We report a case of synchronous double primary tumor of gallbladder and liver. A 63-year-old male was admitted to the hospital complaining of abdominal discomfort. Enhanced computed tomography of the abdomen showed acute cholecystitis with tiny gallbladder stones and a 2.2 cm size enhanced nodule in the left lobe of the liver. Under the impression of acute cholecystitis with gall bladder stones and hepatocellular carcinoma of the left Liver, the patient underwent a laparotomy. At laparotomy, a mass was palpated on the surface of the neck portion of the gall bladder. Intraoperative frozen diagnosis revealed adenocarcinoma of the gall bladder. The patient was diagnosed as having gall bladder cancer and hepatocellular carcinoma, so extended cholecystectomy with dissection of regional lymph nodes and left hemihepatectomy were performed. Histological examination revealed moderated differentiated adenocarcinoma of gallbladder and hepatocellular carcinoma of liver. To our knowledge, the simultaneous occurrence of primary malignant tumor of the gallbladder and liver has never been published before. The patient is doing well with no evidence of recurrence 17 months after surgery. Synchronous double primary malignant neoplasms are a secondary malignancy occurring at the same time or within 6 months after the first malignancy. Improvement of survival rates for patients with malignancy due to early diagnosis and new treatments has enabled more patients to survive long enough to develop the subsequent primary malignancy, and development of more sophisticated diagnostic tools has made possible the detection of synchronous occult malignancies. Synchronous double primary malignant neoplasms in a single patient have been well-documented in the literature. But, synchronous double primary malignant tumor of gallbladder and liver has never been reported. Herein, the authors report a case of a 63-year-old male patient with double primary cancer of gallbladder and liver.3; blood glucose, 209 mg/dl; total bilirubin, 0.5 mg/dl; alkaline phosphatase (ALP), 41 IU/l; aspartate aminotransferase (AST), 193 IU/l; alanine aminotransferase (ALT), 146 IU/l, and amylase 212 IU/l; C-reactive protein 76.7 mg/l. Viral markers were hepatitis B surface antigen [HBsAg(+)], anti-HBs(-) and anti-hepatitis C virus(-). Tumor marker assays showed alpha-fetoprotein was 17.9 n/ml , carcinoembryonic antigen (CEA) was 4.2 ng/ml , carbohydrate antigen 19-9 (CA 19-9) was 112.5 U/ml . A computed tomography (CT) scan of the abdomen showed distension of the gallbladder with gallbladder stones and gallbladder wall thickening, suggesting acute cholecystitis  were within normal limits. The patient was in good general health and had no significant weight loss. On physical examination, the conjunctiva was anemic. The abdomen was soft but tender in the right upper quadrant. Slight resistance, but no rigidity, was recognized in the tender area. Complete blood count and serum biochemistry data on admission showed the following: white blood cell, 16,120/uL; hemoglobin, 8.5 g/dl; hematocrit, 25.3%; platelet, 178000/mms Figure , cirrhoss Figure . Thus, ts Figure . The mass Figure . The tums Figure . Microscs Figure . There wMultiple primary malignant tumors in a single patient are relatively rare. In reviews of the literature regarding multiple primary malignant tumors, the overall occurrence rate of multiple primary malignancies is between 0.73% and 11.7% . MultiplWe report the first case of synchronous double primary malignancies of gallbladder and liver. The possibility of synchronous multiple primary malignancies should be noted in the treatment of elderly patients with malignant tumor. Multiplicity of primary malignancies itself does not necessarily indicate a poor prognosis as long as adequate diagnosis and treatment are performed.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.Ji Won Kim, Jae Pil Jung, Jae Woong Han, So Young Jung and Jeong Won Kim made up the surgical and pathological team involved in the case. Jae Pil Jung and Ji Won Kim wrote and edited the manuscript. All authors read and approved the final manuscript.


We report a 13-year-old girl with bilateral paratubal cysts and left isolated fallopian tube torsion (IFTT). Paratubal cysts are uncommon in children, and IFTT is a rare complication. Awareness of this entity and prompt surgical intervention could potentially salvage the fallopian tube preserving fertility. Paratubal cysts occur in about 4% of adolescents  and can A 13-year-old postpubertal girl presented with vomiting, left flank, and lower abdominal pain for 5 days. She reported a similar, albeit milder episode 3 months ago. She was being treated by the emergency department for suspected urinary tract infection. On examination, she was afebrile and hemodynamically stable. There was tenderness in the left iliac fossa and differential tenderness on rectal examination, in the Pouch of Douglas.Her hemoglobin was 11.0 gm% and total leukocyte count 10,600 cells/cc . Urine microscopy revealed RBC 15–20 cells/high power field (HPF) and WBC 6–8 cells/HPF. Abdominal sonography revealed a right ovarian cyst 6.4 × 3.8 × 5.4 cm.With a provisional diagnosis of torsion of the ovary, emergency laparoscopy was performed. The distal third of the left fallopian tube along with a 5 × 5 cm paratubal cyst Figures  and 2 haThe postoperative period was uneventful, and she was discharged two days later. The parents were counseled of possible infertility in the future due to fallopian tubal stenosis secondary to the paratubal cysts.IFTT occurs mostly in 12–15 year olds and is rare before menarche or during menopause. IFTT may occur due to intrinsic and extrinsic causes . IntrinsParatubal cysts arise from Mullerian or Wolffian structures and are common in adult females (92% of hysterectomies). These are hormone sensitive and are generally asymptomatic . HoweverThe most common symptom is ipsilateral lower abdominal or flank pain. Physical findings include abdominal tenderness with or without peritoneal signs and adnexal tenderness on rectal examination. A mass may not be palpable. Laboratory values are usually nonspecific.The sonographic findings include normal appearing ovaries, free fluid, a dilated tube with thickened, echogenic walls, and internal debris representing a torsed tube . A whirlLaparoscopy is the gold standard in the diagnosis and management of paratubal cysts complicated with IFTT. Adnexal detorsion with resection of the paratubal cyst and preservation of the fallopian tube (if not gangrenous) is the procedure of choice. Fixation of the detorted tube is controversial. Surgery is usually done too late for tubal conservation. A few reports of detorsion salvaging the fallopian tube with subsequent pregnancy have been reported . Salping


An admixture of hyperplastic and adenomatous components within the same polyp is unusual. Adenocarcinoma arising from a mixed hyperplastic/adenomatous polyp (MHAP) occurs even more rarely. We report the first case of a 59-year-old male who presented with invasive adenocarcinoma originating from a MHAP at a sigmoid colon and synchronous transverse colon cancer. As we know, hyperplastic polyps are rarely precursors of malignancy and play no role in adenoma-carcinoma sequence . HoweverA 59-year-old Chinese man had superficial bladder cancer treated by transurethral resection and intravesical biological therapy half a year previously. He presented with a one-month history of abdominal cramping pain and anemia. There was no family history of colon or breast cancer. Laboratory examinations disclosed his hemoglobin was 9 g/dL and his carcinoembryonic antigen (CEA) was 2.16 ng/ml . A computed tomography (CT) scan found wall thickening of the proximal transverse colon and diverticulosis at the ascending colon. Subsequent colonoscopy showed an annular tumor at the proximal transverse colon and a 1 cm sessile polyp at the sigmoid colon (Figure It is generally accepted that hyperplastic polyps are not precursors of malignancy and play no role in adenoma-carcinoma sequence . HoweverTo the best of our knowledge, only two cases of adenocarcinoma originating from a MHAP were reported in the literature. Despite being uncommon, it may be an entity that is underestimated. Herein, we report a case of invasive adenocarcinoma arising from a MHAP at a sigmoid colon and synchronous transverse colon cancer. Some things learned from this case should be emphasized. First, it is difficult to make a clear distinction among pure hyperplastic polyps, adenomatous polyps or mixed polyps by endoscopic features. Thus, endoscopic polypectomy with a free margin is suggested whenever possible. Second, careful pathologic examination of the resected polyps is recommended since the adenomatous component with dysplasia, even the infiltrating adenocarcinoma, may coexist within the hyperplastic polyp. Third, this case with synchronous lesions provides evidence that patients with MHAP have a risk of developing advanced colon cancer.Written informed consent was obtained from the patient for the publication of this report and any accompanying images.CEA: Carcinoembryonic antigen; CT: Computed tomography; MHAP: Mixed hyperplastic/adenomatous polyp; MSI: Microsatellite instability.The authors declare that they have no competing interests.CCW: writing the manuscript. HKH: provide opinion. YCT: pathologic figure. WCC: revise paper. All authors read and approve the final manuscript.


