaginal ultrasonography and computed tomography (CT) revealed a 10 cm diameter pelvic mass Figures . MagnetiThe subserosal uterine tumor measured 10 cm in diameter and had a yellow-white cut surface; the cysts contained yellow serous fluid . No areaThe patient's postoperative course was uneventful, and she was discharged on the seventh day. The TS-1/CDDP chemotherapy was resumed and she remained alive with disseminated metastases 14 months after the surgery.Lipoleiomyoma of uterus is a rare variant of uterine leiomyoma, the incidence reportedly being 0.03% to 0.2% of all uterine tumors . On sonoOther than by direct invasion from adjacent pelvic organs, metastasis of extragenital cancers to the uterus is uncommon . MoreoveThe phenomenon of tumor-to-tumor metastasis was first described in 1930 , 5–11. T


A 37-year-old male athlete was diagnosed with primary tracheal adenoid cystic carcinoma following investigation for dyspnea, wheeze, and eventual stridor. Preoperative bronchoscopy revealed a highly vascular tumor 4 cm distal to the cricoid with no gross disease extending to the carina. Imaging revealed circumferential tracheal irregularity immediately inferior to the cricoid, with no definite cricoid invasion. Locoregional extension of disease was noted invading the thyroid and abutment of the carotid approximately 180°. Intraoperative findings identified tracheal mucosal disease extending distal to the carina and proximally at the cricothyroid joints where bilateral functional recurrent nerves were preserved. A decision made to preserve the larynx given the inability to fully resect distal tracheal disease. A 5 cm sleeve resection of the trachea was made with a cricotracheal anastomosis following suprahyoidal muscle release and laryngeal drop-down. The patient was treated with adjuvant radiotherapy including platinum based chemotherapy in an effort to maximise local control. PET scanning three months after therapy revealed no FDG uptake locally or distally. Adenoid cystic carcinoma is the most common tumour of minor salivary glands; however primary tracheal adenoid cystic carcinoma is a rare occurrence. The classic management involves surgical resection and adjuvant therapy; however achieving clear surgical margins in this disease and the implication of distant dissemination make for the challenging oncological management , 2.We report a case of a 37-year-old professional male athlete presenting with a seven-month history of worsening respiratory function. He was diagnosed with asthma and managed in the community. He was referred for further investigation following worsening of his symptoms and the onset of stridor. Flexible nasendoscopy revealed intact vocal cord function with a mass lesion visible in the trachea. Rigid bronchoscopy reported an intraluminal tracheal mass immediately inferior to the cricoid extending five centimeters caudally resulting in eighty percent tracheal obstruction . The carMRI scan and PET-CT demonstrated a low to intermediate FDG uptake of 5.9. Key findings from imaging included submucosal extension within the tracheal lumen, invasion of thyroid gland, and no direct invasion of the cricoid cartilage. There was no evidence of cervical nodal enhancement or distant dissemination. The patient's case was discussed at our multidisciplinary team meeting with input from cardiothoracic surgery, medical oncology, radiation oncology, and the head and neck team. Tracheal resection with primary cricotracheal anastomosis, total thyroidectomy, and preservation of the larynx was the proposed surgical intervention.T-shaped neck incision at the level of cricoid cartilage was made with a partial sternotomy to afford greater access . Vessel Chin to chest sutures were removed at day 14 after operation and patient was discharged home. Three weeks post-op the patient maintained normal voice quality, normal deglutition and showed significant improvement in exercise tolerance.Histopathological analysis of the operative specimen confirmed adenoid cystic carcinoma with a predominately cribriform pattern . The dis2 were administered under the care of the medical and radiation oncology team. No severe or serious adverse events were reported.Following discussion at our MDT meeting a decision was made to give adjuvant radiation and concurrent platinum based chemotherapy given the potential locoregional advantage its inclusion may afford . A totalA follow-up PET scan performed 3 months after adjuvant radiation and chemotherapy showed no FDG uptake both locoregionally and distally. The patient has also returned to playing football at professional level maintaining an excellent level of pulmonary function, phonation, and deglutition.Primary tracheal malignancy is a rare occurrence accounting for 0.2% of all respiratory malignancies in the United States  or an anR0) rate from a cohort of 6 patients with tracheal minor salivary gland malignancy. An aortic allograft is interpositioned following tracheal resection splinted by a silicon stent. A pectoral muscle flap is then wrapped around the graft to promote revascularization and prevent fistula formation.Surgical excision remains the mainstay of treatment for primary adenoid cystic carcinomas of the trachea. The trachea is a fibroelastic tube supported by incomplete cartilaginous rings. The adult trachea has a mean length of 11 cm (cricoid to carina) with a range of 9–15 cm. The amount of trachea that can safely be excised varies substantially between patients. Young age, thin body habitus, and long neck are favorable patient factors for long tumors. Tracheal mobilization manoeuvres include extreme flexion of the neck (1 to 6 cm), incising the annular ligaments between tracheal rings (1 to 2 cm), suprahyoid or infrahyoid release of the upper laryngotracheal unit (2.5 to 5 cm), and blunt dissection and mobilization of the lower tracheal segment (0.5 to 1 cm). A combination of laryngeal release procedures, blunt mobilization of the lower