Primary hepatic leiomyosarcoma is exceedingly rare accounting for less than 1% of the hepatic tumors. Close to 45 cases have been reported in the English literature. Presentation is usually nonspecific and diagnosis is often delayed until tumors reach a large size. This leads to a dismal prognosis. The tumors are not yet fully understood, hence the standard of care is not well defined. Curative resection remains the mainstay of management. Close association of Epstein Barr virus (EBV) induced soft tissue sarcomas is proven, especially in the presence of immunosuppression encountered in HIV/AIDS patients and in posttransplant patients. We herein present a case report of a 54-year-old man diagnosed to have HIV/AIDS and EBV infection admitted to our hospital with complaints of intractable hiccups for more than a week. Extensive workup revealed primary leiomyosarcoma of the liver. Sarcomas are rare mesenchymal tumors and account for 1% of all adult tumors, of which 5–10% are leiomyosarcomas. Hepatic leiomyosarcomas are unusual tumors. Most of them are metastatic leiomyosarcomas. Primary hepatic leiomyosarcoma is a very rare entity. Primary leiomyosarcomas are known to occur in uterus, retroperitoneum, genital organs, major blood vessels of the body, lungs, and liver [A 54- year- old African American man presented to our hospital with intractable hiccups that started about a week prior to admission. He also reported intermittent low grade fever with poor oral intake and weight loss of 9 kilograms for the last 3 months. He denied abdominal pain, nausea, vomiting, or irregular bowel habits. His comorbidities included HIV/AIDS, seizure disorder, and hypertension. His CD4 count was 24 at the time of admission. He had no history of liver disease or alcohol abuse. He never had any surgery. Family history was negative for any malignancies or liver disease. On physical examination he appeared cachectic. He had no palpable lymph nodes. Abdominal examination revealed minimal tenderness in the right upper quadrant and hepatomegaly of about 6 cm below the right costal margin. Laboratory analysis revealed alanine aminotransferase 81 IU/L (0–34 IU/L), aspartate aminotransferase 56 IU/L (0–40 IU/L), alkaline phosphatase 160 IU/L (28–94 IU/L), serum albumin 2.8 g/dL (3.4–5 g/dL). Serum bilirubin, prothrombin time, white blood cells, and platelets were normal. Septic workup including blood and urine cultures and chest radiograph were normal. Abdominal computed tomography (CT) of the chest and abdomen showed a hypodense rim enhancing lesion about 3.5 cm by 2.5 cm in size with a necrotic center within segment VII  and anotSarcomas constitute about 1-2% of malignant tumors of the liver . The mosMean age of presentation as identified from various case reports is 58 years . HepaticThe findings of hepatic leiomyosarcoma on CT scan and MRI are non-specific. These tumors are found to be hypervascular on imaging. CT may reveal a large, well-defined, heterogeneous hypodense mass with internal and peripheral enhancement or cystic mass with an enhancing thick wall –10. MR fAssociation of EBV infection and increased incidence of leiomyosarcoma in immunosuppressed patients such as HIV/AIDS and posttransplant is clearly documented and well researched in the literature. In situ hybridization studies of leiomyoma and leiomyosarcoma in HIV patients demonstrate EBV in the tumor cell, but not the adjoining normal cells. In AIDS patients the EBV receptor (CD21/C3d) may be upregulated allowing EBV to enter into muscles cells, contributing to malignant transformation. Similar pathogenesis of EBV induced leiomyosarcoma in iatrogenic immunosuppressed patients, especially in post-transplant patients, has been reported. There are isolated reports of decreased incidence of recurrence of leiomyosarcoma in EBV infected patients, in whom immunosuppression is decreased after transplantation.  If liver lesion appears malignant and deemed to be surgically resectable, there is no need for preoperative biopsy. In our case there was concern for abscess and hence CT guided aspiration was performed. Biopsy will reveal intersecting bundles of spindle cells with immunoreactivity to desmin, actin, and SMA but negative for keratin, S-100 protein, and neuron-specific enolase . As in oTherapeutic interventions include surgical resection, systemic chemotherapy, and liver transplantation. Best treatment modality is surgical resection with tumor-free margins which has good long term survival , 17. In Patterns of sites of recurrence of these tumors are similar to other sarcomas. Hepatic leiomyosarcoma tends to metastasize hematogenously to the lung, but lymphatic metastasis and peritoneal seeding have been described. Adjuvant chemoradiotherapy had been recommended for eradication of microscopic residual disease. However the success of this therapy in achieving local control and prolonged patient survival needs to be further studied although a meta-analysis has shown a small benefit . PostopeThe role of liver transplantation remains controversial. In the study by Husted et al., six patients with primary hepatic angiosarcoma and thirteen patients with metastatic sarcoma underwent liver transplantation and the 1-, 3-, and 5-year survival was 47%, 15%, and 5%, respectively. Recurrence rate was 95% after a median interval of 6 months. Given the high tumor recurrence rate and poor survival, Husted et al. discouraged liver transplantation for the management of primary or metastatic sarcoma of the liver . HoweverHigh index of suspicion and prompt investigation will help to diagnose primary hepatic leiomyosarcoma at an earlier stage. Surgical resection without any residual tumor has clearly been demonstrated for prolonging patient's survival. Use of neoadjuvant, adjuvant chemoradiotherapy, and hepatic artery chemoembolization needs to be further evaluated.


A lymphangioma is a benign proliferation of lymph vessels, producing fluid-filled cysts that result from a blockage of the lymphatic system. The incidence of abdominal lymphangiomas is unknown; however they account for from 3% to 9.2% of all pediatric lymphangiomas, with retroperitoneal lymphangioma representing less than 1% of abdominal lymphangiomas. Due to rarity, preoperative diagnosis is often difficult. The incidence of abdominal lymphangiomas is unknown: however they account for from 3% to 9.2% of all pediatric lymphangiomas, with retroperitoneal lymphangioma representing less than 1% of abdominal lymphangiomas , 5. DiffA 8-years-old girl presented to the emergency room with abdominal pain, nausea, and vomiting at another hospital. Ultrasonography of the abdomen revealed a thin-walled, multiloculated, anechoic cystic mass with septations occupying almost left quadrant of the abdomen. The initial diagnosis was mesenteric cyst. One day after the patient has consulted at our hospital. On contrast-enhanced CT Figures , and 3 wAfter stabilisation of the child midline laparotomy was performed and there was neither intraabdominal located mass nor mesenteric cyst. Exploration revealed a retroperitoneal cystic structure beneath the stomach. Retroperitoneal space was explored, dividing gastrocolic ligament, and there was an approximately 20 × 15 cm cystic mass, which showed strong adhesions to the distal part of the pancreas, to the lower pole of the spleen and to the posterior surface of the stomach. Meticulous preparation of the mass was performed and complete excision was achieved without any partial resection of the associated organs. Pathology confirmed the diagnosis of the cystic lymphangioma. Postoperative course was uneventful and the child discharged on postoperative day 5. The lymphatic system is derived during the third or fourth fetal month from 2 paired and unpaired endothelial channels proliferate centrifugally from these sacs, which are located in the neck, mesenteric root, and bifurcation of the femoral and sciatic veins . A lymphOn cut section, they vary in appearance and may contain large macroscopic interconnecting cysts often referred to as cystic hygroma or cystic lymphangioma or microscopic cysts cavernous lymphangioma . The cys Radiologic diagnosis of retroperitoneal masses is often made using ultrasound imaging or computed tomography (CT). Sonographically, lymphangiomas are most often multilocular cystic masses that are anechoic or contain echogenic debris. Intravenous contrast-enhanced CT may show enhancement of the cyst wall and septa . The fluThe sonographic appearance of a septated cystic mass with clear fluid is supposed to be a characteristic of a lymphangioma . HoweverDiagnostic important point for cystic lymphangioma: an elongated shape and a crossing from one retroperitoneal compartment to an adjacent one. Also at CT, cystic lymphangioma typically appears as a large, thin-walled, multiseptate cystic mass , 13. A tThe treatment of choice is complete surgical excision. The long-term prognosis is excellent if complete excision has been achieved. If not the child should be followed up with serial ultrasound to exclude recurrence of the lesion. An alternative treatment available is image-guided percutaneous catheter drainage of lymphangioma followed by sclerotherapy .


Pneumoperitoneum in the newborn is an acute surgical emergency requiring immediate surgical intervention to ensure survival. It refers to radiological evidence of rupture of an air-containing viscus with resultant soiling of the peritoneal cavity. A female baby was born preterm at 29 weeks with birth weight of 650 grams. She developed abdominal distension on day 6, and abdominal radiography revealed presence of free air in the peritoneum. She proceeded for a laparotomy, and intraoperative findings revealed blood in the peritoneum with an area of inflammation and a small perforation. About 5 cm of the inflamed bowel was resected, and an end to end anastomosis performed. The histopathology of the specimen was consistent with Meckel's diverticulum. Symptomatic Meckel's diverticulum is usually seen in the first two years of life, and perforation is a rare presentation. Perforated Meckel's diverticulum in a premature newborn is very rare, and a review of literature reveals only one other reported case. Newborn Meckel's perforation cases often mimic necrotizing enterocolitis, although many present without any feature of peritonitis. Establishing a preoperative diagnosis of perforated Meckel's is difficult and may not be essential as the treatment remains the same. However, prompt surgical intervention confers a good prognosis in neonates with isolated perforated Meckel's diverticulum. Meckel's diverticulum (MD) is the commonest congenital abnormality of the small intestine with prevalence of 2 percent in the general population. Most complications manifest in childhood, usually beyond the age of 4 months . It is uA live female baby was born at 29-week gestation with a birth weight of 650 grams. The mother was a 30-year old G2P0, and her serology was negative. She was a smoker and had abnormal Doppler flows in the umbilical vessels. The antenatal scans revealed intrauterine growth restriction (IUGR), and she received a full course of antenatal steroids at 28 weeks. She was hospitalised for reduced foetal movements and proceeded to deliver by an emergency LSCS for a pathological CTG. She was born with Apgar scores of 6 and 9. She received surfactant and was extubated to CPAP. She had a symptomatic patent ductus arteriosus on day 3 of life requiring inotropic support with dopamine and had successful closure following treatment with a 3 doses of IV ibuprofen. On day 6 of life she had a clinical deterioration with a distended abdomen. An abdominal X-ray revealed presence of free air in the peritoneum, and she proceeded to theatre for a laparotomy on suspicion of a gut perforation. Intraop findings revealed blood from the stomach to the midileum with a large area of inflammation and perforation. About 5 cm of the inflamed bowel was resected, and an end to end anastomosis performed. The histopathology of the specimen was consistent with Meckel's diverticulum. She recovered well from this surgery but demised following a suspected aspiration episode 5 weeks later.E. coli sepsis. The median age at presentation was 4.5 (±4.3) days [Symptomatic Meckel's diverticulum usually occurs in the first two years of life. A perforation of the Meckel's diverticulum is less common and rare in the neonatal period. Diverticular length and base diameter are well known predisposing factors to complications, and long, narrow-based diverticula are thought to be more prone to obstruction or inflammation . Bleedin Pneumoperitoneum in the newborn is an acute surgical emergency with serious implications requiring immediate surgical intervention to ensure survival. The term “pneumoperitoneum” refers to radiological evidence of rupture of an air-containing viscus with resultant gross soiling of the peritoneal cavity . Very feA review of literature reveals only one other reported case of perforated Meckel's diverticulum in a micropremie. That infant had a significant number of factors that may have predisposed him to intestinal perforation, including antenatal and postnatal steroid therapy, hypoxia, and poor intrauterine blood flow . Our patPerforated Meckel's diverticulum in a premature newborn is very rare and can present with a pneumoperitoneum. Establishing a preoperative diagnosis of perforated Meckel's diverticulum is difficult and is not essential as the treatment remains the same, which is a surgical laparotomy. Prompt surgical intervention usually confers a good prognosis and intact survival in neonates with isolated perforated Meckel's diverticulum.