Squamous cell carcinoma is one of the most common malignant tumors of the skin and oral mucosa. However, squamous cell carcinoma involving near total upper and lower lip and oral commissure is rarely seen in the English literature. Simultaneous reconstruction of the upper and lower lips has been inconclusive and presents a challenge to the surgeon. We report such a case and outline our simultaneous reconstruction with local flaps. To the best of our knowledge this has never been reported.A 73-year-old Thai woman presented with a large rapidly growing squamous cell carcinoma involving the upper lip, lower lip, left oral commissure and left cheek. En bloc resection of upper lip, lower lip, left oral commissure and buccal region was performed. Left radical neck dissection and right modified neck dissection were performed. Reconstruction of the upper lip with a left nasolabial-cheek cervicofacial rotational-advancement flap and right cheek advancement with perialar crescent flap was performed. The lower lip was reconstructed with bilateral labiomental advancement flaps.Squamous cell carcinoma can grow rapidly and spread along the orbicularis oris muscle and across the oral commissure to the opposite lip. In advanced cancer, multimodal treatment is necessary. No gold standard in the reconstruction of both upper and lower lips has been established. We report the case of an advanced squamous cell carcinoma involving both the upper lip, lower lip, left oral commissure and buccal area and simultaneous reconstruction with local flap coverage that, to the best of our knowledge, has never been reported. Squamous cell carcinoma (SCC) is one of the most common malignant tumors of the skin and oral mucosa. SCC of the lips accounts for approximately 30% of oral cavity malignancies.The majority of these occur on the lower lip because of its great exposure to precipitating factors. SCC grows along the mucosal surfaces and infiltrates deeper structures in a predictable pattern. Tumors can spread by direct penetration, tracking along nerve and vascular invasion routes . SCC of A 73 -year-old Thai woman presented with a large rapidly growing, proliferative mass involving her upper lip, lower lip, left oral commissure and left cheek. She had a history of a small proliferative mass on the left lower lip four years previously, a total excisional biopsy that showed chronic inflammation. No residual mass was seen after that. Four months prior to admission, she had a proliferative mass on the left lower lip that rapidly grew to involve the circumoral region and the left buccal mucosa Figure .Incisional biopsy revealed well-differentiated squamous cell carcinoma. Physical examination revealed a tumor mass that involved 90% of the upper lip, 80% of the lower lip, the left oral commissure and the left buccal region including mucosa. An enlarged left submental lymph node was palpable. Sensation along her mental nerve was intact. Panorex X-ray showed no bony destruction. A computed tomography (CT) scan revealed an irregular lobulated contour, heterogeneous enhancing mass involving her upper and lower lips, predominantly on the left side but crossing the midline to the right side of the upper lip Figure . The masEn bloc resection with 1 cm margins was performed including near total upper lip (extended to both nostril sills), near total lower lip, left commissure and left buccal region. Only 0.5 cm of the upper lip and 1 cm of the lower lip could be preserved Figure . In addiExamination of frozen sections revealed no malignancy in any of the surgical margins. The upper lip was reconstructed with a left nasolabial-cheek cervicofacial rotational - advancement flap and a right cheek advancement with perialar crescentic flaps. The lower lip was reconstructed with bilateral labiomental advancement flaps. Internal lining of the left buccal area was achieved with skin grafts Figure .The pathology report showed well differentiated squamous cell carcinoma with lymphovascular invasion Figure . All surFive months later, the patient presented with an ulcerative mass at her left cheek, left axillary lymph node and dyspnea Figure . IncisioSCC can be treated with surgery, radiotherapy or a combination of procedures. In advanced cancer, multimodal treatment is necessary. Early postoperative adjuvant radiation showed excellent results. SCC of the lips should be treated with surgery and postoperative radiation when there are poor prognostic indicators that include multiple levels of positive lymph nodes, extracapsular extension of the cancer in lymph nodes, deep invasion of the primary tumor, neural and vascular invasion, tumor margins less than 5 mm, and a need to take multiple layers of frozen section before a 'clear' margin is obtained . LymphadSCC of the upper lip and oral commissure may drain into the periparotid node so the parotid gland should be examined carefully. Patients with well-differentiated tumors have a metastatic rate of 5% but the most predictive factors for metastasis are large tumor size and high grade tumor . CervicaDefects after surgery of the lips may be classified into upper lip, lower lip, and oral commissure. SCC of the lip usually involves the upper or lower lip and oral commissure, but the behavior of SCC in this case was different. The defects in this case involved three parts of the lip, reconstruction was a challenge and there is no gold standard. Reconstructive lip surgery aims to restore function, obtain a watertight-seal, maintain sensation and avoid cosmetic deformity. Many methods are available for the reconstruction of lip defects such as the Wexler and Dingman flap , the temReconstruction of both upper and lower lips is extremely rare and presents a challenge to the surgeon. Upper and lower lip reconstruction using microsurgery has been reported. Jallali and Malata reported reconstruction in cases of total loss of the upper and lower lips with a free vertical rectus abdominis flap in patients with fulminant pneumococcal septicemia after unsuccessful reconstruction of the upper lip with Webster's perialar advancement flap . Daya reIn our case, we used a local flap in the reconstruction of both upper and lower lips and oral commissure in a patient with SCC, a technique that has never been reported. Although, in advanced stages of SCC, microsurgery is recommended and popular, no 'gold standard' exists for large reconstructions of both upper and lower lips, especially in SCC. Local flaps still provide a predictable method to reconstruct perioral defects following resection for oral cancer . We suggComplications included partial flap necrosis and microstomia. The partial upper lip flap necrosis healed spontaneously without further surgery. In microstomia, we planned to perform a right commissuroplasty after completion of adjuvant radiation but our patient developed tumor recurrence. Although, we could not save this patient, the patient had a better quality of life.SCC is one of the most common malignant tumors but SCC involving near total upper lip, lower lip and oral commissure is rarely seen. In our patient SCC grew along the mucosal surfaces and the tumor could spread along the orbicularis oris muscle. Few reports have been published regarding simultaneous reconstruction of both upper and lower lips. Therefore, we report advanced SCC involving both upper lip, lower lip, left commissure and extending to the left buccal area treated with simultaneous resection and reconstruction with local flaps, a technique that has never been reported.Written informed consent was obtained from the patient for the 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.CB and AP were involved in the care of the patient. CB and AP analyzed the case history, investigation and treatment. CB was the major contributor in writing the manuscript. Both authors read and approved the final manuscript.