tracheal segment, and neck flexion will yield about 4 to 6 cm of mobilization depending upon the patient's age and range of neck motion . FurtherMany reports recommend adjuvant radiotherapy (RT) for all patients with adenoid cystic carcinomas to improve local control rates and improve disease free survival , 13. RadCombined chemoradiation is however widely available. There is some evidence that concurrent chemoradiotherapy, especially with platinum based chemotherapy, may have a role in improving locoregional control , 14–16. Allen et al.  reportedThe defining factor in our case is the deliberate decision of laryngeal preservation in managing advanced tracheal adenoid cystic carcinoma. The intraoperative finding of submucosal disease extension, histological reporting of positive margins, and perineural and lymphovascular invasion may argue an aggressive resection. However, performing a total laryngectomy with further 5 cm tracheal resection would leave our patient aphonic with mediastinal tracheostomy, limited respiratory function, and poor quality of life without any oncological gain. The published literature well describes distant metastasis in the setting of advanced AdCC being independent of local and regional control , 2. It iAdCC of the trachea is a rare malignancy that is difficult to diagnose due to the nonspecific nature of the presenting symptoms . The tum


The ameloblastoma has been described as benign but locallyinvasive; benign and locally invasive with strong tendencyto recur; and locally malignant. Recurrence of this lesion into the infratemporal and temporal region are rarely reportedcases. Complete excision of lesion was done with the helpof the advance imaging modalities and possible cause ofrecurrence in this case is discussed. First introduced in 1977 by Robinson and Martinez andpopularized by Gardner in 1983.It has been used to describe an ameloblastomadeveloping within the lining, lumen, or wall of a cyst aswell as an invasive ameloblastoma that has a single cysticspace rather than multicystic spaces. In some publicationsthe term has been used to describe an ameloblastoma limitedto the cyst lining, an ameloblastoma solely within the cystlumen and in still others, an ameloblastoma with varyingdegrees of invasion through the connective tissue layers ofthe cyst. No standard terminology to describe cysticameloblastoma with limited invasion to aggressive invasionis followed, which as lead to inadequate curative surgicalapproaches and inaccurate recurrence rates.A 20 years old female patient reported to our maxillofacialunit with a swelling on the right temporal region of facesince 18 months. The swelling increased gradually with nohistory of pus discharge or pain before and after the onsetof swelling. A significant past history was reported frompatient that she suffered a similar type of swelling on theright side of ramus of the mandible around 30 months back.Patient visited to a regional cancer center where a simpleenucleation was done and reported histopathologically asradicular cyst. Six months following surgery patient startednoticing a swelling in right temporal region which graduallyincreased to present size with gradual decrease in mouthopening .Swelling in the right temporal region extendinganteriorly up to lateral orbital rim and zygomatic processof maxilla. Posteriorly up to root of the ear but notcrossing it. Superiorly 5 cm above the root of the earanteriorly up to temporal hair line and inferiorly 3 cmbelow the zygomatic arch.l × b)Size of the swelling around 10 × 5 cm . Uniform thick cystic lining can beappreciated with thinned out bone margins.CT scan of the recurrent lesion in the right temporal andinfratemporal region shows a bony cystic lesion extendingfrom coronoid and condylar region inferiorly to approximatelysuperficial temporal line superiorly. Anteroposteriorlyinvolving complete infratemporal and temporal region.Zygomatic arch is displaced laterally due to expanding bonycyst. Condylar head is fused to posteroinferior portion of thebony cyst (decreasing mouth opening). There is no bony wallinferiorly and is continuous with soft tissue. Content insidethe cystic lesion shows radiodensity equivalent to fluid. Cystlining cannot be described radiologically. Bones formingboundaries of infratemporal region appear normal. Nointracranial extension noted radiologically .Preoperatively fluid was aspirated from the bony cysticlesion. Fluid sample was sent for biochemistry for proteinanalysis, microbiology for culture and cytology for cells.Protein more than 4 mg/dl, culture reported no growth andcytology reported only macrophages.No preoperative biopsy was planned because clinical,radiological and cyst aspiration findings suggested ofbenign cystic lesion. Histopathological report of previoussurgery was reported as radicular cyst. A preauricularincision extended superiorly in to the scalp around thesuperior border of cyst up to the lateral part of the foreheadanteriorly was placed. Temporal fascia and muscle wasidentified and incised to expose the bony cyst. Anteriorlypericranium was incised to expose lateral orbital rim.Inferiorly zygomatic arch was identified and dissectioncarried out inferior to the arch up to the lower border ofthe bony cyst . Surprising finding was notedintraoperatively that the bony cyst was located within thetemporalis muscle by splitting it in to medial and lateralhalves. Part of the muscle was sandwiched between medialportion of the bony cyst and lateral wall of skull.Zygomatic arch was removed. Complete enmass excisionof the cyst done after separating it from condylar head. Condylar head was resected to increasemouth opening. Infratemporal space was carefullyexamined to find any remnants of cyst lining. Closurewas done in four layers . Specimen was sent forhistopathology and it was reported as unicystic ameloblastoma.Postoperatively patient recovered uneventfullywith improved mouth opening .The hospital pathologist collected