The current study presents the case of a patient with a recurrent carcinoid tumor of the ovary, 13-years after the primary surgery. The primary surgery consisted of a total abdominal hysterectomy and bilateral salpingo-oophorectomy for a left ovarian tumor at 54 years old. Pathologically, the tumor was diagnosed as a carcinoid tumor of the ovary. Following the primary treatment, the patient was admitted to a cardiologist due to carcinoid-induced heart failure. At 67 years old, the patient was referred to Kyoto University Hospital with a solitary mass 8 cm in diameter and located in the paraaortic area, which was detected by routine ultrasonography and subsequent computed tomography (CT) scans. Urinary 5-hydroxyindole acetate (5-HIAA), a serotonin degradation metabolite, was present at elevated levels. With a diagnosis of a recurrent carcinoid tumor, the patient underwent a tumor resection. The pathological diagnosis was that of lymph node metastasis of the trabecular carcinoid. Post-operatively, the 5-HIAA levels returned to normal. Carcinoid tumors occasionally recur following surgery due to borderline malignant potential. Due to the slow growing nature of these tumors, in specific cases, recurrence occurs following a long interval. Therefore, a relatively long follow-up period is required. The patient was also diagnosed with heart failure. The individual underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. The pathological diagnosis was of a trabecular carcinoid tumor of the left ovary with positive ascites cytology. The patient then underwent one cycle of intraperitoneal chemotherapy and three cycles of systemic chemotherapy (detailed information on the treatment was not available). The post-operative serum levels of serotonin and urinary 5-hydroxyindole acetate (5-HIAA) were normal. The heart failure was diagnosed as heart carcinoid disease. The patient was treated with medication prescribed by the cardiology department and ceased attending any gynecology appointments.At 67 years old, an abnormal paraaortic tumor was identified by ultrasound screening at an internal medicine clinic, at which point the patient was referred to hospital. The patient did not complain of any typical carcinoid syndrome symptoms, which include skin flushes, diarrhea, abdominal cramping, peripheral edema or tachycardia. A computed tomography (CT) examination revealed a solitary tumor 30×30×77 mm in size in the paraaortic area , while tNeither chemotherapy nor radiation is considered to be an effective for treating carcinoid tumors. Although the patient in this case had carcinoid heart disease, a surgical resection was performed  based onPrimary carcinoid tumors of the ovary are rare and constitute 0.5–5% of all carcinoid tumors and <0.1% of all ovarian malignancies . The hiset al previously reported 4 cases of ovarian carcinoid, which presented signs of right-sided heart failure and required surgical replacement of the valve on the right side  levels is a marker of a favorable survival rate (Following the primary treatment, the patient did not receive regular follow-ups with a gynecologist concerning the carcinoid tumor. However, patients with carcinoid tumors, and particularly those with cardiac dysfunction, should see a gynecologist and a cardiologist for the early detection of any possible recurrence. No effective treatment exists for carcinoid tumors, with the exception of surgical resection. Therefore, it is particularly important to detect recurrence early to ensure that surgical removal is viable and that the heart function is able to tolerate the surgery. Urinary 5-HIAA is the most reliable follow-up marker of serotonin producing-carcinoid tumors. van der Horst-Schrivers val rate –15. Howeval rate . In the val rate . In addival rate . Octoreoval rate . In this


Gastric pneumatosis (GP) and hepatic portal venous gas (HPVG) have typically been thought of as an ominous radiological sign associated with a grave prognosis, and the observation of HPVG on plain abdominal radiography, ultrasonography, or computed tomography is viewed as a significant finding. It is often associated with severe or potentially lethal conditions warranting urgent diagnosis and possible surgical intervention. Early studies of HPVG based on plain abdominal radiography found an associated mortality rate of 75% primarily due to ischemic bowel. However, modern abdominal computed tomography (CT) has resulted in the detection of HPVG in an increased proportion of nonfatal and benign conditions. We report a nonfatal case of HPVG in a patient with Noonan's syndrome due to acute gastric dilatation in the setting of gastric outlet obstruction caused by a congenital band that is extremely rare in adults. Hepatic portal venous gas (HPVG) was first described more than 55 years ago by Wolfe and Evans and was presented as a radiologic sign associated with more than 75% of mortality rate in infants with necrotizing enterocolitis (NEC) , 2. Sinc9/L (3.9–11.1), hemoglobin 14.9 gm/dL, hematocrit 43.7%, platelets 322,000, sodium 145 mEq/L, potassium 2.8 mEq/L, chloride 83 mEq/L, bicarbonate 49 mEq/L, BUN 32 mg/dL, creatinine 1.90 mg/dL, glucose 188 mg/dL, calcium 11.9 mg/dL, albumin 4.3 g/dL, total bilirubin 0.3 mg/dL, alanine aminotransferase (ALT) 11 U/L, aspartate aminotransferase (AST) 27 U/L, and alkaline phosphatase of 80 U/L. The patient underwent a CT scan of the abdomen and pelvis that showed marked gastric dilatation with extensive circumferential gas (pneumatosis) and portal venous gas suspicious for gastric ischemia. The likely etiology for these findings was the presence of gastric outlet obstruction (Figures A 64-year-old Caucasian female with a history of Noonan's syndrome presented with sudden onset of coffee ground emesis and without any history of black tarry stool. She was recently treated for severe erosive gastritis during her last hospital admission, and an endoscopy done during that admission suggested Los Angles Class C erosive gastritis with an otherwise normal stomach and duodenum. Her past medical history is significant for a history of hypercoagulability, bilateral lower extremity deep venous thromboses, extensive bilateral pulmonary embolism, hyperlipidemia, hypertension, and dextrocardia. She complained of vague upper abdominal discomfort, and on further clinical examination, her epigastric region was soft, and distended and demonstrated no rebound. She was hemodynamically stable. Blood laboratory investigations revealed the following: white count 21.2 × 109/L .9–11.1, Since the first description of HPVG in the literature more than half a century ago, there has been no clear evidence identifying the exact nature of the gas observed in imaging studies. However, the leading hypotheses are  microbe-Hepatic portal venous gas is recognized not as a specific disease entity but rather as a diagnostic clue in patients with underlying acute abdominal pathology. HPVG can be visualized at conventional radiography, but substantial amounts of gas must be present for detection . HPVG isMost cases of HPVG are caused by mesenteric vascular occlusion and subsequent bowel necrosis. HPVG can occur alone or in association with PI. When associated with PI, the origin of the gas seems to be intestinal ischemia. However, HPVG is not related to the severity of intestinal ischemia  and has We report a case of acute gastric dilatation that caused HPVG and gastric emphysema, which was treated by bypassing the obstruction. Our case is unique in that the diagnosis of gastric emphysema as the cause of portal venous gas occurred in the setting of acute gastric dilatation caused by a congenital band that is extremely rare in adults and related to the abnormal anatomy of this patient with Noonan's syndrome. While HPVG in previous decades was clearly defined as an ominous radiologic finding that portended a poor prognosis and a high mortality rate, today it can be a puzzling finding to reflect a benign etiology. The radiologic presence of HPVG does not necessarily indicate severe underlying pathology. It can be seen in relatively benign situations, such as following endoscopic procedures and gastric dilatation, and may require only conservative therapy. Treatment of patients with HPVG depends mainly on the underlying disease. Nowadays, with the development of advanced imaging techniques such as CT, severe pathologies such as bowel ischemia are diagnosed at much earlier stages, allowing prompt treatment and significantly reducing mortality rates.


Introduction. Retroperitoneal sarcomas comprise a small proportion of all soft tissue sarcomas, and multiple factors influence their clinical behavior. Histopathological type and grade as well as complete surgical resection especially on the first operative attempt are well recognized as the main prognostic factors. Multifocality is another prognostic factor, which compromises therapy and finally makes prognosis worse due to multiple adverse implications. Case Presentation. A rare case of a 65-year-old male patient suffering from a multifocal retroperitoneal liposarcoma successfully treated in our hospital is presented herein. Discussion. Also, general considerations for these tumors are discussed, and especially multifocality is underlined as an ominous sign of retroperitoneal sarcomas behavior. Despite multifocality, once again complete surgical excision remains the mainstay of treatment of these patients, as long as further systemic and local therapies do not provide durable results.  Sarcomas are uncommon neoplasms of mesenchymal origin accounting for 1% of all solid tumors, while retroperitoneal sarcomas (RPS) are even less often found accounting for 10% of all soft tissue sarcomas . MultiplA 65-year-old white male complaining of mild abdominal discomfort and nonspecific abdominal pain, as well as a swelling in his left inguinal area, underwent an abdominal computed tomography (CT) scan . A largeThe overall incidence of RPS is 0.3-0.4% per 100,000 of the population, and usually patients are on their fifth to sixth decade of life . RetropeApproximately, 11% of patients who have a primary retroperitoneal sarcoma also present with metastatic disease, with lung mostly and liver secondly, being the most common site of metastasis. Imaging is of paramount importance because staging, surgical plan, and sometimes image-guided biopsy are based on it. Abdominal CT scan is the most important tool for demonstrating an RPS and its relation with vital structures, great vessels, and neighboring organs that may need to be resected en bloc, and for the case of liposarcoma, can predict the histologic type and even the grade of the tumor . CT scanTreatment is based on surgical resection as other options like chemotherapy and radiotherapy have poor results and can be regarded only in adjuvant and rarely neoadjuvant setting. In various series, complete resection rates vary from 54–93% [However, even when RPS is surgically completely resectable (R0), the main challenge is the high rate of local recurrence, which results in poor overall survival . In receIn a previous report from our institution, the incidence of multifocal disease was 20.3% for primary RPS which is comparable with a recent series from MD Anderson Cancer Center , 11. MulFor all the above, resection of RPS through an extended laparotomy with wide en bloc excision of surrounding soft tissues seems absolutely necessary. The existence of “satellite tumors” on the preoperative CT scan may be used as a guide for appropriate surgical approach and an extension of resection and may serve as a prognostic indicator .