Appendiceal mucocele is a rare clinical entity, which is however quite often associated with mucinous ovarian tumor. The coexistence of mucinous cystadenoma of the appendix and endometrial adenocarcinoma has not been reported before. A 49-year-old woman presented to our clinic with postmenopausal bleeding and no other symptom. Endometrial biopsy revealed endometrial adenocarcinoma of endometrioid type (grade I). Preoperative CT scanning revealed an appendiceal mucocele, and a colonoscopy confirmed the diagnosis. The patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and appendectomy. The final histopathological examination showed a mucinous cystadenoma of the appendix and confirmed the diagnosis of endometrioid endometrial adenocarcinoma. The coexistence of appendiceal mucocele and female genital tract pathology is rare. However, gynecologists should keep a high level of suspicion for such possible coexistence. Both the diagnostic approach and the therapeutic management should be multidisciplinary, most importantly with the involvement of general surgeons. Appendiceal mucocele is an uncommon but well-characterized entity that can either present with clinical symptoms or as an incidental radiological or surgical finding. The term includes three histopathological types, that is, mucosal hyperplasia, mucinous cystadenoma, and mucinous cystadenocarcinoma, and the overall prevalence is reported to be 0.2–0.4% among appendectomies. More than half of appendiceal mucoceles are mucinous cystadenomas, have low malignant potential, and can be treated with appendectomy alone, combined with thorough exploratory laparotomy for mucinous peritoneal adhesions (pseudomyxoma peritonei). Notwithstanding, appendiceal mucinous tumors have repeatedly been reported to coexist with ovarian epithelial tumors, especially of mucinous type –3. In faWe present a case of a postmenopausal woman with endometrial adenocarcinoma that was diagnosed with appendiceal mucocele during the preoperative radiological staging with computed tomography (CT) scan, in the context of the relevant literature.A 49-year-old Caucasian woman with two previous pregnancies (gravida 2) presented to our outpatient department with postmenopausal bleeding and no other symptoms. The patient's medical history included a pituitary microadenoma that was operated a year ago, ACTH-dependent Cushing, type II diabetes mellitus, and central type hypothyroidism. Transvaginal ultrasonography revealed multilocular cystic masses in both ovaries, while the endometrial thickness of the uterus was 14 mm. To further investigate the ovarian cystic masses, CT scan of the abdomen was performed. The CT scan confirmed the ultrasonographic findings regarding the ovaries and revealed an appendiceal mucocele with mural calcification. All the serum tumor markers  were normal. The patient was subjected to colonoscopy according to the recommendation of the surgical team that was involved in our diagnostic workup, which confirmed the diagnosis of appendiceal mucocele. Endometrial biopsy by dilatation and curettage (D&C) revealed endometrial adenocarcinoma of endometrioid type (type I), well differentiated (grade 1).The patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy as well as intact appendectomy  with theAn appendiceal mucocele was first described by Rokitansky in 1842 and characterized in 1973 by Aho et al., .In 50% of cases, a palpable mass is found and the patient complains of abdominal pain in the right lower quadrant. In other cases, apparent cystic structure may be an incidental finding during a routine gynecologic screening. The ultrasound of the abdomen often mimics an adnexal mass. CT scan is the ideal imaging method for the diagnosis of appendiceal mucoceles , 6.Both mucinous cystadenoma and cystadenocarcinoma have the potential to seed the peritoneum, leading to pseudomyxoma peritonei or mucinous carcinomatosis. Consequently, removal of the appendix requires caution to avoid potential rupture of the mucocele , 7.The best surgical management of such patients remains controversial. There is no consensus regarding either the optimal surgical procedure, which may be right hemicolectomy or simple appendectomy, or the optimal surgical technique, that is, laparoscopy versus laparotomy . While tMucinous tumors of the appendix and ovary can occur simultaneously in association with pseudomyxoma peritonei. The pathogenetic relationship between the two side tumors, as well as the primary origin, remains obscure. It has been supported that the origin of such mucinous tumors comes from gastrointestinal tract and more specifically from the appendix , 7. TherApart from the coexistence of appendiceal mucocele and ovarian tumors, appendiceal pathology may also be present in patients with cystic fibrosis or carcinoid, although being rare . ConcernTo the best of our knowledge, this is the first reported case where appendiceal mucocele was found in combination with endometrial adenocarcinoma. It is not clear whether there is a common underlying pathogenetic mechanism for this uncommon coexistence. Gynecologists should be able to integrate the identification of an appendiceal mucocele in their diagnostic approach, focusing on ovarian pathologic structures, but at the same time staying alert for additional pathology of the female genital tract, such as the endometrium. In such cases, the diagnostic and therapeutic approach needs to be multidisciplinary, and general surgeons should be involved so as the optimal approach of the appendicular pathology is ensured.


We report a rare case in which hemothorax occurred in addition to hemoperitoneum due to spontaneous rupture of hepatocellular carcinoma (HCC) originating from the caudate lobe of the liver. The case pertains to a 56-year-old female who was transported to our hospital for impaired consciousness due to hemorrhagic shock. Computed tomography (CT) demonstrated ruptured HCC originating from the caudate lobe and accompanying hemoperitoneum and right hemothorax. Hemostasis was carried out by transcatheter arterial embolization (TAE), and surgery was conducted approximately one month after TAE. In the present case, no lesions as possible sources of bleeding were observed inside the pleural cavity, and, moreover, the diaphragm had no abnormalities in the intraoperative findings, suggesting that blood from the ruptured tumor may have traversed the intact diaphragm to enter the right pleural cavity soon after the HCC rupture. However, to the best of our knowledge, no similar cases of HCC have been reported to date, and this case is thus believed to be very rare. This unusual phenomenon may therefore be strongly associated with the location of the ruptured tumor and the formation of a hematoma inside the omental bursa. We discuss the mechanism causing hemothorax in the present case and also review the previously reported cases of ruptured HCC complicated by hemothorax. Spontaneous rupture of hepatocellular carcinoma (HCC) is known to be a condition with poor prognosis. The liver is an organ inside the peritoneal cavity, so the rupture of HCC generally causes hemoperitoneum. Among these cases, few reports exist on the rupture of HCC originating from the caudate lobe,2 in whi3/μl, and the blood sugar level was 256 mg/dl, whereas all other items were within reference values. In addition, the tumor markers measured at a later date were α-fetoprotein (AFP) 814.2 ng/ml  and protein induced by vitamin K absence or antagonism factor II (PIVKA-II) 33 mAU/ml . A routine chest radiograph and a chest CT confirmed the presence of fluid collection in the right pleural cavity. An abdominal CT demonstrated a tumor and extravasation of the contrast medium in the caudate lobe of the liver as well as hemoperitoneum including a hematoma inside the omental bursa. The tumor seemed to be localized in the Spiegel lobe , but her blood pressure was characteristic of shock, namely at 70/40 mmHg, whereas her heart rate was regular at 71/min, her respiratory rate was 18/min, her body temperature was 35.5°C, and tenderness was observed in the epigastric fossa. A blood test indicated that hepatitis B antigen was positive, the hemoglobin was 11.5 mg/dl, white blood cell count was 11400/μl, the platelet count was 14 × 10Spontaneous rupture of HCC is not uncommon in Eastern countries, but there are not many reports on cases regarding ruptured HCC originating from the caudate lobe,2. When On the other hand, hemothorax is a very unusual presentation of ruptured HCC. The PubMed 1966-), Google Scholar, and Japan Medical Abstracts Society databases 1983-) were searched for articles published before May 2012 in English and Japanese languages that report on ruptured HCC accompanied by hemothorax. According to our search, only 16 cases of ruptured HCC complicated by hemothorax have been reported in the literature-19, and 66-, Goog3- were slocus minoris resistentiae around the aorta and/or around the esophagus and penetrating into the pleural cavity; This route taken by the pancreatic juice seems to be similar to the aforementioned one. However, no apparent hemomediastinum was present in our case, but this may have been due to the rupture of the mediastinal pleura occurring earlier than the blood coagulation inside the mediastinum, causing blood to flow into the pleural cavity.The diaphragm is a muscular tissue which separates the thoracic cavity from the abdominal cavity and has three openings: a caval opening, an esophageal hiatus, and an aortic hiatus. The caudate lobe is a section that anatomically comes in contact with the inferior vena cava, wherein in this case, the tumor and hematoma intensely retracted the inferior vena cava. Consequently, it is possible that the blood flowed along the connective tissue sheaths of the inferior vena cava, and, after entering the mediastinum through the caval opening, may have ruptured the pleura and flowed into the right pleural cavity. One example that may support this is a reported case in which the left gastric artery aneurysm ruptured, causing hemomediastinum and right hemothorax. Moreoveet al., who presented three cases of hemoperitoneum with secondary hemothorax in their article[There are other possible routes. Though a precise formation mechanism of hepatic hydrothorax is still unknown, it probably results from the passage of ascites through small diaphragmatic defects. These defects are generally located in the tendinous portion of the diaphragm which is said to be congenitally weak,23. No c article, althougOn the other hand, the lymphatic transfer through the diaphragmatic lymphatics, which is a well-known phenomenon in Meigs’ syndrome, may be Nevertheless, it is believed that the pressure around the tumor and in the omental bursa increased and blood passed through the diaphragm near the caudate lobe in the present case. This passage of blood seemed to occur soon after the HCC rupture, thus resulting in the coexistence of hemothorax and hemoperitoneum on the patient’s arrival at our hospital.Based on the aforementioned factors, at least three factors are necessary to explain the mechanism causing concomitant hemothorax in the present case. The first is the location of the ruptured tumor, that is, in the caudate lobe, which made it possible for the tumor to bleed into the omental bursa. The second is the presence of either the structural weakness or a defect of the diaphragm which may or may not have been congenital. The third is the large amount of bleeding that increased with sufficient rapidity to cause the pressure in the peritoneal cavity, especially in the omental bursa, which caused an unusual amount of stress to bear on a limited part of the diaphragm.The inside of the pleural cavity is negatively pressured, so it was predicted that the escape of blood from the peritoneum into the pleural cavity would make spontaneous hemostasis difficult. In actuality, when considering the amount of chest tube drainage, blood data, and abdominal findings, it is believed that after the patient’s admission into the hospital, most bleeding flowed into the pleural cavity in the present case. As shown in TableIt is said that TAE is not always effective and may not be easily performed for ruptured HCC in the caudate lobe because of the multiple tumor-feeding arteries. SuccessSpontaneous rupture of HCC is capable of causing hemothorax, which leads to very poor outcome. In the present case, the location of the ruptured tumor and the formation of a hematoma inside the omental bursa are considered to be strongly associated with the mechanism causing hemothorax. TAE is therefore believed to be an extremely useful treatment for hemothorax secondary to HCC rupture.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.This research was approved by the ethical committee of the institution.AFP: α-fetoprotein; BCLC: Barcelona Clinic Liver Cancer; HCC: Hepatocellular carcinoma; CT: Computed tomography; JIS: Japan Integrated System; TAE: Transcatheter arterial embolization.Fuminori Ono and other co-authors have no competing interests.FO performed TAE and surgery. FO also drafted the manuscript. Other authors helped to draft the manuscript. All authors read and approved the final manuscript.