multiple samples fromthe excised bony cystic lesion. Histological study reportedas unicystic ameloblastoma. High-power view showsperipheral columnar cells, resembling preameloblasts,exhibiting reverse polarity.The ameloblastoma has a long history of recognition,reportage, and controversy. It is the most recorded lesion ofthe odontogenic series and continues to evoke widedifferences of opinion among pathologists and surgeonsconcerning its position in the neoplastic spectrum, biologicbehavior, and management. The ameloblastoma has beendescribed as benign but locally invasive; benign and locallyinvasive with strong tendency to recur; and locallymalignant. The 1992, WHO classification categorizes theameloblastoma as a benign but locally invasive epithelialodontogenic neoplasm.Consider the following description of the basal cellcarcinoma, an acknowledged malignant tumor: slowlygrowing, infiltrative, capable of great destruction of softtissue and bone, recurrent when not completely eradicated,capable of invading vital structures ,seldom metastatic but capable of metastasis, presenting anumber of histologic patterns, and arising from the epithelialskin surface and skin adnexa. Substitute oral epithelium forskin surface and dental lamina/enamel organ for skin adnexa,and you are describing the known characteristics of theameloblastoma.A unicystic lesion may arise as an ameloblastoma denovo or from secondarily in an odontogenic keratocyst ordentigerous cyst. Occasionally, a unicystic lesion, particularlyof the plexiform type, is misdiagnosed as an inflamedcyst with hyperplastic epithelial lining.Ameloblastomas have locally invasive nature so they mayspread to the infratemporal fossa, pterygopalatine fossa,parapharyngealspace, orbit, or intracranial space. Ameloblastomacan alsooccur in the temporal region due to implantationof tumor cells in the soft tissues during surgery.In this case the lesion did not present with deep seatedpain or cranial nerve involvement which is a commonpresentation of infratemporal masses.Anatomically, the infratemporal fossa lies on the lateralaspect of the cranial base, deep to the zygomatic arch,masseter and mandibular ramus. The infratemporal spaceor fossa is basically pyramidal in shape. The base of thepyramid is formed by the medial aspect of the ramus andthe upper surface of the pyramid is the floor of the skull.The anteromedial aspect corresponds to the posterior aspectof the maxilla and the posteroinferior aspect to thepterygomaxillary fascia.Cohen et alThe surprizing finding of this lesion intraoperatively wassplitting of temporalis muscle in to medial and lateral halvesby the growing bony cystic lesion. This shows the localinfiltrating behavior of the ameloblastoma. The problem ofrecurrence following removal of an ameloblastoma can beattributed to inadequate excision and the spread of residualtumor fragments within adjacent bone.


At present, two years after this multimodal therapy, the patient exhibits no signs of loco-regional recurrence or distant metastases. This study highlights the importance of a multidisciplinary approach in the diagnosis and treatment of this extremely rare entity with intracranial extension. In addition, the study reviews the English literature with regard to SS of ITF and discusses the clinicopathological features, management and outcome.Synovial sarcomas (SS) are high-grade soft-tissue sarcomas, predominantly found in the deep soft tissues of the lower extremities, with only 3–5% occurring in the head and neck region. Primary SS of the infratemporal fossa (ITF) is exceptionally uncommon. The present study reports the case of a 23-year-old female with an SS arising in the ITF. To the best of our knowledge, this case is only the second patient with intracranial involvement recorded in the literature. The patient was treated primarily with surgery, followed by a total of 60 Gy adjuvant radiotherapy and chemotherapy, consisting of cisplatin (25 mg/m Synovial sarcomas (SS) are high-grade soft-tissue sarcomas that are associated with poor survival . The tumDue to the low clinical morbidity, concealed anatomical site, non-specific symptoms and heterogeneous histopathological features, SS in the ITF are often misdiagnosed. As a result, clinical diagnosis and treatment planning remain a challenge. To the best of our knowledge, a search of the English literature indicated that there are eight previous studies revealing nine cases of SS located in the ITF, including only one case with intracranial involvement  5–12). –12. 5–122 intravenously on days one to three), epirubicin (25 mg/m2 intravenously on days one and two) and ifosfamide (1.8 g/m2 intravenously on days one to five) for three cycles was administered to prevent local recurrence and distant metastasis. At the 24-month follow-up neither local recurrence nor metastatic disease were apparent. Written informed patient consent was obtained for publication of this study.A 23-year-old female was referred to the Stomatology Hospital of Xi’an Jiao Tong University  for consultation, due to painless swelling of the right cheek that had been present for five months. The patient had no history of trauma or facial surgery. Upon physical examination, facial asymmetry and slight right-sided cheek swelling was observed . The maxA review of the English literature revealed that a total of 10 cases, including the present case , regardiThe tumor site determines the clinical presentation of the head and neck SS . The ITFTumors of the ITF present with a wide spectrum of pathologies, both benign and malignant . A smallThe diagnosis of SS is made on the basis of its relatively distinctive, yet markedly variable, histopathological appearance, in conjunction with histochemical findings, immunohistochemistry, electron microscopy and cytogenetic analysis, which have proved valuable in confirming the morphologic diagnosis ,17. Two In the present case, the diagnosis of biphasic SS was made from the