Rosai–Dorfman disease (RDD) is a rare proliferative histiocytic disorder of unknown etiology. RDD typically presents with generalized lymphadenopathy and polymorphic histiocytic infiltration of the lymph node sinuses; however, occurrences of extranodal soft tissue RDD may rarely occur when masquerading as a soft tissue sarcoma.A comprehensive search of all published cases of soft tissue RDD without associated lymphadenopathy was conducted using PubMed and Google Scholar for the years 1988 to 2011. Ophthalmic RDD was excluded.Thirty-six cases of extranodal soft tissue RDD, including the current one, have been reported since 1988. Anatomical distribution varied among patients. Four (11.1%) patients presented with bilateral lesions in the same anatomic region. Pain was the most common symptom in six (16.8%) patients. Sixteen (41.6%) patients were managed surgically, of which one (2.8%) case experienced recurrence of disease.RDD is a rare inflammatory non-neoplastic process that should be considered in the differential diagnosis of a soft tissue tumor. Thus, differentiation of extranodal RDD from more common soft tissue tumors such as soft tissue sarcoma or inflammatory myofibroblastic tumor is often difficult and typically requires definitive surgical excision with histopathological examination. While the optimal treatment for extranodal RDD remains ill-defined and controversial, surgical excision is typically curative. Sinus histiocytosis with massive lymphadenopathy (SHML) is a class II histiocytosis first described as a unique clinicopathologic entity by Rosai and Dorfman in 1969 . The disA 56-year-old African American female presented to the Saint Barnabas Medical Center  with a 1-year history of an enlarging painful right medial thigh mass. Her medical history was significant for hypertension, diabetes, hypercholesterolemia, degenerative disk disease and asthma. The mass was noted to be extremely painful and was located anterior and superficial to the adductor muscle group in the right medial thigh. The patient reported a pulling, painful sensation in the knee joint as well as discomfort upon standing for long periods of time. She denied any constitutional symptoms and had no reported neurological deficits. No weight loss was reported. A chest, abdomen, and pelvis computed tomography scan was performed, demonstrating no systemic adenopathy. A magnetic resonance imaging study of the right lower extremity was obtained and revealed a 7 cm × 5 cm × 3.2 cm ill-defined, enhancing soft tissue mass, suspicious for a soft tissue sarcoma located in the deep subcutaneous tissues of the right thigh, adjacent to the vastus medialis muscle . CD1a immunohistochemical staining was not performed on the excisional biopsy tissue.A comprehensive search of all published cases of soft tissue RDD without associated lymphadenopathy was conducted using PubMed and Google Scholar for the years 1988 to 2011. Ophthalmic RDD was excluded. Among reported cases of Rosai–Dorfman tumors, 43% of patients had extranodal disease with associated lymphadenopathy; however, only 3% of the patients had soft tissue RDD without detectable lymph node involvement ,6. OveraA class II histiocytosis, RDD was first described as a distinct clinicopathologic entity by Rosai and Dorfman in 1969 . RDD exhin situ hybridization in seven of nine SHML cases, while Luppi and colleagues had demonstrated human herpes virus-6 antigen expression by abnormal histiocytes [in situ hybridization; however, this was detected in only one of nine SHML cases, suggesting that Epstein–Barr virus infection is probably not causative but may be a contributing factor to the development of RDD [RDD is of unknown etiology, although viral agents such as human herpes virus-6 and Epstein–Barr virus are thought to play a role in the pathogenesis via immune system dysregulation ,18. Levitiocytes ,20. Botht of RDD . Yoon ant of RDD . Howevert of RDD .RDD is classified as either nodal or systemic . RDD typically presents insidiously with generalized lymphadenopathy and a polymorphic histiocytic infiltration of the lymph node sinuses. The cervical lymph nodes are most commonly affected, followed by inguinal, axillary and mediastinal lymph node basins . RDD maySystemic RDD is more prevalent and is characterized by tumors at other sites such as bone, upper respiratory tract, skin and retro-orbital tissue . The cutThe differential diagnosis for RDD is challenging and is based on clinical features as well as immunohistological analysis and radiographic features. Lymph nodes tend to be hypermetabolic and positive on positron emission tomography. Upwards of 80% of patients demonstrate polyclonal hypergammaglobulinemia and as many as 65% have a hypochromic or normochromic normocytic anemia . HistoloThe differential diagnoses of RDD-type lesions include lymphoreticular malignancies when cervical lymphadenopathy is present or soft tissue sarcomas when patients present with extranodal disease. Table Soft tissue RDD is particularly challenging to diagnose since it is often difficult to discern the exact morphology of soft tissue samples. Extranodal soft tissue RDD usually demonstrates a spindled morphology with abundant collagen deposition resulting in the hallmark emperipolesis to become more inconspicuous . FurtherDue to the rarity of the disease and its self-limited course, no treatment protocol has been established for RDD . In sympAlthough RDD in extremities had been described in a limited number of cases, this case highlighted the importance of better differentiation from more common malignancies. The painful symptoms experienced both before and after resection in the current case were uncharacteristic, as most patients with extranodal RDD had experienced pain-free results following resection. Furthermore, the development of bilateral disease in a different site had been only rarely reported ,5,14,16.In summary, a defined treatment strategy for RDD has not been well described, given the rarity of the lesions and the difficulty in diagnosing them preoperatively. To date, surgical resection has proven most successful in preventing recurrences. Only one case of local recurrence for extranodal soft tissue RDD following surgical resection has been reported. However, as in the current case, bilateral disease presentation is also possible and requires close clinical follow-up. Given the rarity and indolence of RDD, surveillance cannot be endorsed; however, it is important to consider extranodal soft tissue RDD amongst the differential diagnosis of patients presenting with suspicious soft tissue masses. Future studies may help to elucidate the natural history of this disease process, as well as the possibility for malignant potential, thus permitting the development of more evidence-based treatment strategies. Until then, known RDD lesions should be excised using established surgical principles.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-in-Chief of this journal.H & E: Hematoxylin and eosin; IFN: Interferon; LCH: Langerhans cell histiocytosis; RDD: Rosai–Dorfman disease; SHML: Sinus histiocytosis with massive lymphadenopathy.The authors declare that they have no competing interests.MK and LSS reviewed the literature and drafted the manuscript. RSC was clinically responsible for the patient’s care and revision of the manuscript. MD and MLS were responsible for the pathology. All authors read and approved the final manuscript.


The involvement of the ovary by malignant lymphoma is a well-known late manifestation of disseminated nodal disease. Primary ovarian lymphoma is rare. We herein describe a case of primary ovarian diffuse large B-cell lymphoma involving unilateral ovary in a 38-year-old woman which was detected incidentally. Preoperative ultrasonic imaging showed a 46∗42 mm heterogeneous cystic mass. Laparotomy revealed that left adnexal mass and left salpingo-oophorectomy was performed. The current diagnosis was determined after immunostaining. The patient was treated with R-CHOP regimen after the operation. She remains cancer-free 24 months after chemotherapy. Lymphomas presenting as ovarian tumors are uncommon and may occur as de novo or secondary as a part of systemic disease . Primary A 38-year-old woman, gravida 4, para 4, was admitted to the hospital with complaints of irregular bleeding from the vagina for two-month duration, and she also had a desire of a tubal ligation. A physical examination, pelvic exam, and digital rectal examination were normal. Routine hematology and chemistry showed no abnormality. Serum carcinoembryonic antigen, cancer antigen 125, and cancer antigen 19–9 were within normal limits. Cancer antigen 15–3 was positive 30, 33 . Ultrason examination showed a 46∗42 mm heterogeneous cystic mass surrounded by thick wall and containing intense fluid in the left ovary whereas the right ovary was normal. Because of the ultrasonographic appearance and cancer antigen levels, this cystic mass was evaluated as benign and no further investigation was done. Laparatomy was performed. The patient was explored with a pfannenstiel incision. Laparatomy revealed that left adnexal mass measuring 5 cm was coarse and consisting of solid component. So left salpingo oophorectomy was performed. Peritoneal surface was clean and there was no ascetic fluid accumulation. Right ovary and fallopian tube were normal.2 day; cyclophosphamide, 750 mg/m2 day; doxorubicin, 50 mg/m2 day; vincristine, 1.4 mg/m2 day; prednisone, 50 mg/m2 day) 6 times intravenously and treatment progressed well. The patient is alive without disease 24 months after the operation without additional surgery. The histology of the specimen was malignant but suspicious for undifferentiated carcinoma. The definite pathologic diagnosis after immunostaining was diffuse, large-cell malignant lymphoma of B-cell lineage. Work-up for the lymphoma after the surgery included abdominal and thoracic magnetic resonance imaging, bone marrow biopsy, and a whole body positron emission tomography scan. The findings from all these studies showed no other sites involved. The patient later received an adjuvant R-CHOP chemotherapy  Figures . Reactiv Non-Hodgkin lymphoma rarely involves the gynecologic tract. However, when involved, the ovary is one of the more commen anatomic sites . True pr The presence of positive staining for leukocyte commen antigen distinguishes malignant lymphoma from nonlymphoid neoplasms. In our case tumor cells were positive for LCA and B cell CD20. The difference between primary and secondary lymphomas is important in terms of prognosis. The initial clinical manifestation of an occult nodal lymphoma as an ovarian mass is known as having a poor outcome with a survival rate ranging from 7% to 38% at 5 years. But primary ovarian lymphoma has better prognosis . The his Primary ovarian lymphomas are staged as other extranodal NHLs (Ann Arbor staging system) . Treatme The question of whether some ovarian lymphomas can be considered truly primary in the ovary and not merely a localized initial manifestation of a generalized disease cannot be answered yet. But Fox et al.  have sugFerrozzi et al.  reportedYamada et al.  report a To conclude, if stringent criteria are used primary ovarian malignant lymphoma is a very rare disease. It is diagnosed after the operation using immunohistochemistry and treated with chemoteraphy. The authors experienced a case of a primary ovarian lymphoma presenting a heterogeneous cystic mass. Patients with localized disease and B cell phenotype have a good prognosis.