A 58-year-old man presented acutely with features of post-surgical adhesive small bowel obstruction. Following an unsuccessful trial of conservative management, computed tomography (CT) of the abdomen was performed. This revealed a mass in the ileocaecal region, for which he underwent a subsequent right hemicolectomy. Histology revealed diffuse B-cell Non-Hodgkin’s lymphoma of the terminal ileum. Confounding obstructive lesion of the intestine in patients with a history of previous laparotomy is extremely uncommon. Early high resolution imaging may predict diagnosis and consolidate clinical management plans. Adhesions after abdominal surgery are so common that often no other diagnosis is considered in patients presenting with symptoms and signs consistent with SBO: dilated small bowel loops on plain abdominal X-ray and abdominal scar(s). Intra-abdominal adhesions are fibrous bands of scar tissue that bind two separate parts of tissue together. These adhesions develop when the body’s repair mechanisms respond to tissue disturbance, such as infection, trauma, radiation and surgery. It is reported that 93% of patients develop adhesions after one laparotomy compared to 10% of patients with no previous abdominal surgery.[Other common causes of SBO include tumour of the caecum, lesions within the small bowel and extrinsic compression by intersussception, hernial neck or extraintestinal tumours. We report a case of a 58 year-old Asian man, with a history of laparotomy for perforated appendicitis a few years earlier, who presented acutely with SBO. Eventually, it was found the cause be due to lymphoma of the terminal ileum. We present the case, discuss management and review the literature.A 58-year-old man presented acutely with clinical and radiological  features of SBO. There was no significant past medical history. Past surgical history included cholecystectomy and emergency laparotomy for perforated appendicitis, which were performed years ago. On physical examination, he was hemodynamically stable. His abdomen was diffusely distended and tender, with no signs of peritoneal irritation. There were no palpable masses and bowel sounds were high-pitched. No external hernias were identified. All laboratory investigations were within normal limits, including hemoglobin concentration. Given that, adhesions were considered the most likely cause. Following an unsuccessful trial of conservative management including nasogastric aspiration and intravenous fluids for 48 hours; contrasted abdominal CT was performed on day 3. This revealed a mass in the ileocecal region , for whiAdhesive SBO accounts for about 1% of all emergency surgical admissions and 3.3% of all emergency laparotomies. While ma


Giant condyloma acuminatum, also called a Buschke-Löwenstein tumor, first described in 1925, is a slow-growing, locally aggressive, destructive tumor of the ano-genital region. Scrotal tumors are rare. Reports on giant condyloma acuminatum lesions in patients with HIV and AIDS are surprisingly even rarer.In this report, we present the case of a 42-year-old African man with AIDS who was undergoing anti-retroviral therapy. He was found to have a giant condyloma acuminatum of the scrotum. Wide surgical excision and scrotal reconstruction with a pedicled anterolateral thigh flap was performed, significantly improving his quality of life.Decision making regarding the goals of surgical intervention in the terminally ill is a complex process. The options include conservative medical palliation or palliative excision versus a curative excision that has the potential for significant morbidity. Wide surgical excision with local flap reconstruction significantly improved the quality of life of the patient described herein. The challenges presented by emerging or unusual presentations of surgical pathology secondary to HIV and AIDS in patients who are on anti-retroviral therapy provide an opportunity for research and the establishment of guidelines for the use of adjuvant chemotherapy in these patients. Giant condyloma acuminatum, also known as Buschke-Löwenstein tumor, was first described by Buschke and Löwenstein in 1925. This slow-growing, locally destructive tumor of the ano-genital region is thought to be induced by human papillomavirus (HPV), most commonly HPV types 6 and 11 and occasionally types 16 and 18. It is associated with extensive local infiltration and a high propensity to recur. Most authors consider it to be a verrucous carcinoma, a variant of squamous cell carcinoma that seldom metastasizes .Scrotal giant condyloma acuminatum tumors are rare. While ano-genital condylomata acuminatum or warts are common lesions in patients with HIV, giant condyloma acuminatum and Buschke-Löwenstein tumors in patients with HIV are very rare ,5.In this report, we describe the case of a patient with AIDS undergoing anti-retroviral (ARV) therapy who presented to our hospital with a giant condyloma acuminatum lesion of the scrotum, and we explore the therapeutic difficulties posed by his care.A 42-year-old African man was referred to our institution from another country with a large scrotal tumor first noted five years prior to his presentation to our institution. The mass had grown slowly over the years, with ulceration associated with foul-smelling discharge noted with time, along with seepage of urine from multiple fistuli in the growing scrotal mass Figure . He had The patient's physical examination revealed that he was emaciated. He had a large scrotal mass that extended laterally into both thighs, superiorly into the supra-pubic region and posteriorly to the anal verge. His hips had a fixed flexion and abduction deformity.3. During surgery, the tumor was found to have extended laterally into the adductor musculature bi-laterally, requiring their inclusion in the excision. The deep margins included the rectus muscle fascia, pubis, pelvic diaphragm, and rectum  versus surgical palliation .Giant condyloma acuminatum, rarely reported in patients with HIV/AIDS, is a rare, locally aggressive tumor that may recur early after surgical excision. Decision making regarding the goals of surgical intervention is complex and involves palliative excision versus a curative excision that has the potential for significant morbidity in a patient who is terminally ill. Wide surgical excision with local flap reconstruction significantly improved the quality of life of the patient presented here.The challenges of treating patients with emerging or unusual presentations of surgical pathology secondary to HIV/AIDS who are receiving ARV therapy provide the opportunity for research and the establishment of guidelines on the use of adjuvant chemotherapy.AIDS: acquired immunodeficiency syndrome; ARV: anti-retroviral; HAART: highly active anti-retroviral therapy; HIV: human immunodeficiency virus; HPV: human papillomavirus.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.PMN, PNg, and PNy came up with the idea of writing the manuscript. PMN, PNg, and PNy made significant contributions to the writing of the manuscript. PMN provided leadership in writing the manuscript. All authors read and approved the final manuscript.


Warts are tumor due to the infection with human papilloma virus HPV. We report the case of a giant wart evolving in the back of a thirty-three year-old man, This had been present since he had 7 years-old, but had been growing rapidly for the two last years. His medical history was devoid of recurrent infections, tumor pathologies. The exam revealed a brown tumor on the right paravertebral region that measured 25cm/7cm. It had irregular borders but well delineated. The surrounding skin was unaffected. Examination of the lymph nodes revealed multiple bilateral adenopathies exceeding 2cm in diameter. The rest of the physical examination revealed no additional abnormalities. A biopsy revealed papillomatosis, hyperkeratosis with parakeratosis, and the presence of koilocytes. The diagnosis was a giant wart confirmed by PCR. Neoplastic pathology was put forward as a second option due to the large size of the lesion, the speed of development, the peripheral adenopathies, as well as impaired overall health. These symptoms prompted the surgical excision of the tumor for a histological analysis on multiple slices of the specimen; no neoplastic proliferation was found. The uniqueness of our observation lies in the size, location, and length of evolution, as well as the unexplained increase in activity in the two years prior to examination. He reported having been employed as a mason during these years and frequently worked topless in the open air carrying heavy sachets on his back. These conditions could explain the warts increased activity related to autoinoculation.