histopathological findings and supporting immunohistochemical features. FNAC was performed pre-operatively and yielded no definitive diagnosis. In fact, of the four patients that underwent FNAC in the reviewed cases, only one case led to a confirmed diagnosis, indicating the limited nature of this technique as a routine diagnostic procedure.The optimal approach to the treatment of this malignancy remains undefined and there is no standard treatment protocol for SS of the head and neck . From thFor soft-tissue sarcomas in general, the prognosis is associated with the resection margins . SS, oneet al reported a similar case of SS in the ITF with intracranial extension (Recently, Aslan xtension . In thisDue to the limited number of ITF cases, every new case will highlight novel information about management strategies and prognosis. The current study presented an extremely rare case of primary SS in the ITF with intracranial involvement. Albeit with only a short follow-up period, the patient achieved good outcome after multimodal therapy. Based on a review of the literature, SS in the ITF is insidious due to its special anatomical features in the skull base, and it may not be noticed until there is impairment of function and the appearance of symptoms. CT and MRI are useful non-invasive diagnostic tools, and the final diagnosis of SS is made on the basis of unique pathological and immunohistochemical findings. In this rare entity with or without surrounding soft/bony tissue extension, multimodal therapy with surgical excision followed by early post-operative chemoradiotherapy can be a promising factor controlling local recurrence and distant metastasis.


Neurenteric cysts are CNS lesions most frequently occurring in the spinal cord. Intracranial neurenteric cysts are rarer, typically presenting with headache, mass effect, or location-specific symptoms. The area postrema is known as the emetic center of the brain; lesions can cause nausea and vomiting. Our case, featuring a neurenteric cyst of the area postrema, illustrates the importance of considering a neurological etiology for nonspecific symptoms that otherwise elude explanation. Our patient presented with acute decompensated hydrocephalus upon exploratory abdominal laparoscopy for unresolving abdominal pain. The patient had an eight-month history of unexplained intermittent nausea, vomiting, and abdominal pain. These bouts increased in frequency during the weeks before acute presentation, prompting exploratory abdominal laparoscopy. The acute decompensation was managed by ventriculostomy, and cranial MRI revealed a cystic mass by the floor of the fourth ventricle. After the patient stabilized and returned to neurological baseline, suboccipital craniectomy and resection were performed. The mass was histologically identified as a neurenteric cyst. The patient was free from neurological complaints at one-year follow-up, indicating that the successful resection of the area postrema-associated neurenteric cyst resolved her previous symptoms. Thus, some intracranial lesions can masquerade as nonspecific symptoms, presenting a challenge to accurate diagnosis. Neurenteric cysts (NCs) are rare congenital abnormalities occurring within the central nervous system. They most frequently present as lesions of the ventral spine , 2 and aWe present a case of NC adherent to the area postrema (AP), successfully treated surgically. The AP is an emesis-inducing center in the medulla, located at the caudate floor of the fourth ventricle. AP tissue is densely vascularized and lacks tight junctions, enabling the detection of toxins in both blood and cerebrospinal fluid (CSF) –12. AlthA 34-year-old Caucasian female presented with an eight-month history of frequent emergency department (ED) visits, related to episodically severe abdominal pain with nausea and vomiting. Within several weeks prior to presentation, she presented at the ED four times for migrating abdominal pain with associated nausea and vomiting. During that time, she was treated for gastroenteritis and irritable bowel syndrome but continued experiencing abdominal pain and nausea. With each ED visit, she underwent similar clinical and imaging evaluations that focused on the chief complaint of nausea, vomiting, and abdominal pain .Several ovarian cysts, thought to be physiologic, were identified via abdominal scan. Finally, exploratory laparoscopy was performed. The following day, she exhibited progressive neurological deterioration. Cranial CT demonstrated hydrocephalus. In an obtunded state, she was emergently transferred to neurosurgery service.Upon physical examination, she was unresponsive to verbal cue. She was able to moan and to move all four extremities. Her pupils were large and sluggishly reactive.She underwent emergent ventriculostomy and returned to her normal neurological baseline within 24 hours. Cranial MRI evaluation demonstrated a cystic mass near the caudal region of the fourth ventricle, resulting in obstructive hydrocephalus and brainstem compression Figures .She subsequently underwent suboccipital craniectomy for gross total tumor resection. Intraoperatively, after the arachnoid overlying the cistern magna was opened and the cystic mass was punctured and well decompressed, the cyst wall was dissected free from surrounding brain, including the floor of the fourth ventricle and cerebellum. The cyst wall was noted to be particularly adherent to the AP.Microscopic examination of the dissected tissue revealed a cystic mass lined by mucous columnar cells, surrounding proteinaceous material, calcifications, vascular ectasia, and reactive changes with embedded small glandular structures. The diagnosis of NC was confirmed  symptoms correctly prompted diagnostic attention towards the abdomen. Although an extensive diagnostic workup was repeatedly performed and ovarian cysts were incidentally identified, an etiology for the patient's symptoms was never determined. She was treated for