Adenocarcinoma of the cervix is a rare condition that has shown an increase in incidence, especially in the 20- to 34-year-old group. Adenocarcinoma represents about 5–10% of all tumours in this area, and, among these, the clear cell type accounts for 4–9%. This type of tumour affects mainly postmenopausal women but also occurs in young women with a history of prenatal exposure to diethylstilbestrol (DES). The prognosis for adenocarcinoma of the cervix is poor overall and worse for the clear cell variety. This article discusses a case of clear cell adenocarcinoma of the cervix, unrelated to intrauterine exposure to DES, in a woman of childbearing age who wished to preserve her fertility and was therefore treated by radical vaginal trachelectomy and pelvic lymphadenectomy. Worldwide, cervical cancer has an annual incidence of 493,000 cases . Of the Adenocarcinoma of the uterine cervix is a rare type of tumour, although the incidence of these tumours has increased in recent years, particularly in the 20- to 34-year-old age group . AdenocaA 28-year-old woman, nulligravida, presented at our cervical disease clinic with a suspected low-grade squamous intraepithelial lesion (LGSIL) and human papilloma virus (HPV), positive cytology (carried out at another centre), and bleeding during intercourse for the last year. She had no substance abuse or significant medical or family history.A 2-cm vascularised tumour outgrowth, indurated and bleeding, was found on investigation ; colposcFollowing this, a further investigation was carried out on the patient, including gynaecological examination, gynaecological ultrasound, laboratory tests, and tumour markers, which were all normal. Magnetic resonance imaging (MRI) was also carried out and revealed a cervical lesion 25 mm in diameter with no infiltration into the parametria .A diagnostic cone biopsy was performed; the histopathological result was a clear cell adenocarcinoma measuring 20 x 15 mm with 3-mm stromal infiltration, clear margins, and no lymphovascular invasion. The tumour stage was determined to be International Federation of Gynecology and Obstetrics (FIGO) IB1; since the patient wanted to preserve her fertility, she was offered a radical trachelectomy with selective biopsy of the sentinel lymph node (SLN).The SLN technique included injection at the four cardinal points of the cervix, on the day before surgery, of Tc-99m  and lymphoscintigraphy prior to surgery. Subsequently, in the operating theatre, 4 mL of undiluted methylene blue was injected into the same points (1 mL at each point), and one lymph node was identified in the external iliac vessels on either side. These were removed laparoscopically; intraoperative analysis found them to be negative .Since our centre is involved in a validation study on SLNs in cervical cancer, a bilateral laparascopic pelvic lymphadenectomy was then performed. Next, an RVT was carried out, with no complications . The finThree months later, the patient was symptom free; an MRI showed a 3-cm lymphocoele in the left groin, which had abated at the next review.The patient was followed up quarterly at the gynaecological oncology clinic, with a gynaecological examination, vulvoscopy, colposcopy, and laboratory tests; at six months after the RVT, the gynaecological ultrasound and MRI showed no further effects. After 18 months of follow-up, the patient is still symptom free and is planning a pregnancy.Cervical cancer is the second most common cancer in Europe in women of childbearing age. More than 40% of stage I patients are under 40 years of age. There is a documented trend toward delaying motherhood to the late 30s and early 40s, leading to a higher proportion and more patients being diagnosed with cervical cancer before having started or completed their family. The review by Leitao demonstrates that a very high proportion of patients with gynaecological cancer are under 40 years of age [This intervention includes amputation of the cervix below the uterine isthmus, with removal of parametrial tissue and bilateral pelvic lymphadenectomy. In selected cases, it may be decided to carry out a biopsy of the SLN, as in our case.The selection criteria for radical trachelectomy (with some variability from one author to another) are a strong desire for pregnancy, confirmed diagnosis of cervical cancer , tumour size equal to or less than 2 cm, less than 50% stromal invasion, FIGO stage IA1 with LVI, IA2 or IB1, absence of any evidence of metastasis in or away from the pelvic lymph nodes, and age under 40 years –10.Our clinical case met the inclusion criteria, as the patient was a woman of childbearing age who had a strong desire to start a family, with stage IB1 clear cell adenocarcinoma; therefore, radical trachelectomy would be the technique of choice.More than 900 cases have been published in the literature, all performed at a small number of specialist centres. The recurrence rate and mortality following this procedure are 4.2% and 2.8%, respectively, which are similar to those offered by radical hysterectomy without fertility sparing [The SLN technique would allow pelvic lymphadenectomy to be avoided, thus reducing complications and morbidity .The most important factors for disease prognosis are the tumour stage, size, growth pattern, nuclear atypia, and mitotic activity. In the case of clear cell carcinoma at stages I–II, five-year survival does not exceed 60%, when considering this subtype of poor prognosis .It is not completely clear whether clear cell cervical adenocarcinoma has a worse prognosis than squamous cell cancer, and therefore in our case, we decided not to exclude the patient for a fertility-sparing technique –15.Korhonen suggested, having analysed 163 cases of primary cervical adenocarcinoma of different subtypes, that the prognosis for clear cell and non-clear cell adenocarcinoma is similar .However, in a review of the literature, Niibe suggests that the five-year survival rate in cervical adenocarcinoma is worse than that in squamous cell cancer . All of In this case, the FIGO tumour staging was IB1, and the standard treatment for these initial stages is radical hysterectomy together with pelvic lymphadenectomy, but in view of the patient’s young age, it seemed reasonable to attempt fertility-sparing treatment.et al in 2007 (17). In his work, Landoni analyses his experience from 1995 to 2007, in a total of 21 patients. Twenty-one patients aged < 40 years, with tumours measuring < 3 cm, received three cycles of chemotherapy with cisplatin 75 mg/m2, paclitaxel 175 mg/m2, and ifosfamide 5 g/m2, followed by conisation and pelvic lymphadenectomy.When the selection criteria are not met, another fertility-sparing option is neoadjuvant chemotherapy followed by conisation; this technique was first proposed by Landoni After 69 months of follow-up, the authors observed no recurrence, and there were nine pregnancies; they therefore concluded that neoadjuvant chemotherapy could reduce tumour volume and allow safe removal of the cervical cone .There are few references in the literature to obstetric results after radical trachelectomy. In his review, Boss analysed a total of 355 patients on whom he performed RVT, observing that only 43% wished to become pregnant and, of these, 70% were successful. Losses in the first trimester were 21%, comparable with that of the general population, but losses in the second trimester were 8%, almost double that of the general population, with 42% full-term pregnancies .To prevent the obstetric complications associated mainly with radical trachelectomy, some authors such as Shepherd and Plante suggest waiting for six months to one year before trying to get pregnant , 20. OthIn conclusion, treatment for women with early-stage cervical cancer can include fertility sparing, always on a personalised basis. Further studies are needed on controversial histological types such as clear cell adenocarcinoma, but the final decision must always be given to the correctly informed patient.The authors declare that they do not have any financial or potential conflicts of interest of any kind.


Although palsy of the long thoracic nerve is the classical pathogenesis of winging scapula, it may also be caused by osteochondroma. This rare etiopathology has previously been described in pediatric patients, but it is seldom observed in adults.We describe three cases of static scapular winging with pain on movement.Case 1 is a Caucasian woman aged 35 years with a wing-like prominence of the medial margin of her right scapula due to an osteochondroma originating from the ventral omoplate. Histopathological evaluation after surgical resection confirmed the diagnosis. The postoperative course was unremarkable without signs of recurrence on examination at 2 years.Case 2 is a Caucasian woman aged 39 years with painful scapula alata and neuralgic pain projected along the left ribcage caused by an osteochondroma of the left scapula with contact to the 2nd and 3rd rib. Following surgical resection, the neuropathic pain continued, demanding neurolysis of the 3rd and 4th intercostal nerve after 8 months. The patient was free of symptoms 2 years after neurolysis.Case 3 is a Caucasian woman aged 48 years with scapular winging due to a large exostosis of the left ventral scapular surface with a broad cartilaginous cap and a large pseudobursa. Following exclusion of malignancy by an incisional biopsy, exostosis and pseudobursa were resected. The patient had an unremarkable postoperative course without signs of recurrence 1 year postoperatively.Based on these cases, we developed an algorithm for the diagnostic evaluation and therapeutic management of scapula alata due to osteochondroma.Orthopedic surgeons should be aware of this uncommon condition in the differential diagnosis of winged scapula not only in children, but also in adult patients. Winging of the scapula  is defined as the prominence of the medial  border of the scapula. This entity was first described by Velpeau . SerratuThe winged scapula can be either dynamic or static Figure . In dynaScapular tumors, such as osteochondroma (osteocartilaginous exostosis), have rarely been described as causes of pseudowinging ,4,6-8. OWe report on three cases of static scapular winging caused by osteochondroma in adult patients between 35 and 48 years of age Table . These pA Caucasian woman aged 35 years was referred to our hospital because of a mass at the medial margin of her right scapula. Several months prior to presentation, swelling on her scapula emerged, accompanied by local inflammation. The patient complained about inconstant pain on abduction of her right arm. There was no pain at rest, nor fever or chills. Her past medical and family histories were uneventful except for chronic asthma Table . OrthopeSurgical resection of the exostosis was performed through a medial approach Figure . HistopaA Caucasian woman aged 39 years presented at our department because of neuralgic pain of her left ribcage. The patient has had multiple cartilaginous exostoses and underwent resection at various sites in childhood. She suffered pain for several months, which worsened during the weeks before. Her family history was unremarkable. On clinical examination, her left scapula showed a wing-like prominence of the medial margin but could be moved freely without local tenderness. There was no snapping on any movement. Examination of her left shoulder revealed a sharp pain at maximal abduction, felt along the 3rd left rib. The patient was neurologically intact. A true lateral radiograph of her left scapula revealed a large exophytic mass extruding from the ventral part of the scapula and a deformity of her left 3rd rib. A CT scan confirmed the suspected diagnosis of osteochondroma and showed an accompanying bursa originating from the scapula with contact to the 2nd and 3rd rib Figure .After surgical resection Figure , the hisA Caucasian woman aged 48 years was referred to our clinic because of intermittent pain of her left shoulder for several years. Over the last months prior to admission, she noticed an increasing swelling with accompanying pain on movement of the joint. The patient had unremarkable medical and family histories. At examination, a swelling over her left omoplate was evident with no local tenderness. The left shoulder was higher than the right one and there was no limitation in the range of motion or snapping of the scapula during movement. The neurological examination was without pathological findings. A true lateral X-ray of the left shoulder demonstrated a large exophytic mass on the ventral part of the scapula. A CT scan showed a large exostosis taking its origin from the lateral margin of the scapula Figure . The MRIBecause of suspected malignancy, an incisional biopsy of this soft-tissue tumor was performed initially. Histopathological examination revealed the presence of a pseudobursa and excluded malignancy. The exostosis and the pseudobursa were then removed though a medial approach. The patient had an unremarkable postoperative course. At follow up after 1 year, she was doing well and had no complaints or limitation of movement. No signs of a recurrence were present on radiographs.The differential diagnosis of winged scapula can be complicated. When treating patients with scapular winging, orthopedic surgeons should be aware of the different etiopathologies Figure . In the Although scapula alata usually is indolent, patients with subscapular osteochondroma often complain of pain and grating originating from the scapulothoracic articulation ,4. On orAs anteroposterior radiography may not always delineate the subscapular osteochondroma, a true lateral X-ray or a CT scan is necessary to demonstrate the mass and confirm the diagnosis: the pathognomonic radiographic feature is the cortex of the host bone flaring into the cortex of the exostosis and the cancellous bones blending into each other. Furthermore, a CT is not only helpful for confirming the diagnosis but also for preoperative planning. Moreover, a non- or poorly mineralized mass of a large osteochondroma may indicate the presence of secondary chondrosarcoma in the exostosis . MRI is In cases of painful winged scapula caused by entities other than osteochondroma, the usual treatment options consist of scapulothoracic fusion  or pectoThe prognosis of (solitary) osteochondroma is excellent. Local recurrence after surgery is very rare . HoweverOsteochondroma of the ventral scapula should be excluded not only in children ,6,7, butWritten informed consent was obtained from the patients for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.PO carried out the analysis, review of the literature, and writing of the manuscript. KA participated in research, writing of the manuscript, and review of the literature. EF and DK performed surgery and participated in the review of the manuscript. HM conceived the study, carried out surgery, and participated in research as well as in writing and reviewing the manuscript. All authors read and approved the final manuscript.