Chondromas are benign cartilaginous tumors usually localized within the tubular bones of the extremities. Soft tissue chondromas (STCs) are rare and only few cases have been reported in the oral cavity. The present case documents the exceptional finding of a 12-year-standing STC of the hard palate of a 63-year-old man. The tumor measured approximately 6 cm in its larger size and it was radically excised through the use of a quantic resonance molecular (QRM) lancet. No recurrence was observed during 1-year follow-up. A concise review of the relevant literature is included in the present paper. Soft tissues chondromas (STCs) are rare, benign tumors occurring in extraosseous and extrasynovial locations. By definition, these tumours are not attached to bone, intra-articular synovium, or periosteum .STCs are usually diagnosed within the soft tissues of the hands and feet, the fingers being frequently affected , 2. OtheOral STCs are apparently more common in the third and fourth decades –14.From the clinical point of view, these neoplasms usually present as nonsymptomatic, slow-growing, and well-defined nodules expanding into the surrounding tissues , 15.STCs are predominantly composed of adult type hyaline cartilage, devoid of other differentiated elements, except osseous, fibrous, and/or myxoid stroma .With specific regard to oral STCs, the tongue is the most common site of occurrence. Sera et al. described an elastic, semicircular nodule with a diameter of approximately 5 mm localized on the dorsum of the tongue. The authors also reported the presence of other 45 cases in the literature of fibrochondroma of the tongue .Onodera et al. reported one of the 5 cases in the literature of STCs of the cheek and de Riu et al. described a case of a STC of the masticatory space , 14. ThiTo the best of our knowledge, only five cases of STC of the palate have been reported in the literature, one of these being associated with cleft palate –20.No reliable theories have been put forward to clarify the etiology and pathogenesis of oral STCs even though an origin from aberrant embryonal cartilage or metaplastic changes due to chronic inflammation or trauma may be hypothesized , 21.A genetic relation to the pathogenesis of this condition, in particular to chromosome 12 and chromosomes 6 and 11, has been hypothesized in two studies , 23.The present case documents the exceptional finding of a STC of the hard palate altogether with a concise review of the literature.A 63-year-old man was referred to the Center of Oral Laser Surgery and Oral Pathology, Dental School, University of Parma, Italy, with a diagnosis at admission of a “fibroma of the hard palate.” Patient complained of a slow-growing 12-year-standing lesion that was starting to interfere with chewing and speech. He stated that he never underwent oral examination before because of a superstitious and popular attitude, considering the lesion as a punishment from God.Familiar anamnesis was negative for chondromas and social history did not highlight relevant aspects.Clinical evaluation revealed the presence of a nodular mass, approximately measuring 6 cm in its larger size, localized on the palatal side of an edentulous ridge corresponding to the 1.3–1.7 region . The lesRadiological examination did not show presence of bone involvement or intraneoplastic calcifications.Working diagnoses included minor salivary gland neoplasm and peripheral ossifying/cementifying fibroma. Because of the pedunculated nature of the lesion, an excisional biopsy, using a molecular quantic resonance scalpel , was performed Figures  and 3. Hμm-thick sections were obtained for hematoxylin and eosin, periodic acid-Schiff, and Alcian Blue. Additional histological sections were cut for immunohistochemical analysis which was performed according to the manufacturers' protocols. Negative control procedures were performed by omitting the primary antibody, in each case.After the excision, the specimen was immediately fixed in a 10% buffered formalin, cut into several tissue slices, and embedded in paraffin tissue blocks, according to conventional methods. Five-On the cut section, this lesion appeared multinodular and blue-greyish with slightly well-defined edges. This appearance corresponded, histologically, to a cartilaginous tumour characterised by multiple nodules composed of mature chondrocytes placed in an abundant blue chondroid matrix . These cSwellings on the hard palate are very common and may depend on a variety of factors including minor salivary glands neoplasms, traumatic, or idiopathic fibrous reactions as well as acute and chronic infectious processes.Very few cases of STCs limited to the hard palate were reported , 17. PatThe majority of patients with a diagnosis of STCs are middle-aged with a range between 26 and 60 years , 9. A slAccording to Onodera et al. intraoral STCs mainly involve the lateral borders and the dorsum of the tongue, even though cases have been described on the soft palate, cheek, tonsil, flabby ridge beneath dentures, and the masticatory space , 16.For tumors of the tongue, a possible pathogenetic mechanism involving the paraphysiologic cartilaginous tissue of the lingual septum  might be considered . Such a Radiological appearance of oral STCs is rather unspecific. These neoplasms may appear as a well-demarcated, lobulated mass with peripheral or central calcifications often curvilinear in nature . HoweverDiagnosis of oral STCs may be difficult and usually requires histopathological confirmation. Several tumors of soft tissue may display cartilaginous differentiation as primary phenomenon  or as a secondary metaplastic process  , 24, 25.According to Nayler and Heim, STCs are microscopically composed of lobules of mature, adult hyaline cartilage, with chondrocytic cells often growing in clusters . One-thiTen to 15% percent of tumors may recur locally after surgical excision [Differently from osseous and synovial cartilaginous tumors, transformation to chondrosarcoma has not been described in extraskeletal chondromas , 8.


Nasal teeth eruption is a rare phenomenon. The variability of symptoms and generic history makes the diagnosis difficult. This difficulty is more challenging when the tooth is placed in the depth of septum.Our case is an example of this problem. Herein, we present a case of intraseptal tooth with nasal obstruction and septal deviation and recurrent sinusitis. We present preoperative imaging.Great suspicion may helpful for preoperative diagnosis and good deciding. Ectopic teeth can be seen in different areas of the body. Nasal teeth eruption is a rare phenomenon . It can A 19 years old male patient, showed up to our clinic with bilateral nasal obstruction, open mouth breathing and intermittent headache, which was more dominant in the mornings. He also complained about episodes of recurrent sinusitis in the past. But he did not complain about nasal discharge at the moment.Neurological exams were normal. No tenderness detected at the time of compression of cheeks. His throat was mildly erythematous; no significant postnasal discharge (PND) was detectable at the time of examination. Rhinologic exam revealed a septal deviation, which was spurred like.CT scan showed severe septal deflection and deviation, and a density in the floor of nasal septum that was noted in the second look .According to the severe septal deviation we decided to perform conventional septoplasty. Killian incision performed and submucoperi- chondrial flap was held, we saw a whitish hard object while dissecting quadriangular cartilage. We pulled it out after septal floor osteotomy; it was relatively developed tooth, which was highly resembled to a canine tooth. It had one root which was not too firm . Not anyEruption of ectopic teeth can be seen in about 0.5% of population ,7. The m If abnormal tissue interactions disrupt the oral epithelium and the underlying mesenchymal tissue interactions process, the result is ectopic tooth development and eruption . It may This condition is accompanied by variety of non specific symptoms which can postpone the diagnosis and therefore complications such as rhinosinusitis, osteomyelitis, dacryocystitis, nasal septal abscess, septal perforation, oronasal fistula and nasal deformity may develop . In Albert Chen and colleagues study they explain the clinic, as a characteristic and determining factor for diagnosing; but in our case physical exam was normal, excepta spur like septal deviation. We did not see any white mass in the nasal cavity; it may because of the site of nasal tooth.Radiologic examinations, especially CT scan, can reveal the intranasal tooth and even the depth of eruption site1 ,8,12. BuHowever other articles suggested early removal of intranasal teeth ,8, it seOur case was a patient with nasal obstruction and headache and positive history of sinusitis with septal deviation as main symptom. Clinical and radiological presentations were not characteristic; so we did not diagnose it until performing the surgery. After surgery all of the symptoms relieved. In this case surgery was both diagnostic and curative. In conclusion,ectopic tooth should be indiferential diagnosis of any radioopaque mass in imaging,such as osteoma and rhinolitis,great suspicion is essential for physician to consider this matter in patient with recurrent sinusitis or nasal obstruction