gastroenteritis and irritable bowel syndrome but continued experiencing abdominal pain and nausea. Such symptoms promptly resolved after surgical resection of the fourth ventricular NC. In this case, it is difficult to explicitly ascribe such presenting symptoms to mass versus local chemical effect on the AP. Notably, other authors have reported that a lesion of the AP can create nausea, vomiting, and appetite change .The AP is a sensory circumventricular organ associated with the fourth ventricle, first associated with nausea and vomiting in the 1940 s/50 s . CompareAlthough the AP is well associated with nausea and vomiting , 12, andThe AP is also clinically associated with neuromyelitis optica (NMO). The association illustrates that altered cell function can also induce nausea and vomiting. In NMO, autoantibodies against aquaporin 4, a water channel thought to be an important component of the astrocytic foot processes of the vascular epithelium of the AP, instigate an inflammatory process resulting in damage to the other cells in the area and demyelination , 23. If  (H&E) stained sections from the brain lesion revealed that cystic areas were predominantly lined by simple columnar epithelium with nonciliated cells, while cuboidal epithelium was uncommon. Goblet cells were evident throughout the epithelium , cytokeratin-20 (CK20), and epithelial membrane antigen (EMA) . The posWe describe an uncommon presentation of an already rare central nervous system cyst. Our patient presented with unexplained and intermittent nausea, vomiting, and abdominal pain and was found to have a NC attached to the AP, or emetic center of the brain. The AP has few associated published case studies; the presentation of our patient may be relevant to the understanding of lesions of the AP in future patients. Our case also serves as a reminder to consider a neurological etiology for common and nonspecific symptoms that lack an alternative explanation. One of the medical challenges illustrated by this case is the need to define when, in the context of nonspecific symptoms such as nausea and vomiting, a neurological etiology should be considered.


Hepatic abscesses and hepatic metastasis are common diseases. However, hepatic abscesses seldom occur in patients with hepatic metastases. We describe a case of a 67-year-old female patient with abdominal pain in the right upper quadrant. Magnetic resonance imaging revealed several lesions, with the largest lesion displaying features of both hepatic pyogenic abscess and liver metastasis. These features included iso- or hypointense signaling on T1WI and T2WI, hyperintense signaling on diffusion weighted imaging of the thick wall, and mixed hyperintense signal in the center on DWI, as well as dramatic and irregular peripheral enhancement was detected on LAVA dynamic contrast scanning.Klebsiella pneumoniae and a pathologic diagnosis of adenocarcinoma. At this point, the patient admitted a history of colon adenocarcinoma 9 years ago treated with hemicolectomy. Therefore, this patient was considered to have a hepatic pyogenic abscesses complicated by hepatic metastasis. The patient began treatment for the responsible pathogens and underwent chemoembolization of the liver lesions. In special cases, we could attempt to pursue a more detailed search for coexistence of microorganism infection and tumor.Aspiration and culture of the largest lesions revealed  The pyogenic liver abscess is a type of liver abscess caused by bacteria. Pyogenic bacteria can gain access to the liver by direct extension from contiguous organs or through rich dual blood supply provided by the portal vein and hepatic artery. Magnetic resonance imaging (MRI) is a non-invasive way to detect the abnormalities in the liver and has become essential to the diagnoses of both hepatic abscess and hepatic metastasis.In this case, a 67-year-old patient presented with abdominal pain in the right upper quadrant for more than one day. Physical examination demonstrated right upper quadrant tenderness without rebound or guarding. Carcinoembryonic antigen (CEA) level was 35.34 ng/ml. She did not have past medical history of hepatitis, tuberculosis or malaria.MR of the abdomen was performed through a 1.5-Tesla MR Scanner . Axial T1 Weighted Image (T1WI) , axial TKlebsiella pneumoniae; pathological examination further demonstrated adenocarcinoma. The immunohistochemical studies revealed tumor cell positivity for cytokeratin 19, carcinoembryonic antigen , villin, caudal-related homeodomain transcription-2, and Ki67 50%, but studies were negative for TTF-1, cytokeratin 7, cytokeratin 20, and NapsinA. Combining the aspirated pus and the pathological results, we established the diagnosis of liver metastasis complicated by hepatic pyogenic abscess. Retrospective clinical history indicated that the patient had a history of colon adenocarcinoma 9 years ago, for which she underwent a hemicolectomy but did not receive chemotherapy.The largest lesion was aspirated and culture returned positive for The patient began treatment for the responsible pathogens and she underwent chemoembolization of the liver lesions, after which she demonstrated clinical improvement.Hepatic pyogenic abscesses usually arise from portal pyaemia of various causes. The etiologies of these abscesses are divided into six categories based on the extension route of infection: 1) through the bile ducts, 2) by way of the portal vein, 3) by direct extension, 4) from blunt or penetrating trauma, 5) through the hepatic artery, or 6) of obscure origin, or cryptogenic, where no primary source of infection is found, even after abdominal exploration or autopsy.Various imaging modalities can help diagnose hepatic pyogenic abscesses. Among those tests, MRI is a non-invasive way to detect the hepatic abscess. The center of a liver abscess often appears as mixed signals with hypointense signaling on T1WI and hyperintense signaling on T2WI. The wall of the abscesses