Amyloidosis most often manifests as a systemic involvement of multiple tissues and organs, and an amyloidal deposit confined to the stomach is extremely rare. It is sometimes difficult to provide a definitive diagnosis of localized gastric amyloidosis by biopsy specimen and diagnosis of amyloidosis in some cases has been finalized only after surgical resection of the stomach.A 76-year-old Japanese woman with epigastric discomfort underwent an esophagogastroduodenoscopy procedure. The esophagogastroduodenoscopy revealed gastric wall thickening, suggesting scirrhous gastric carcinoma, at the greater curvature from the upper to the lower part of the gastric corpus. A biopsy specimen revealed amyloid deposits in the submucosal layer with no malignant findings. We resected a representative portion of the lesion by endoscopic mucosal resection using the strip biopsy method to obtain sufficient tissue specimens, and then conducted a detailed histological evaluation of the samples. The resected specimens revealed deposition of amyloidal materials in the gastric mucosa and submucosa without any malignant findings. Congo red staining results were positive for amyloidal protein and exhibited green birefringence under polarized light. Congo red staining with prior potassium permanganate incubation confirmed the light chain (AL) amyloid protein type. Based on these results, gastric malignancy, systemic amyloidosis and amyloid deposits induced by inflammatory disease were excluded and this lesion was consequently diagnosed as localized gastric amyloidosis. Our patient was an older woman and there were no findings relative to an increase in gastrointestinal symptoms or anemia, so no further treatment was performed. She continued to be in good condition without any finding of disease progression six years after verification of our diagnosis.We report an unusual case of primary amyloidosis of the stomach resembling scirrhous gastric carcinoma. This case of localized gastric amyloidosis was differentiated from scirrhous gastric cancer after performing endoscopic mucosal resection without an invasive surgical resection, as endoscopic mucosal resection provided sufficient tissue specimens from the lesion to make an accurate histological evaluation. The term amyloidosis refers to a group of disorders characterized by extracellular accumulation of insoluble, fibrillar proteins in various organs and tissues. Amyloidosis most often manifests as systemic involvement of multiple tissues and organs, and an amyloidal deposit confined to the stomach has been extremely rare in previously published reports. It is sometimes difficult to provide a definitive diagnosis of localized gastric amyloidosis by biopsy specimen and diagnosis of amyloidosis in some cases was definitively made only after surgical resection of the stomach.A 76-year-old Japanese woman presented to our facility with epigastric discomfort; she had previously undergone an esophagogastroduodenoscopy (EGD) procedure at another hospital. The EGD had revealed gastric wall thickening, suggesting scirrhous gastric carcinoma, so our patient was referred to our hospital for further examination and treatment.A barium upper gastrointestinal X-ray series indicated rigidity and poor extensibility of the gastric wall from the fornix to the lower gastric body, and irregular, enlarged folds were also noticeable Figure . EGD revWe resected a representative portion of the lesion by endoscopic mucosal resection (EMR) using the strip biopsy method to obtain sufficient tissue specimens, and we then conducted a detailed histological evaluation Figure . There wThe term amyloidosis refers to a group of disorders characterized by extracellular accumulation of insoluble, fibrillar proteins in various organs and tissues . It can Clinical symptoms of localized gastric amyloidosis are often uncharacteristic and varied, including epigastric discomfort, poor appetite, hematemesis, hematochezia and gastric perforation ,3. In adWith regard to diagnosis of scirrhous type gastric cancer, histological confirmation using biopsy specimens is also sometimes difficult; accuracy of pre-operative histological diagnosis of scirrhous type gastric cancer has ranged from 58% to 70% in previously published reports ,14. In tPrimary amyloidosis refers to the disorder in patients with no preceding or co-existing disease except immunocyte dyscrasis in which the extracellular substance is composed of AL protein produced by plasma cells, as typically seen in multiple myeloma. The major forms of systemic amyloidosis also include reactive systemic amyloidosis consisting of a non-immunoglobulin protein secreted by the liver in the setting of chronic inflammatory disorders or cancers . LocalizCurrently, there are no published reports that mention in particular any specific therapy for localized gastric amyloidosis. Some reports have documented that surgical resection with lymph node dissection may be a preferable therapeutic strategy to prevent possible complications such as bleeding and obstruction ,6. In coIn summary, this rare case of localized gastric amyloidosis was differentiated from scirrhous gastric cancer without performing invasive surgical resection as the use of EMR provided sufficient tissue specimens from the lesion to make an accurate diagnosis of localized deposition of AL type amyloid with no malignant findings possible.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-in-Chief of this journal.The authors declare that they have no competing interests.TK was the main contributor to the preparation of the rough draft. HS critically revised the manuscript. HT and RK was involved in histopathological analysis. SY, SN, TF, HK and IO helped to draft the manuscript. All authors read and approved the final manuscript.


Cholesteatoma is a relatively common disease within the middle ear cavity, but rarely it manifests in the paranasal sinuses. There is, to the best of our knowledge, only one other published case of cholesteatoma inside the concha bullosa in the English language literature.An 81-year-old Caucasian woman was admitted to our hospital complaining of nasal obstruction, headache and diplopia. After endoscopic and radiological evaluation a transnasal endoscopic approach was chosen. The diagnosis of cholesteatoma was established by histopathological evaluation of the mass inside the concha bullosa.Although it is rarely seen, cholesteatoma should be considered in the differential diagnosis of slow-growing and destructive paranasal masses. Concha bullosa (CB) is the pneumatization of the middle turbinate and is one of the most common variations of the sinonasal anatomy. A 14% to 53.6% frequency of CB has been reported by various studies -4. CholeAn 81-year-old Caucasian woman presented to the outpatient clinic of our hospital with nasal obstruction, headache and diplopia. She had experienced nasal obstruction for over ten years and her headache had worsened for three months. She did not have epistaxis or epiphora. She had been treated medically with antibiotics, nasal decongestants, and nasal steroids. However, her complaints were not alleviated. Her otorhinolaryngological history was unremarkable.An endoscopic examination revealed a massively large middle turbinate on the left side. The mucosa was normal and no infection signs were detected in the nasal passage. The enlarged middle turbinate contacting the lateral nasal wall and the septum was the only endoscopic finding. Our patient has proptosis, but her ophthalmic examination revealed 20/20 visual acuity in both eyes and normal intraocular pressures.Computed tomography (CT) scans showed a homogenous fluid or soft tissue density lesion surrounded by a bony shell in the left nasal cavity. Erosion of lamina papyracea and ethmoid roof was determined from the CT scans Figure . NeoplasIntraoperatively, when we removed the lateral part of the CB for biopsy, a yellow-white colored mass was found inside the CB cavity Figure . The masFour theories on the pathogenesis of paranasal sinus cholesteatomas have been put forward . The mosMost of the paranasal cholesteatoma cases were adults, as in our patient . HoweverDue to the rarity of this lesion, cholesteatoma in the paranasal sinuses is seldom pre-operatively diagnosed. Cholesteatoma has a slow-growing pattern and it has an erosive effect on bone. In our patient, we initially considered the possibility of a neoplastic lesion or pyocele during the differential diagnosis. However, as shown in this case, cholesteatoma can cause similar findings.Although it is rarely seen, cholesteatoma should be considered in the differential diagnosis of slow-growing and destructive paranasal masses. Total excision is the treatment of choice for paranasal cholesteatomas. Our patient was treated successfully with a transnasal endoscopic approach.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-in-Chief of this journal.The authors declare that they have no competing interests.IC performed the surgery and supervised the writing of the manuscript. ED was the major contributor to the writing of the manuscript. IBA had a role in writing a final revision of the manuscript. SC performed the histopathological analysis and final revision of the manuscript. All authors read and approved the final manuscript.