Malignant triton tumors are relatively rare and highly aggressive tumors in which malignant schwannoma cells coexist with rhabdomyoblasts. Their occurrence in the retroperitoneum is uncommon and has been rarely reported.We present the case of a patient with a retroperitoneal malignant triton tumor. A 32-year-old male was referred with epigastric pain and an abdominal mass. Computed tomography revealed a huge soft tissue retroperitoneal mass that involved adjacent organs and vessels. Complete resection was undertaken. Pathological examination confirmed the presence of a malignant triton tumor. The patient died two and a half months after surgery, as a result of local recurrence.Malignant triton tumors are uncommon sarcomas that are associated with a high incidence of developing local recurrence and distant metastases. Immunohistochemical staining showing nerve sheath differentiation with rhabdomyoblastic cells confirms the diagnosis. Complete excision of the tumor is the most effective treatment strategy for these retroperitoneal tumors. Malignant triton tumor (MTT) is a relatively rare subtype of malignant peripheral nerve sheath tumors (MPNST), histologically defined as a malignant peripheral nerve sheath tumor with additional rhabdomyoblastic differentiation. MTTs are usually located in the head, neck, extremities and trunk . Less coA 32-year-old male, previously fit and healthy, presented with epigastric pain and was found to have an abdominal mass on abdominal ultrasound. He was referred to the First Affiliated Hospital, School of Medicine, Zhejiang University for further investigation and treatment in October 2010. There were no signs of neurofibromatosis type 1(NF-1). Physical examination revealed a firm, ill-defined, fixed mass in the upper abdomen. Laboratory findings, including leukocyte and platelet counts, hemoglobin, serum creatinine, liver function, alpha-fetoprotein, carcinoembryonic antigen and cancer antigen 19–9, were all within normal limits.Contrast-enhanced computed tomography (CT) of the chest and abdomen showed a heterogeneous tissue retroperitoneal mass, approximately 16 cm in diameter  and hypercellularity. Rhabdomyosarcomatous differentiation was evidenced by foci of scattered, round cells with a prominent eosinophilic cytoplasm and atypical nuclei, which were identified as rhabdomyoblasts Figure . MicroscImmunohistochemistry demonstrated positive staining of the rhabdomyoblastic cells for desmin Figure  and vimeAccording to the Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grading system , the tumFollowing surgery, the patient made a good recovery and was discharged two weeks later. However, during his first follow-up assessment one month after surgery, CT imaging revealed multiple lesions in the liver, which were considered a recurrence of the tumor. Clinical deterioration occurred rapidly, and no subsequent treatment was undertaken. The patient died two and a half months after the operation.et al.[et al.[et al.[Rhabdomyosarcomatous elements within MPNSTs were first described by Masson in patients with neurofibromatosis . The namet al. on the bl.[et al. also prol.[et al. later sul.[et al.. The prel.[et al.,12,14. IEpidemiologically, the mean age of patients with MTT has been estimated to be 31.7 years ,4, althoTo date, approximately 170 cases of MTT have been reported in the literature worldwide ,16,17. Aet al.[Due to the rarity of MTT, treatment modalities, especially in advanced and metastatic cases, are not well established, and there are no standardized management guidelines. As with many soft tissue sarcomas (STSs), the most effective therapeutic strategy for retroperitoneal MTT is complete resection of the tumor. The status of the microscopic margins after resection has a significant impact on local outcome and survival . Ennekinet al. describeet al.. In our The role of adjuvant therapy, for example radiotherapy and chemotherapy, is less well defined and has not been proven to be effective ,14,15, aMalignant triton tumors are uncommon sarcomas that are associated with a high incidence of local recurrence and distant metastases. Immunohistochemical staining showing nerve sheath differentiation with rhabdomyoblastic cells confirms the diagnosis of MTT. Complete excision of the tumor is the most effective therapeutic strategy for retroperitoneal MTTs.Written informed consent was obtained from the patient for publication of this case report and the accompanying images. Copies of the written consent form are available for review upon request.CT, computed tomography; FNCLCC, Fédération Nationale des Centres de Lutte Contre le Cancer; MPNST, malignant peripheral nerve sheath tumor; MTT, malignant triton tumor; NF-1, neurofibromatosis type 1; STS, soft tissue sarcomas.The authors declare that they have no competing interests.LZW formulated the manuscript, XJ prepared the histological figures, YS and GF provided the clinical history and clinical figures, ZSS is the guarantor. All authors read and approved the final manuscript.The work is supported in part by the Foundation for Innovative Research Groups of the National Natural Science Foundation of China (No.81121002) and the Zhejiang Provincial Natural Science Foundation (No.J20100398).


Papillary intralymphatic angioendothelioma is a rare, low-grade neoplasm of lymphatic channels that usually presents intradermally or subcutaneously. We report the case of a 24-year-old male presenting with an isolated intratesticular palpable mass and symptoms of testicular pain. Preoperative ultrasound examination showed an irregular, heterogeneous mass. Subsequent surgery and pathologic assessment revealed a papillary intralymphatic angioendothelioma (PILA), formerly known as Dabska tumor within the lymphatic spaces. Papillary intralymphatic angioendothelioma, formerly known as Dabska tumor, was first described in 1969 by Dabska –5.A 24-year-old male initially consulted his urologist for pain and tenderness in the scrotum. There was no previous history of genital trauma. On examination, the scrotum showed a discrete swelling of the upper pole of the left testis. There was no abnormality to the right testis. Ultrasound examination revealed a circumscribed mass with heterogenous echotexture. The possibility of a testicular tumor was suggested, and the patient was sent to hospital for further investigation. Subsequently, orchidectomy was carried out. The specimen was sent for histopathological examination.The gross examination revealed a tumor located centrally in the testicular parenchyma; the shape of the testis was maintained. The tumor measured 1.4 cm in diameter. Outer surface was smooth and brownish in colour. The cut surface was reddish brown with areas of hemorrhage. Microscopic examination showed a lesion consisting of numerous thin-walled ectatic vascular spaces. Many of these contained proteinaceous material and, hence, closely resembled cavernous lymphangiomas, except for the fact that there were prominent intraluminal papillae composed of plump rounded cells with a limited amount of eosinophilic cytoplasm .Immunohistochemical staining revealed strong nuclear positivity for ERG and CD31 in the cells lining the spaces, as well as the papillary tufts. Staining for D2-40 only showed positivity for the vascular channels, suggesting a lymphatic origin of the vessel structure. The papillaries did not show any positive staining results for D2-40 . In 1969, Dabska was the first to describe endovascular papillary angioendothelioma in skin and subcutaneous tissues in 6 cases of children [Since 1969, approximately 30 cases have been described in the literature. The age of the patients ranged from few months to 83 years, and there was no sex predilection , soft tiIn our case as well as one other case of the literature, the tumor was located in the testis. Prognostically, these tumors are low-grade lesions with a capacity to metastasize to regional lymph nodes . Three o


In pediatric patients renal lymphoma frequently presents in the form of multiple, bilateral mass lesions, infrequently as a single or retroperitoneal mass, and rarely as diffuse infiltrative lesions. In patients with apparent central nervous system involvement close attention to other physical and laboratory findings are essential for preventing a delay in the final diagnosis. Herein we present a pediatric patient with renal lymphoma that presented with central nervous system findings that caused a delay in diagnosis.Conflict of interest:None declared. Rapid and accurate diagnosis of non-Hodgkin lowercase L (NHL) the second most common malignant tumor of childhood in developing countries- is important for reducing morbidity and mortality. Radiologically, primary renal lymphoma has a similar appearance in pediatric and adult cases ,2,3. HerAccording to the discharge report of a 4-year-old female patient, she presented to a university hospital with a 1-month history of abdominal distention, and a 5-7-d history of headache and inability to move her right eye. Right-eye proptosis and abdominal distention were observed on physical examination. Laboratory findings were as follows: WBC count: 11,400 mm–3 ; urea: 65 mg dL–1 ; uric acid: 9.4 mg dL–1 ; LDH: 1107 U L–1 ; creatinine: 1.92 mg dL–1 . Additionally, cranial CT performed due to ptosis and inability to move the right eye showed a 35x25x15-mm lesion in the sellar region that exhibited homogeneous contrast enhancement with intravenous contrast material (IVCM) . ContrasAt the time of admission physical examination of the patient showed pale skin, right-eye proptosis, ptosis, right central facial paralysis, external ophthalmoplegia, abdominal distention, enlarged superficial veins of the abdomen, and an abdominal mass with ill-defined borders. The patient’s uric acid level was 18.9 mg dL-1 and suggestive of tumor lysis syndrome; as such, 1 session of hemodialysis was administered. Thereafter, alkaline fluid, allopurinol treatment, and close electrolyte monitoring were performed, and tumor lysis syndrome prophylactic treatment was given. Abdominal USG examination showed renal enlargement, significant corticomedullary separation, and elevated intraabdominal fluid. Abdominal CT showed bilateral kidney enlargement and free fluid . PeripheLumbar puncture showed L3-type blasts in cerebrospinal fluid, which was consistent with Burkitt’s lymphoma. The NHL-BFM 95 chemotherapy protocol was administered to the patient, but following the first course the patient experienced a severe neutropenic sepsis. Meropenem and vancomycin were initially given due to severe febrile neutropenia, and severe oral and anal mucositis. Amphotericin-B and amikacin were added to the therapy on d 3 because of persistent fever, neutropenia, and hypotension, but unfortunately she died on treatment d 5. Pseudomonas aeruginosa was cultured in blood and urine samples obtained during the febrile neutropenic episode. Written informed consent was obtained from the patient’s parents to publish the findings.Renal lymphoma rarely presents as a single mass lesion in 1 or both kidneys, and may present in the form of a retroperitoneal mass and diffuse renal enlargement ,3. Follo5%-10% ,3,4. DiaCT findings of renal lymphoma are generally non-specific ,2,3. ThePalpation of the abdominal mass and an elevated uric acid level were noteworthy in the presented patient, and diagnosis was based paracentesis, despite the absence of enlarged retroperitoneal lymph nodes. Renal insufficiency associated with tumor lysis syndrome is a frequent complication that clinicians must be aware of ,7,8. BilIn pediatric lymphoma cases the presence of renal disease at the time of diagnosis does not indicate a poor prognosis, but recurrence is associated with a 5-year survival rate of only 30% . PrimaryThe authors of this paper have no conflicts of interest, including specific financial interests, relationships, and/ or affiliations relevant to the subject matter or materials included.