is peripherally enhanced. Additionally, abscesses tend to demonstrate high signaling within the abscess cavity on DWI. This DWI signal is a sensitive marker to detect the hepatic abscesses. Purulent content of an abscess restricts the diffusion of water molecules, resulting in a hyperintense appearance. In this case, the center of the biggest lesions demonstrated the hyperintense signaling on DWI.A liver metastasis is a malignant tumor in the liver that has spread from another organ affected by cancer. During the development of invasive tumors, tumor cells disobey the social order of organ boundaries and cross into foreign tissues.Klebsiella pneumoniae. In view of the widely varying appearances of hepatic abscesses on imaging, and their ability to masquerade as metastatic lesions, it may be necessary to confirm the diagnosis with a needle biopsy.9Our patient’s case was rare in that the hepatic metastasis was infected with


A 13-year-old boy with severe clinical symptoms and signs underwent surgery for a posterior fossa ependymoma in 1954. The tumor was adjacent to the floor of the fourth ventricle, and surgery was complicated by profound bleeding. Therefore, only a partial resection was performed. Postoperative radiotherapy was given to the posterior fossa. The recovery was uneventful, and he has been in full-time work until the age of 62 years and is now 74 years old. Repeated MRI scans demonstrate a stable residual fourth ventricular tumor. Ependymoma is the third most common posterior fossa tumor in childhood after astrocytoma and medulloblastoma/PNET , 2. A spIn September 1954, a 13-year-old boy was referred to our department with vertigo, episodes of unclear vision, and later on, vomiting and headache. Clinical investigation revealed choked disks with hemorrhages, nystagmus, a slight right-sided facial paresis, dysdiadochokinesis, and spasticity in the right arm as well as a positive Babinski sign on the left side.3 CSF from the lateral ventricle, the intradural pressure was normalized. After dural incision, a red-brown tumor was exposed medially in the right cerebellar hemisphere and into the fourth ventricle. The tumor was resected, but only partially removed because it was adjacent to the floor of the fourth ventricle, and surgery was complicated by the occurrence of profound bleeding. Microscopic examination revealed a well-circumscribed, moderately cellular glial tumor with several pseudorosettes. No mitoses, microvascular proliferation, or necrosis was found. The tumor cells were positive for glial fibrillary acid protein (GFAP) and negative for epithelial membrane antigen (EMA), synaptophysin, neurofilament protein, and the IDH-1 (R132H mutation). Less than 5 % of the tumor cells were Ki-67 positive. The tumor was classified as an ependymoma, WHO grade II.Ventriculography demonstrated an enlarged supratentorial ventricular system and a tumor in the fourth ventricle. The patient had surgery where a partial occipital bone resection was performed with opening of the foramen magnum and laminectomy of atlas. The dura was pulseless, but after removal of 60 cmPostoperatively, the boy recovered clinically within weeks and the choked disks resolved completely. During the next 2 months, he was given high-voltage radiotherapy to the posterior fossa (45 Gy in 30 fractions).Later on, he returned to school and finished a year later than his peers. He married and got two children. After 26 years as a bank officer, the started his own accountant firm.He worked full time until the age of 62. At the age of 67, he experienced an episode of syncope but recovered completely without treatment. An MRI demonstrated a large contrast-enhancing tumor of the fourth ventricle. His clinical situation has been stable and good, and the MRI findings are also unchanged after several years of observation Fig. .Fig. 1MRLong-term survival after operation for posterior fossa ependymoma in childhood has been observed for many years, especially after GTR , 7–9, 11In small children, chemotherapy has been given as a substitute, but the benefit remains uncertain.The present case demonstrates that a very good clinical result may follow after initial surgery, even when incomplete, when followed by local radiation. This is in contrast to our previous experience, and such a good result is not to be expected. The original pathology has been reviewed and confirms the diagnosis of an ependymoma, WHO grade II Fig. .Fig. 2PaThis now 74-year-old man has no clinical symptoms from the posterior fossa, and 3 different MRI studies have not demonstrated any progression with respect to his residual tumor during a 4-year period. The first MRI was taken after an episode of syncope, 54 years after primary treatment. Most probably, his residual tumor has been present ever since the operation in 1954. The situation therefore seems stable and resembles the puzzling situation we have observed in a small group of pediatric patients with recurrent disease decades after primary treatment for distal spinal ependymoma [In 2011, Witt and colleagues described two clinically and molecularly distinct subgroups of posterior fossa ependymoma . Group AThe present case is also in accordance with the statement made by van Veelen-Vincent and colleagues, “The risk of recurrence or progression becomes quite small once the first 5 years after the initial surgery have passed uneventfully, regardless of whether initial surgery was complete or incomplete or whether the patient received radio- or chemotherapy” .Many children who survive treatment for a posterior fossa ependymoma are left with permanent neurological sequelae after aggressive surgical resection and may furthermore suffer from long-term consequences of radio- or chemotherapy . The quaAlthough the prognosis was considered dubious, the boy recovered completely. It is therefore important to notice that a remarkably good result may occur even for a very long time even if a GTR is not achieved.