We present a case of a 44-year-old female who was evaluated for left recurrent flank and abdominal pain. Abdominal ultrasonography demonstrated a large cystic mass on the upper pole of the left kidney. Magnetic resonance imaging showed a large, homogenous cystic mass measuring 8.5 × 9.5 cm with well-defined contours, being hypointense on T1-weighted images and hyperintense on T2-weighted images on the upper pole of the left kidney without a distinct plan between adrenal gland. According to clinical and radiological findings, surgical excision was carried out with a subcostal flank incision. Histologic and immunohistochemical examination demonstrates that the definite diagnosis is cystic lymphangioma of the left adrenal gland. Adrenal lymphangioma is a very rare lesion. This is the unique case of an adrenal lymphangioma considered as a renal cyst because of its radiological appearance. Adrenal cystic lesions are rare and mostly identified incidentally during radiological investigations or at surgery for unrelated reasons. Its incidence in autopsy series varies between 0,064 and 0,18%. Adrenal cysts are histologically classified into four main groups: endothelial cysts (45%), pseudocysts (39%), epithelial cysts (9%), and parasitic cysts (%7). Endothelial cysts are divided into two subgroups: lymphangiomatous and angiomatous cysts. Lymphangiomatous adrenal cysts are also known as adrenal lymphangioma (AL) .We report a case of an AL, which is mimicking a renal cyst, with an emphasis on the symptoms, differential diagnosis, and treatment.A 44-year-old female presented to the outpatient clinic with left recurrent flank and abdominal pain during the last 5 years. On physical examination, she was relaxed with palpation of the abdomen and was known to have a stabile hypertension and panic attack diseases. On laboratory evaluation, a normal urine analysis and normal hemoglobin, urea, and creatine levels were identified. Abdominal ultrasonography (US) demonstrated a large cystic mass on the upper pole of the left kidney. Magnetic resonance imaging (MRI) showed a large homogenous cystic mass measuring 8.5 × 9.5 cm with well-defined contours, being hypointense on T1-weighted images and hyperintense on T2-weighted images on the upper pole of the left kidney without a distinct plan between adrenal gland .According to clinical and radiological findings, surgical excision was carried out with a subcostal flank incision. A cystic mass, approximately 10 cm in diameter, was identified on the suprarenal area without a distinct plan between left kidney and adrenal gland; therefore, the cyst was excised with a small part of the left adrenal gland without any intraoperative complications. Macroscopically there were osseous and cartilage-like tissues partly on the surface of the cyst wall. The cyst was filled with clear, nonviscous, brown colored fluid. After a 3-day hospital stay, the patient was discharged and the postoperative course was uneventful.Histologically, the cyst showed a typical multicystic architecture with dilated spaces lined by flattened, bland, simple lining. The cystic spaces occasionally contained proteinaceous material, lacking red blood cell content, and the cyst wall contained adrenal cortex . On immuAL is a benign vascular lesion and a subtype of endothelial adrenal cysts. The first case of AL had been reported in 1965 and recently less than 50 immunohistochemically proven cases have been reported in the literature . The larThe etiology and pathogenesis of AL are still unknown and matters of debate. The most favored theories are: malformation of lymphatic channels, ectasia of lymphatic channels, obstruction of proximal lymphatic channels, and a cystic degeneration in a hamartoma , 5.ALs are macroscopically multilocular, thin-walled cystic lesions and are filled with nonviscous, clear, yellow-brown-colored fluid. Histologically, they are multicystic lesions formed by irregular dilated spaces lined with flattened, simple endothelial cells. Endothelial atypia has never seen in any of the lesions in the literature , 3. HistALs are usually asymptomatic. If they are symptomatic, symptoms are usually related to the mass effect and position of the cyst. The most common symptoms are abdominal or flank pain, gastrointestinal disturbance, palpable mass, headache, and arterial hypertension and palpitation especially in functional cysts –6.The majority of the adrenal cysts are benign and non-functional. Neri and Nance reported 6 adrenal cyst cases of their own and reviewed over 600 cases, in which they detected that the malignancy incidence was 7% [On US, AL is a well-marginated, anechoic, cystic lesion typically located on the suprarenal area. There can be acoustic shadows and internal echoes, if calcifications or internal debris are present , 3, 8. AIn our case, MRI confirmed that the cystic mass were appeared that a part of the left kidney without a distinct plane between the kidney and the adrenal gland, so it was concluded that the the cyst originated from kidney rather than adrenal gland. We decided to resect the cyst surgically because of its large size and bothersome pain to the patient. To our knowledge, this is the unique case of an AL considered as a renal cyst because of its radiological appearance. But there are examples of misdiagnosis of other type of adrenal cysts in the literature , 5.The subject of debate is which cysts need surgical intervention and which cysts need to be treated conservatively. The management of adrenal cysts and indications for surgery have to be based on oncological, endocrinological and clinical findings: (1) oncological indications: large cysts (5 cm or more in diameter), high density on unenhanced CT >10 HU), delayed wash-out of the contrast medium, significant growth of the cyst on serial imaging, (2) Functional cysts, (3) Symptomatic cysts and cysts which can cause complications such as hemorrhage, rupture, or infection [ HU, dela


Primary small cell carcinoma of the pancreas (SCCP) is a rare malignancy with an extremely poor prognosis which accounts for 1 to 1.4 percent of all pancreatic malignancies.We present the case of a 62-year-old man with a half-month history of upper abdominal discomfort who was diagnosed with SCC of the pancreatic tail. A Chest X-ray showed no evidence of primary lung tumor. The diagnosis of a SCCP was confirmed by post-surgery pathology and immunohistology. In our review of the published reports of SCCP, we only found a few cases reported in the literatures. The diagnosis of SCCP needs the post-surgery pathology and immunohistology and the prognosis of SCCP is extremely poor. There was a significant increase in median survival, from 1 to 6 months, in treated patients compared to patients treated only by symptomatic management. Chemotherapy was the most common treatment and the combination of cisplatin/etoposide was most frequently prescribed.The accurate diagnosis of (SCCP) is necessary for determining prognosis and deciding appropriate therapy. Small cell carcinomas (SCCs) is an aggressive tumor, which account for 18-20% of all primary lung cancers, but they are also described in the urinary bladder, prostate, salivary glands, pharynx, larynx, esophagus, stomach, pancreas, colon, rectum, skin, and cervix . Similar9/L; platelets: 78 × 109/L; AST: 21 U/L; ALT: 14 U/L; total bilirubin: 14.2 μmol/L; direct bilirubin: 6.6 μmol/L; serum creatinine: 75 μmol/L;AFP:2.2 ng/mL ; CA19-9:122.7 U/mL ; CEA: 2.82 ng/ml . Chest X-ray had no sign of primary lung cancer and metastasizes. In addition, there was no evidence of ascites, and liver metastasizes; therefore exploratory laparotomy was performed routinely.The patient was a 62-year-old man. Except hypertension, he had no significant past medical history. He went to our hospital on July 21, 2011, with the symptom of upper abdominal discomfort. The local hospital CT scan revealed a low-density mass at the pancreatic tail , the liver (38%), the lungs (14%), the bone marrow (14%), the bone (10%), the colon (10%), and the adrenal gland (10%); rarer sites included the spleen, gallbladder, kidney, skin and brain . In our 2%, the lAs for the pulmonary SCC, the encouraging long remission rates could be achieved by chemotherapy and/or radiation. Surgery alone is generally unsuccessful in managing either pulmonary or extrapulmonary SCC . BecauseThe SCCP is a rare type pancreatic cancer and has poor prognosis; therefore the accurate diagnosis of SCCP is necessary. Whether the patients had their tumors resected, the adjuvant chemotherapy should be performed, and cisplatin/etoposide appears to be the preferred chemotherapy regimen at present.Written informed consent was obtained from the patients for publication of this report. A copy of the written consent is available for review with the Editor-in-Chief of this journal.The authors declare that they have no competing interests.DSW, YFR and DYJ: Operating team and drafted the manuscript; WCW: operating team, post-surgery care. All authors read and approve the final manuscript.


Pilonidal sinus is a long-standing chronic inflammatory condition consisting of a sinus tract from the skin-lined orifice extending into subcutaneous tissue, with hairs attached to the wall of the tract and projecting outside of the opening. Penile location is rare, and differential diagnosis with severe balanoposthitis, epidermal cysts, and neoplasms can be difficult. We report a rare case of pilonidal cyst located between coronal sulcus and prepuce which, due to its ulcerated aspect and absence of a tract with projecting hairs, simulated a penile carcinoma. Pilonidal sinus is a long-standing chronic inflammatory condition consisting of a sinus tract from the skin-lined orifice extending into subcutaneous tissue, with hairs attached to the wall of the tract and projecting outside of the opening. It occurs most commonly in the sacrococcygeal area but can be seen also in scalp, ear, brow, cervical subcutaneous region, axilla, interdigital clefts, anterior chest wall, nipple, umbilicus, suprapubic region, perineum, clitoris, anal canal, sole of foot, and amputation stumps . Penile We report a rare case of pilonidal cyst located between coronal sulcus and prepuce which, due to its ulcerated aspect and absence of a tract with projecting hairs, simulated a penile carcinoma. A 26-year-old Caucasian uncircumcised man presented with a 3-month history of an ulcerated lesion located between the coronal sulcus and the prepuce of the dorsum of his penis, not associated with pain or discharge. Past medical history disclosed that two years ago he had noticed a tiny cyst in his dorsal foreskin. On physical examination, the foreskin was swollen and a bit difficult to retract. Between the coronal sulcus and the prepuce of the dorsal aspect of the penis there was a 1.5 cm whitish ulcer overlying a hard nodule apparently fixed to the corpora cavernosa mimicking a penile carcinoma . InguinaThe patient was admitted for surgical excision. The ulcerated lesion with the underlying nodule, hardly attached but not infiltrating Buck's fascia, was widely excised. Frozen sections of the resected specimen ruled out malignancy. Formal circumcision was performed. Histology showed a thick-wall cyst lined by a stratified squamous epithelium. Few hair fragments were recognizable in the lumen and in the wall itself, where they elicited a foreign body granulomatous reaction.Recovery was uneventful, and he remains asymptomatic 10 months after surgery.Pilonidal sinus of the penis is a rare condition with 15 cases (including the present one) having been reported in literature. Analysis of reported cases shows that it affects uncircumcised males between 21 and 59 years of age and is usually located between coronal sulcus and prepuce, mostly dorsally (60%) or ventrally (33%). It is believed that the coronal sulcus acts as a cleft where hairs may collect and are forced to penetrate into penile shaft and foreskin by the natural movement that occurs between these two surfaces [Our patient did not present with a sinus nor with a cyst, but with an ulcerated hard lesion without discharge or evidence of hairs projecting from it, thus mimicking penile carcinoma.Unusual lesions of the penis should be biopsied for both bacteriological and histological assessment. Surgeons should be aware that classic penile pilonidal sinus, with hairs projecting from it, can sometimes be associated with actinomycosis needing surgical excision and long-term penicillin treatment to avoid systemic complications of this disease . It is a