Rhinosporidiosis is a disease endemic to South India, Sri Lanka and some areas of the African continent. The nasal lesions can sometimes be confused with nasopharyngeal malignancy. We report here a clinically unsuspected case of rhinosporidiosis, diagnosed correctly by intraoperative FNAC, and later confirmed by histopathological examination. Rhinosporidium seeberi, in recent studies has been established as an aquatic protistan parasite [Rhinosporidiosis is a rare chronic granulomatous disease of mucocutaneous tissue, endemic in South India, Sri Lanka, and some areas of the African continent . The etiparasite . It commparasite . In the parasite . Howeverparasite . Surgicaparasite . We repoA 21 years-old-man presented to the ENT outpatient department with obstruction of nose on right side. On examination there was an erythematous, irregular mass, 3 cm in diameter, obstructing the right nasal cavity and extending into the sinuses as well. No abnormality was seen in the contralateral nasal cavity or nasopharynx. Since, preoperatively, the exact nature of mass could not be clinically established with surety, an intraoperative aspirate smear was prepared to rule out malignancy. Rapid Hematoxylin & Eosin (H&E) and Periodic acid Schiff (PAS) stained smears revealed numerous globular sporangia containing spores  along wiμm and 50–1000 μm in size. These findings made easier the distinction of Rhinosporidium seeberi from another common nasal mycosis etiological agent, Coccidioides immitis. And hence, the intraoperative cytological diagnosis was confirmed.Histopathological examination of the resected mass revealed many globular cysts, each representing a thick-walled sporangium containing numerous daughter spores in a background of fibroblasts and acute and chronic inflammatory cells, covered by flat multistratified squamous epithelium Figures . The endThe patient did not assume any drug therapy and, until today, after one year of followup, during which he, underwent clinical examination twice, remains healthy with no sign of recurrence.Rhinosporidiosis is a rare disease affecting people of any age and sex . InitialRhinosporidium seeberi causes granulomatous inflammation of mucocutaneous sites, presenting most frequently as polypoidal lesions in the nose. Sites like the conjunctiva, trachea, nasopharnyx, skin, and genitourinary tract are less frequently involved [The great majority of cases are sporadic. The etiological agent involved . R. seeberi was debated in the last decades, since the microorganism is intractable to isolation and microbiological culture [The taxonomy of  culture . Moreove culture . Interes culture . Recentl culture . R. seeberi, is through the traumatized epithelium  most commonly in nasal sites [The presumed mode of infection from the natural aquatic habitat of al sites . There ial sites . The nasal lesions may sometimes present clinically as ulcerated growths which could mimic malignant lesions such as sarcomas and carcinomas .The definitive diagnosis of rhinosporidiosis is by histopathology on biopsied or resected tissues, with the identification of the pathogen in its diverse stages, demonstration of sporangia and endospores . The spoHowever, cytodiagnosis on aspirates from rhinosporidial lumps or on smears of secretions from the surfaces of accessible polyps and fine-needle aspirates from lumps provide, with suitable stains, distinctive diagnostic features . The varOn direct examination, the cytological smears show spherules as well-circumscribed, globular structures with several endospores within . The diaRhinosporidium seeberi should be distinguished from another microorganism, Coccidioides immitis [μm versus 50–1000 μm) and contain small endospores (diameter of 2–4 μm). Moreover, Coccidiodes does not stain with the mucicarmine [ immitis . This laThe only curative approach is the surgical excision combined with electrocoagulation. There is no demonstrated efficacy in using antifungal and/or antimicrobial drugs. Recurrence, dissemination in anatomical close sites and local secondary bacterial infections are the most frequent complications . To conclude, Rhinosporidiosis is a condition which both clinicians and pathologists should keep in mind when managing patients from endemic countries with nasal masses. The cytological appearance of fine needle aspiration smears is distinctive, and a definitive diagnosis of rhinosporidiosis can be made easily and quickly in clinically unsuspected cases.


In female adolescents and young adults, malignancies of the genital tract are the most frequent type of cancer, closely followed by Hodgkin’s and non-Hodgkin’s lymphomas.We report an unusual case of sporadic Burkitt’s lymphoma (BL) presenting with massive bilateral ovarian infiltration, peritoneal carcinomatosis and diffuse nodular lesions of the stomach and the intestine mimicking Krukenberg tumor. Diagnostic biopsies were obtained by endoscopy of the upper gastrointestinal tract. With intensive chemotherapy, complete remission was rapidly achieved, without life-threatening tumor lysis syndrome.Besides metastatic gastric adenocarcinoma, BL is an important differential diagnosis in adolescents presenting with Krukenberg tumor. Cancer in adolescents and young adults is 2.7 times more common than cancer occurring during the first 15 years of life. In females aged between 15 to 29 years old, malignancies of the genital tract are the most frequent type of cancer (18%), closely followed by lymphomas (17%) [3/mm3, elevated platelets of 484,000/mm3 . Total serum proteins as well as albumin were slightly reduced with 43 g/L and 23 g/L respectively, with normal liver and renal function tests. Serum lactate dehydrogenase (LDH) level was 334 UI/L, uric acid 334 μmol/l and ferritin 36 μg/l confirming absence of tumor lysis. Both alpha foeto-protein (AFP) and beta human chorionic gonadotropin (βHCG) were within normal range. The abdominal CT and MRI showed bilateral ovarian masses, peritoneal carcinomatosis, ascites and diffuse and nodular thickening of the gastric and intestinal wall  of 16.6 x10Suspecting obstructive endoluminal masses, an upper endoscopy was performed and revealed multiple, large (2 to 3 cm in diameter), raised ulcerated tumors involving the stomach, as well as multiple similar lesions throughout the duodenum and jejunum  followed by BL (21%) .Patients with gastrointestinal BL may present with abdominal pain or distension, gastrointestinal bleeding or intestinal obstruction resulting from direct compression of the lumen by an expanding mass or by an intussusception triggered by the intraluminal projection of the tumor mass -7. TheseSpontaneous intestinal perforation is an uncommon complication of BL, even though previously reported in children . PossiblBurkitt’s and B-cell lymphomas in childhood have an excellent overall prognosis regardless of the location , especially when treated with contemporary chemotherapy protocols .Burkitt lymphoma has to be considered as differential diagnosis in adolescent girls presenting with apparent Krukenberg tumors. Prognosis remains excellent with precise histological and molecular diagnosis, early treatment and careful monitoring and prevention of tumor lysis.The authors declare that they have no competing interests.FZ drafted the manuscript and collected data related to the subject; NVDW was involved in revising critically the manuscript and helped to draft the manuscript; MBP made substantial contributions in conception and design of the manuscript. AN participated in the design of the manuscript and the coordination. All authors read and approved the final manuscriptThe pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2431/12/113/prepub