Pseudolymphoma is a benign lymphocytic tumor-like lesion, and its occurrence in the liver is rare. Here, we report the case of a 78-year-old woman with pseudolymphoma of the liver. She had a history of tremors for several years. Therefore, she underwent computed tomography (CT) for screening, and liver tumors were incidentally identified. She did not have any history of liver disease. Liver function test results and tumor marker levels were all within normal limits, and viral markers for hepatitis were negative. Contrast-enhanced CT revealed four nodules measuring up to 13 mm in diameter with ring enhancement in both lobes of the liver. On magnetic resonance imaging, the lesions showed slightly high intensity on T2-weighted images and high intensity on diffusion-weighted images. Because of atypical imaging findings, the tumors could not be definitively diagnosed. Therefore, we performed laparoscopic limited resection of segments 2, 3, 4, and 8 of the liver. The final pathological diagnosis was pseudolymphoma of the liver. The patient has had no signs of recurrence for 6 months after the surgery. Although pseudolymphoma of the liver is rare, it is necessary to consider it in the differential diagnosis of a liver tumor. Pseudolymphoma or reactive lymphoid hyperplasia is a benign nonspecific lesion characterized by a marked proliferation of polyclonal lymphocytes forming follicles with an active germinal center , 2. AlthA 78-year-old woman with a history of tremors for several years presented to the Department of Neurology in our hospital. She was admitted to our hospital, and she underwent computed tomography (CT) for screening. The CT scan incidentally revealed multiple lesions in the liver; therefore, a qualitative diagnosis was required.3; hemoglobin level, 14.1 g/dL; platelet count, 20.8 × 104/mm3; serum aspartate aminotransferase level, 21 IU/L; serum alanine aminotransferase level, 19 IU/L; alkaline phosphatase level, 204 IU/L; γ-glutamyl transpeptidase level, 37 IU/L; and total bilirubin level, 1.0 mg/dL. The levels of tumor markers, including alpha-fetoprotein, a protein induced by vitamin K absence, carcinoembryonic antigen, carbohydrate antigen 19-9, and soluble interleukin-2 receptor, were all within normal limits. Viral markers for hepatitis B and C were negative. Levels of immunoglobulin (Ig) G, including IgG4, IgA, and IgM were normal. Tests for antinuclear antibody, anti-DNA antibody, and anti-smooth muscle antibody were negative.The results of laboratory examinations, including liver function tests, were all within normal limits. The results were as follows: white blood cell count, 6940/mmImaging revealed four nodules measuring up to 13 mm in diameter in segments 2, 3, 4, and 8 of the liver. Ultrasonography revealed low echoic lesions in segments 2 (13 mm in diameter) and 3 (8 mm in diameter) of the liver. On contrast-enhanced ultrasonography using perflubutane, these lesions showed enhancement in the arterial phase, subsequent washout in the portal phase, and defects in the Kupffer phase. On contrast-enhanced CT, these nodules showed ring enhancement in the arterial phase and subsequent washout in the portal phase. In addition, small nodules with the same enhancement pattern were identified in segments 4 and 8 , metastatic liver tumor (unknown primary origin), malignant lymphoma, inflammatory pseudotumor, and pseudolymphoma. We performed laparoscopic limited resection of segments 2, 3, 4, and 8 of the liver.On visual examination of the resected liver specimens, the tumors appeared well circumscribed and ash colored Fig. . On histWe reported the case of a patient with pseudolymphoma of the liver. Pseudolymphoma is a nodular lesion considered to result from a reactive immunological response; however, the etiology of pseudolymphoma is yet unknown . It was We reviewed the PubMed database from 1985 to 2014, using the keywords “liver,” “pseudolymphoma,” and “lymphoid hyperplasia,” and we found 50 cases of pseudolymphoma of the liver. These cases and our case are summarized in Table Because pseudolymphoma is a lymphoid reaction, it has been suggested that immunological dysregulation is associated with pseudolymphoma . Among tAmong the 51 cases reviewed, 14 (27.5 %) had chronic liver diseases, including primary biliary cirrhosis in 6 cases , 26, 30,Imaging findings of pseudolymphoma resemble those of other vascular tumors of the liver, such as HCC, cholangiocarcinoma, and metastatic liver tumor; therefore, preoperative diagnosis of pseudolymphoma of the liver is extremely difficult with imaging studies alone. In terms of preoperative imaging findings, it has been reported that most pseudolymphomas appear as low echoic lesions with or without well-defined margins on ultrasonography and as lesions having a low density on plain CT . In thisHistologically, pseudolymphoma consists of hyperplastic lymphoid follicles, lymphocytes, and other inflammatory cells, and an accurate diagnosis of pseudolymphoma relies on immunohistochemical analysis . ImmunohThe difference between pseudolymphoma and malignant lymphoma is significant. Although the precise etiology and pathogenesis of pseudolymphoma remain unknown, its prognosis is much better