Lobular capillary hemangiomas (LCH) have been cited in the literature as a rare potential cause for recurrent hemoptysis. They are mostly associated with cutaneous and mucosal surfaces. Rarely do they affect the trachea and associated airways in adults. Lobular capillary hemangiomas can be associated with previous trauma to the airway; however, drugs, hormonal shifts, viral oncogenes, production of angiogenic factors, and cytogenetic clonal deletion abnormalities can also influence these lesions. We document a case of a posterior wall tracheal hemangioma with associated recurrent hemoptysis in a 22-year-old male on testosterone therapy. An LCH attached to the posterior tracheal wall can be technically difficult to remove due to risk of perforation and bleeding. There have been no cases documented of posterior wall LCH. Lobular capillary hemangiomas (LCH), formally known as pyogenic granuloma, usually present as a painless, bleeding mass adherent to the mucosal or cutaneous surfaces of the upper airways. Most commonly they are present on the lip, nose, oral cavity, and tongue. According to a pathologic review of 639 vascular lesions of the upper airway , they ar A 22-year-old male presented to our clinic with recurrent episodes of hemoptysis over a 4-week period with 3 distinct events. He stated that this started with an incidence of heavy coughing. He then would cough up to 2–4 tablespoons of blood. After presentation to the emergency room, it was initially thought this was related to epistaxis, and he was sent home with treatment for allergic rhinitis. He had no other symptoms, denying fever, weight loss or gain, chest pain, easy bruising, dyspnea, or rash. He did complain of chronic postnasal drip, nasal congestion, and occasional pruritis, which he attributed to allergies. He had a past medical history of eosinophilic colitis, obsessive-compulsive disorder, history of an aneurismal bone cyst status post-removal, and allergic rhinitis. He had also been recently diagnosed with hypogonadism over the last 5 months, and was on injectable testosterone supplementation. He denied smoking, alcohol, and illicit drug use and had no known inhalant exposures and trauma to the airway that he could recall. His family history included a brother with eosinophilic colitis and his mother had vascular ectasias of her spinal cord. He did note that these episodes seemed to occur 5 days after his dose of intramuscular testosterone. Physical exam was otherwise unremarkable and laboratory values were unrevealing. Computed tomography did not reveal any notable lesion of the upper airway or lung parenchyma.  Upon flexible bronchoscopic evaluation, the following lesion was seen in the distal trachea . The pur Upon followup, the patient had no hemoptysis episodes and has subsequently stopped his testosterone therapy. Lobular capillary hemangioma is a well-known entity that commonly involves skin and oral/nasal mucosa, developing rapidly over a number of days to weeks. Its cause is not well defined but certain postulates correlate to previous trauma, hormonal shifts, viral oncogenes, Bartonella infection, production of angiogenic factors, and cytogenetic clonal deletion abnormalities, although certain studies have disproven some of the above , 8. Othe Regardless of the causal relationships, the position of the lesion became technically difficult due to the posterior wall location and the vascular structure of the tumor itself. The treatment in general for these lesions depends on the extent or size of the lesion, age, comorbidities, and other factors contributing to the overall clinical scenario. Because of inherent thinness of the posterior tracheal wall, there is a risk of iatrogenic tears and perforation. These defects are treated with various approaches; surgical intervention is sometimes required . Lobular Lobular capillary hemangioma found in the trachea continues to be a rare entity and has yet to be described abutting the posterior wall. Posterior laryngeal wall lesions can be challenging due to risk of laceration and perforation and the appropriate equipment needs to be readily available to avoid complications. Though no direct causal link could be found in this patient, we believe that hormonal supplementation may have played a role in its development.


Gastrointestinal stromal tumors are rare tumors. They commonly metastasize within the abdominal cavity, particularly to the liver. Less commonly, metastases can be found in the bone.We here present a case of metastasis to the scapula in a 54-year-old Caucasian male patient with an advanced gastrointestinal stromal tumor, which was subsequently successfully treated with resection and sunitinib.The present study is, to the best of our knowledge, the second to describe scapular metastasis of a gastrointestinal stromal tumor. Our patient was treated by scapulectomy. The overwhelming majority of scapular tumors are metastases that arise from soft tissue, hepatocellular and thyroid tumors. Gastrointestinal stromal tumor metastasis occurs rarely. Scapular surgery can successfully provide local control of the disease. After the surgery, patients should continue with medical treatment. Gastrointestinal stromal tumors (GISTs) are the most common connective tissue tumors of the gastrointestinal (GI) tract . GISTs oSurgery is the standard treatment for primary resectable GISTs; however, surgical resection is seldom curative and by five years after complete removal, 40% of patients have relapsed . Imatinien bloc resection technique. A computed tomography (CT) scan of his thorax demonstrated no pathological finding. Multiple and non-resectable bilobar liver metastases were found on abdominal magnetic resonance imaging (MRI). Our patient was started on imatinib 400 mg daily in 2001. After an event-free follow-up period, our patient was readmitted in 2010 with complaints of right shoulder pain and limitation of shoulder movement. His liver metastases were seen to be stable on the abdominal and pelvic MRI. No local recurrence was found during a colonoscopic examination.A 54-year-old Caucasian male patient with no previous history of any disease was referred to our clinic on January 2001 with rectal bleeding. A colonoscopy showed an ulcerated polypoid mass in his rectum that was 12 cm distant from the anal verge. A pathological examination of the biopsied specimens revealed a GIST. Surgery was performed and the mass was excised by MRI showed a 13 cm × 10 cm × 7 cm mass with partial contrast-enhancement that was T1- and T2-hyperintense and heterogeneous. The mass was destroying the body of his right scapula and had widespread involvement in the infraspinatus muscle. A fluorodeoxyglucose positron emission tomography (PET)-CT showed a hypermetabolic mass in his posterior right shoulder , whose ligand is a stem cell factor. The PDGFRα gene also encodes an oncogenic transmembrane receptor tyrosine kinase (PDGFRα).GISTs are mesenchymal tumors with generic histological features that arise from the gastrointestinal or abdominal tract. GISTs originate from interstitial cells of Cajal - intestinal pacemaker cells that arise from the muscularis propria of the gastrointestinal tract wall . The c-kc-kit and PDGFRα genes are associated with the tumorigenesis of GISTs. The most frequent mutations are observed in the juxtamembrane domain of KIT, which is coded by exon 11. Mutations in the extracellular domain of KIT exon 9 are seen less often. Although most GISTs express KIT (95%), a minority will be negative for KIT or express wild-type KIT [Gain of function mutations of the type KIT .GISTs commonly occur in the stomach (50% to 60%) and small intestine (25%). They can also occur in the colorectum (10%), esophagus (5%), anywhere along the GI tract and, rarely, in extraintestinal sites, including the mesentery, omentum, peritoneum, gallbladder and liver (3%) . The clic-kit-positive, several immunohistochemical markers in the diagnosis of GISTs, the differential diagnosis and prognosis assessment is currently under investigation [Biopsy material is essential for the definitive diagnosis of GISTs, as radiological and endoscopic examinations are insufficient as diagnostic tools. A percutaneous biopsy should not be performed in patients with potentially resectable tumors, due to the low diagnostic yield and the risk of seeding malignant cells. GISTs present in a wide range of size (from a few millimeters to >30 cm). They commonly arise from the wall of the GI tract. GISTs are usually well-demarcated encapsulated firm nodules with a tan-gray appearance on the cut surface. Large tumors may show cystic degeneration, central necrosis and hemorrhage. GISTs can be composed of spindle cells, epithelioid cells, or a mixture of both spindle and epithelioid cells (pleomorphic). The best immunostaining method for identifying GISTs is to test for expression of KIT, also known as CD117. About 5% of GISTs do not stain positive for KIT. Other immunohistochemical markers often observed to be positive in GISTs are CD34 (60% to 70%) and smooth muscle actin (30% to 40%). A small percentage of GISTs may show positivity for desmin, or S-100. This is not important if the GIST is tigation ,16.The liver is the most common site of GIST metastasis. Distant metastasis to other sites, especially bone, the lungs and lymph nodes, is relatively rare. The mandible, spine, femur and humerus have been reported as localizations of bone metastasis. Only one case of scapular metastasis has been reported . The preAccording to a retrospective analysis, 17 of 309 GIST patients (5.5%) had bone metastasis; scapular metastasis was not observed and bone metastasis was not a primary clinical feature at their initial presentation. The patients with bone metastasis had a median survival of 325 weeks from initial diagnosis, 252 weeks from distant recurrence-free status, and 135 weeks after the diagnosis of bone metastasis. CT, MRI and PET-CT were used to identify metastases; however, PET-CT was more effective for diagnosis, staging, restaging and measurement of therapeutic response of these neoplasms. Bone scanning was not routinely performed .Despite advances in drug therapy, surgical resection remains the primary treatment of GISTs and total resection remains the most effective. However, drug treatment should be used instead of surgery in some patients, such as those with widespread metastasis, local progression or a general medical condition that contraindicates surgery, and when the surgical intervention is associated with a high rate of morbidity and/or mortality. If technically feasible, resection can be performed in patients with liver metastasis; however, surgery is often not applicable due to multiple liver metastases, very large metastases or peritoneal involvement . HepaticImatinib mesilate inhibits KIT tyrosine kinase activity. Imatinib is the first molecular targeted therapy. The current recommendation is to initiate imatinib treatment at 400 mg daily for at least three months and then commence follow-up. Initiation at 800 mg daily often causes side effects, which eventually lead to dose reduction. Thus, an initial dose of 400 mg daily can be increased according to follow-up results ,21. In pThe present study is the second to describe scapular metastasis of a GIST. Our patient was treated by scapulectomy. The overwhelming majority of scapular tumors are metastases that arise from soft tissue, hepatocellular and thyroid tumors. GIST metastasis occurs rarely. Scapular surgery can successfully provide local control of the disease. Following the surgery, patients should continue with medical treatment.Written informed consent was obtained from the patient for publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.All the authors have contributed to the submitted case report. The design of the case report was done by all authors. Imaging and pathology studies were realized by SD, MH and SD. Analysis and interpretation of the data were realized by DT, FS, MAÖ and NMM. The results were approved by all authors. All authors read and approved the final manuscript.


Lobular capillary hemangioma (LCH) is a benign lesion of vascular origin. It rarely involves nasal cavity which most commonly manifests as progressive nasal obstruction and epistaxis. In this report we present a case of LCH of the nasal cavity which occurred approximately one month after delivery. There was no recurrence after complete endoscopic resection during one year follow up.  Lobular capillary hemangioma (LCH) is a benign lesion of vascular origin. The most common sites of lesion are the skin and oral mucosa. LCH rarely involves nasal cavity which most commonly manifests as progressive nasal obstruction and epistaxis. Trauma and hormonal changes are presumed as two major predisposing factors -4. In thA 32-year-old woman came to hospital clinic with complaint of progressive right-sided nasal obstruction associated with intermittent mild epistaxis, muco-purulant rhinorrhea and post nasal drip since 10 months ago began one month after her uncomplicated delivery of a normal baby. She was completely asymptomatic during her pregnancy.. She didn’t mention any history of nasal trauma or nasal intubation but she has had history of multiple nasal packing during recent months. Endoscopic examination of nasal cavity revealed a red large polypoid mass which bled easily and originated from posterior part of middle turbinate.A CT scan of the paranasal sinuses also revealed a right nasal cavity soft tissue mass without any evidence of bony destruction or extension of the mass into the adjacent paranasal sinuses .The patient was taken to the operating room where the soft tissue mass was completely excised using endoscopic techniques. The surgery involved partial resection of the middle turbinate .Histologic finding showed proliferation of small capillaries lined by plump endothelial cells in a loboular pattern .There was no recurrence during the one-year follow-up period.Hemangiomas are vascular neoplasms that morphologically classified in to capillary, cavernous, arteriovenouse and epitheliod type . LCH, whThe etiology of LCH remains unknown but trauma and hormonal changes, such as those that occur in pregnancy, are thought to be major etiologic factors. Viral oncogenes, microscopic arteriovenous malformations, cytogenetic abnormalities and production of angiogenic factors are also associated with LCH . In fourA giant pyogenic granuloma of the posterior part of the nasal septum that occurred after prolonged use (30 days) of a nasogastric feeding tube has also been recorded . In a st Clinical symptoms include nasal obstruction, Epistaxis, Epiphora, purulant or mucous rhinorrhea and nasal deformity . In a stThe treatment of choice for these lesions is complete excision using endoscopic approach even for large lesions. In our case, there was no recurrence after complete endoscopic resection during one year follow up.


A 56-year-old female teacher presented to Shariati Hospital, Tehran, Iran, with history of progressive headache, difficulty to read and write (dyslexia and dysgraphia), and seizure since 3 months ago. Neurological examination demonstrated mild bilateral papilledema and left side Babinski sign. The exam was otherwise normal.What are the imaging findings?What are the probable differential diagnoses?What is the most probable diagnosis?Does the lesion explain dysgraphia and dyslexia?Brain magnetic resonance imaging (MRI) was performed .What areThe brain MRI showed an ill-defined fluid attenuated inversion recovery (FLAIR) hyperintense/T1 hypointense lesion in right deep parietotemporal lobes extending to contralateral occipital lobe via splenium of corpus callosum causing mass effect and subfalcine herniation. Some small FLAIR hyperintense lesions were also seen in left frontoparietal subcortical area. After contrast administration, non-homogenous enhancement was seen in some parts of the lesion.Mass lesions involving splenium of corpus callosum include glioblastoma multiforme (GBM), anaplastic astrocytoma and primary central nervous system (CNS) lymphoma (PCNSL).1Being hyperintense in FLAIR, and non-homogenous faint gad-enhancement (Gadolinium) were in favor of GBM. Anaplastic astrocytoma scan was similar to GBM, but usually did not enhance. PCNSL was usually hypointense in T2 and showed avid enhancement, except in immunosuppressed (e.g. AIDS) patients.2 Surprisingly, the brain MRI of our patient did not show the left inferior parietal (angular gyrus) involvement. However, GBM was an infiltrative neoplasm, with histologic involvement far beyond visible radiologic tumor. Therefore, it can be concluded that though it was not seen on MRI, inferior parietal lobe was infiltrated by tumor.Alexia with agraphia is usually seen in left angular gyrus lesions.3 Presenting symptoms are diverse and related to the site of tumor. Diagnosis was proposed by imaging and established by histopathological study. MRI is used as a standard imaging modality in many centers. However, newer sequences  and modalities such as positron emission tomography (PET) are emerging which can help in determining the diagnosis, prognosis, and treatment plan.4 GBM lesions are generally heterogeneously hyperintense in T2/FLAIR and hypo/isointense in T1 sequence. Hemorrhage, necrosis, cyst, fluid/debris level and flow voids (sign of neovascularity) were common findings. Different patterns of enhancement  may be seen.1 Surgery has been the mainstay of treatment, but radiotherapy, radiosurgery and new chemotherapeutic agents have been introduced as adjuvant or alternative treatment options.5Glioblastoma multiform is the most common primary malignant brain tumor.


Carney complex is a very rare multiple neoplasia syndrome with cardiac, cutaneous, and neural tumours with a variety of pigmented lesion of skin. We are reporting a rare case of carney complex in which left atrial myxoma with superficial angiomyxoma, giant cell tumour of bone and lentigines showed a unique association. This patient underwent successful surgical excision of left atrial myxoma under cardiopulmonary bypass. Carney complex is a rare syndrome characterized by neoplasia involving heart, central nervous system and endocrine organs. Presence of pigmented skin and mucosal lesions are an important hallmark of this syndrome. We report a case of left atrial myxoma with giant cell tumor, superficial angiomyxoma and skin pigmentation.A 47 year old male with one year history of exertional dyspnea & palpitation was admitted with complaints of syncopal attacks of sudden onset. He denied any head ache, chest pain, palpitation, edema legs, persistent fever, weight loss or loss of appetite.He had been seen at the trauma center 4 years ago following a bike accident. X-Ray at that time showed a lytic lesion in the distal end of the right femur with destruction of the lateral cortex and break in the anterior and posterior cortex of the lateral condyle with extension of the lesion to the distal femoral articular surface and a pathological fracture and periosteal reaction along the lateral aspect of distal femur Figure . CT scanA biopsy was sent from lytic lesion of fractured lower end of femur. A curettage followed by cement application with cancellous leg screw was done. After 15 days bone cement removal, bone grafting and internal fixation with condylar blade plate was performed. Post operatively patient had minimal restriction of right knee flexion. Clinical and radiological follow up showed healing of the lesion. Cut section of the curettage material consisted of multiple hemorrhagic and tiny grey white focal areas. Microscopy showed bony trabeculae and a tumour composed of sheets of mononuclear stromal cells and many scattered multi nucleated osteoclast like giant cells Figure . HistoloHe had multiple spotty pigmentations (lentigines) on his trunk for several years Figure . He alsoHis cardiac exam showed normal sinus rhythm at 84 beats per minutes and blood pressure of 130/80 mmHg. Mitral first heart sound was slightly accentuated, but the pulmonic sound was normal. Grade-I diastolic murmur was heard over the mitral area. Opening snap was absent. Lungs were clear and chest radiograph showed slight cardiomegaly. Trans-thoracic Echo cardiography revealed an intra-cardiac tumour attached to inter atrial septum, which was almost filling the left atrium & obstructing the mitral inflow. Moderate amount of mitral regurgitation was present Figure . CoronarPatient underwent surgical excision of left atrial myxoma under cardiopulmonary bypass through right atrial approach. When a finger was introduced through the right atrial appendage a firm, smooth, egg sized tumour was encountered. The mitral valve was normal in structure and function. It was excised under vision. The patient had an uneventful recovery without neurologic or renal damage with significant relief of clinical symptoms. Follow up Echocardiography after 6 months showed no evidence of any intra cardiac recurrence.Pathological examination of the tumour revealed a solitary mass weighing 50 gms & measuring 6.5 × 4.5 × 2.5 cms. Externally the tumour appeared congested, shining with myxoid areas Figure .Microscopically it was a hypocellular myxoid tumour with small polygonal, spindle & ovoid tumour cells (Myxoid cells) with round to oval nucleus, scanty eosinophilic cytoplasm, arranged in strands, along with large " Lipidic" cells having abundant vacuolated, clear cytoplasm arranged around thin walled blood vessels in perivascular pattern. Focal nesting of tumour cells was seen. Stroma showed extensive myxoid change. Hemosiderin laden macrophage, focal dense lymphocytic infiltrate, plasma cells, mast cells were also seen Figure .Carney complex as described by J. Aiden Carney in 1985 is an autosomal dominant disorder characterized by neoplasia involving heart, central nervous system and endocrine organs. Presence of pigmented skin and mucosal lesions along with these tumors is an important hallmark of this syndrome . Most caThe most recent diagnostic criteria for Carney Complex includes clinical findings such as spotty skin pigmentation, cutaneous and cardiac myxomas, breast myxomatosis, paradoxical positive response of urinary gluco- corticosteroids to dexamethasone administration during liddle's test, acromegaly, Blue nevus, epithelioid blue nevus, osteochondromyxoma, thyroid carcinoma and mutation of the PRKARIA gene etc. .Lentigines & blue nevi followed by cardiac myxomas are the most common clinical manifestations of the complex. Cardiac myxomas show no age, sex or location preponderance and they are notorious for frequent recurrences . ApproxiEcho Cardiography can accurately determine the location, size, shape, attachment and mobility of the tumour. Coronary angiography is only advised if coronary artery disease is suspected or if the patient is above 40 years of age .Histologically, superficial angiomyxoma is characterized by a myxoid lesion with prominent thin walled blood vessels. The usual site for angiomyxoma is dermis and cutis especially in head and neck region . IncomplAmong bone tumours, osteochondromyxoma has been described in literature which affects about 1% patients of Carney complex. These tumors present as painless mass in diaphyses of distal long bones as well as small flat bones. In this case, we report the presence of giant cell tumour in association with Carney complex. This is the first case report of such an association. Complete tumor resection is curative whereas incomplete resection is associated with high rates of recurrence .At molecular level mutations in chromosome 2 in band p16 and chromosome 17 in bands q22-24 are associated with Carney complex. PRKAR1alpha is a tumor suppressor gene which is found to be mutated in almost 50% of carney complex cases .This case is the first reported case of Left atrial myxoma, lentigines, superficial angiomyxoma and giant cell tumour of bone in a middle aged patient.Written and informed consent obtained for publication of this case report and accompanying images. A copy of the written consent is available for review by editor- in- chief of this journalThe authors declare that they have no competing interests.SB and SG were involved in the surgery and Patient care. NK and AC wrote the manuscript. RW and SS supervised the manuscript. LR provided pathological opinion. All authors read and approved the final manuscript.