than that of malignant lymphoma, especially follicular lymphoma. Follicular lymphoma includes atypical cells and shows monoclonal proliferation. Additionally, Bcl-2 expression in lymphoid follicles is distinct for follicular lymphoma. Moreover, in situ hybridization demonstrates intermixed kappa and lambda light chains within interfollicular plasma cells. DNA analysis helps to distinguish these lesions from those capable of malignant clonal lymphoproliferation by ruling out monoclonal rearrangements of immunoglobulin heavy chains or T cell receptor beta and gamma genes .In conclusion, pseudolymphoma of the liver should be considered in the differential diagnosis of small hepatic tumors, especially in female patients and patients with no risk factors for hepatocellular carcinoma. Because it is difficult to preoperatively distinguish pseudolymphoma from malignant tumors and metastases on the basis of imaging findings, careful follow-up of patients is essential and surgical intervention should be the choice of treatment on suspicion of malignancy.Consent was obtained from the patient for the publication of this case report.


In this report, however, we present a case of a 7-month-old girl with the focal form of CHI, caused by a loss-of-function mutation in the ABCC8 gene, whose lesion was clearly visible as a hyperenhancing nodule on contrast-enhanced CT and dynamic MRI imaging.The focal form of congenital hyperinsulinism (CHI) is characterized by a cluster of abnormal insulin-oversecreting β cells within a restricted area of the pancreas. Although identification of the focal lesion is very important in the management of CHI, it has been reported that imaging studies, including computed tomography (CT), magnetic resonance imaging (MRI) scans, or angiography, are not helpful in identifying the focal lesion. Currently, fluorine-18-L-dihydroxyphenylalanine positron emission tomography ( Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in the neonatal/infantile period which often requires pancreatectomy when unresponsive to medical treatments –7.ATP channel), KCNJ11 or ABCC8, both located side-by-side in the chromosomal 11p15.1 region ) and length 54 cm (+2.7 SD). Both parents were healthy and there was no family history of hypo- or hyperglycemia. On the second day of life, she presented with hypothermia associated with hypoglycemia. A diagnosis of hyperinsulinemic hypoglycemia was made, based on the characteristic findings at the time of hypoglycemia: plasma glucose 1.6 mmol/L with serum insulin 26.7 pmol/L, undetectable ketone bodies, normal lactate, and normal ammonia. She was unresponsive to diazoxide at a dose of 20 mg/kg/day, and required 8.6 mg/kg/min of intravenous glucose infusion to maintain normoglycemia. From the 15ABCC8 gene . 18F-DOPA PET performed at 4 months of age revealed focal uptake in a single region in the head of the pancreas  instrument, as described in the manufacturer instructions. The study protocol was approved by the Institutional Review Board (No. 743), and written informed consent was obtained from the guardians of the patient.Mutational analysis was performed as described previously . BrieflyATP-channel CHI can be visualized by conventional imaging modalities such as contrast-enhanced CT or dynamic MRI imaging. Although CT scans are not advisable for infants in view of the risk of irradiation, MRI imaging might be worth trying especially where 18F-DOPA PET scans are not easily available.Contrary to the previous belief, our report shows that some of the focal KThe reason why the focal lesion of our patient could be clearly visualized by contrast-enhanced CT and MRI scans is not clear. Generally, for pancreatic nodules to be visualized by these imaging modalities, they need to grow fast enough to have a mass effect. In addition, they need to have a higher vascularity, or higher vascular permeability, to be enhanced by the contrast media. The focal lesion of our patient somehow attained both of these properties. In contrast, the size and cell density of the lesion do not appear to be critical factors for visualization by these techniques , 20.IGF2 gene, although no mutations could be identified in the coding region of IGF2 in our patient (data not shown).Since the tumor suppressor activity of our patient is presumed to be lost as in all other cases of focal CHI, the higher growth potential of the focal lesion in this case might be explained by a difference in the growth promoting activity of the Microscopically, the vascularity in the focal lesion of our patient was not particularly different from that of other patients with the focal form of CHI. However, if the focal lesion has higher growth potential, capillary dilatation without angiogenesis could follow , which mWe report the unprecedented findings in a case of a focal form of CHI whose lesion was clearly visible as a hyperenhancing nodule on contrast-enhanced CT and dynamic MRI imaging. The mechanism leading to the visualization of the focal lesion in our patient needs further investigation.Written informed consent was obtained from the parents of the patient for publication of this Case Report and the accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.