Dedifferentiation is a phenomenon that is well characterized in a variety of tumors and is defined by the occurrence of a high-grade or undifferentiated tumor, typically unrecognizable regarding its line of differentiation, from a low-grade/borderline neoplasm. This phenomenon has previously been described in 2 uterine leiomyosarcomas, but both were devoid of heterologous elements. The authors describe herein a case of a dedifferentiated leiomyosarcoma of the uterus with osteoid heterologous elements, believed to be the first such reported case. The original tumor was a high-grade leiomyosarcoma with large low-grade and leiomyoma-like areas and whose constituent cells displayed intense nuclear immunoreactivity for both estrogen receptor (ER) and progesterone receptor (PR) in approximately 30% of cells. The tumor recurred six months after its resection as an undifferentiated sarcoma that was negative for smooth muscle markers, but which remained positive for ER and PR. Osteoid production was only identified in the recurrent tumor and was significant in extent therein. This case highlights the immunophenotypic changes that may occur in dedifferentiated leiomyosarcomas, and this possibility should be a consideration when an apparently undifferentiated sarcoma is identified in a patient with a history of uterine leiomyosarcoma. In our case, the expression of ER and PR provided significant supportive evidence of the uterine origin of the recurrent tumor. Leiomyosarcoma of the uterus is uncommon but represents the most frequently diagnosed pure sarcoma of the uterine corpus , 2. The A 48-year-old female underwent a supracervical hysterectomy for presumed uterine leiomyomata. Following a pathologic diagnosis of a uterine leiomyosarcoma, she underwent a bilateral salpingo-oophorectomy with full staging procedures shortly thereafter and was assigned an International Federation of Gynecology and Obstetrics stage of IA after a detailed evaluation of the resultant specimens. She underwent 4 cycles of adjuvant chemotherapy with gemcitabine and Taxotere. Six months after her hysterectomy, she underwent a followup computed tomographic scan, which revealed an 11 cm posterior pelvic mass as well as multiple intraperitoneal serosal implants. An exploratory laparotomy was performed, during which some tumor debulking was done, including a mass that was infiltrating a segment of small bowel. She was started on Adriamycin and is currently alive with disease, 8 months after her hysterectomy.The hysterectomy specimen was received, morcellated, and in aggregate, measured 18 × 16.8 × 5.6 cm, and weighed 579 grams. In addition to conventional leiomyomata, there were several fragments that displayed morphologic features diagnostic of leiomyosarcoma. The latter areas showed a striking spectrum Figures . At one Dedifferentiation is a phenomenon that is well characterized in a variety of tumors and is defined by the occurrence of a high grade or undifferentiated tumor, typically unrecognizable regarding its line of differentiation, from a low-grade/borderline neoplasm, and usually with a well-defined demarcation between them . In the mesenchymomas, with leiomyosarcomatous, osteosarcomatous, and liposarcomatous elements [The question of whether there are any true low-grade leiomyosarcomas of the uterus is controversial, since most of these “low-grade” cases are biologically aggressive . Howeverelements , 14, 1 celements  and 1 caelements . In 3 ofelements –15. In telements . Althougelements  was repoIn summary, a case of dedifferentiated leiomyosarcoma of the uterus with heterologous elements is reported. This possibility should be considered whenever a patient with a history of a resected uterine leiomyosarcoma presents with an apparently undifferentiated pleomorphic sarcoma or a sarcoma with heterologous elements at another site, and myogenic differentiation is not demonstrable in the extrauterine tumor. This case suggests that ER and PR may be useful in establishing a uterine origin for some of these cases, although their expression in the recurrent tumor may be substantially lower than in the primary tumor.


Mesenteric venous thrombosis is a rare cause of intestinal ischemia which is potentially life-threatening because it can lead to intestinal infarction. Mesenteric venous thrombosis rarely develops after abdominal surgery and is usually associated with coagulation disorders. Associated symptoms are generally subtle or nonspecific, often resulting in delayed diagnosis. A 68-year-old woman underwent laparoscopic exploration for small bowel obstruction, secondary to adhesions. During the procedure, an intestinal perforation was identified and repaired. Postoperatively, the abdominal pain persisted and repeat exploration was undertaken. At repeat exploration, a perforation was identified in the small bowel with a surrounding abscess. After the second operation, the abdominal pain improved but anorexia persisted. Contrast enhanced abdominal computed tomography was performed which revealed superior mesenteric venous thrombosis. Anticoagulation therapy with heparin was started immediately and the thrombus resolved over the next 6 days. Although rare, this complication must be considered in patients after abdominal surgery with unexplained abdominal symptoms. Mesenteric venous thrombosis is an unusual cause of intestinal ischemia and potentially life-threatening because it can result in intestinal infarction. Mesenteric venous thrombosis accounts for 5 to 15% of all mesenteric ischemic events and usually involves the superior mesenteric vein . SeveralA 68-year-old woman with a history of previous abdominal surgery presented with abdominal pain and vomiting. One day prior to admission, she noted the gradual onset of abdominal pain. She had one bowel movement but the abdominal pain persisted. The pain was intermittent and gradually worsened. She vomited several times. She underwent a hernia repair 15 years previously and had a lower midline incision, although the details of that procedure were unavailable. On physical examination, her lower abdomen was slightly distended with mild tenderness to palpation. Dilated intestine was palpable, but there were no signs of peritonitis. Nasogastric suction was initiated but inadequate and the abdominal pain persisted. Abdominal CT scan revealed dilated loops of small intestine with a small amount of ascites.The diagnosis of small bowel obstruction was established and exploration undertaken. This was begun laparoscopically which demonstrated multiple areas of adherent loops of small bowel. The adhesions were lysed sharply and further exploration revealed a small bowel perforation, which was repaired in a conventional manner after conversion to open laparotomy.On postoperative day (POD) 1, her temperature increased to 39°C; however, it resolved over five days without specific treatment. The abdominal pain persisted and became more intense on POD 6. Abdominal CT scan was performed on POD 7, which showed a small fluid collection with some air. Due to persistence and increasing severity of the abdominal pain, repeat operative exploration was undertaken on POD 8. Exploration revealed abscesses in the abdominal wall and between loops of small bowel, as well as a site of perforation, which was resected and repaired with a primary anastomosis.The postoperative course was uneventful except for persistent anorexia. Ten days after the second exploration, CT scan was obtained due to the persistent anorexia. The CT scan revealed edematous small intestine and dilatation of the mesenteric veins. The scan also revealed a filling defect in the superior mesenteric vein  suggestiMesenteric venous thrombosis is a rare cause of intestinal ischemia which rarely occurs after abdominal surgery. Its frequency remains obscure but Kim et al. reported that 0.3% of patients after laparoscopic bariatric surgery develop portomesenteric venous thrombosis . James eThe etiologic factors associated with mesenteric venous thrombosis are varied, most commonly associated with coagulation disorders such as Factor V Leiden, protein C and protein S deficiencies, and antithrombin III deficiency –3, 5, 6.The diagnosis of mesenteric venous thrombosis is often delayed because the associated symptoms are usually subtle or nonspecific. The most common symptom is unexplained abdominal pain. Abdominal distension, anorexia, diarrhea, and vomiting have also been associated with mesenteric venous thrombosis –3, 5. SeThe treatment of mesenteric venous thrombosis includes systemic anticoagulation. Surgical intervention is sometimes required. When the diagnosis of mesenteric venous thrombosis is made, systemic anticoagulation with heparin should be started immediately , 2. BrunThis rare complication must be considered when evaluating patients after abdominal surgery with unexplained abdominal symptoms. Nonoperative management is feasible when indicated.


This case illustrates the clinical and pathology characteristics of angiosarcoma that is a rare entity secondary to chronic lymphedema. It is the first reported case for which the c-MYC amplification status was assessed. The diagnostic value of this amplification should be further evaluated in this specific context.Angiosarcomas are rare aggressive neoplasms that can occur secondary to chronic lymphedema (Stewart-Treves syndrome). Although secondary angiosarcomas are commonly described after-mastectomy and/or after-radiotherapy, few cases have been reported in association with chronic lymphedema of congenital origin. We report the clinical, pathological, and cytogenetic findings in a case of cutaneous epithelioid clear cells angiosarcoma that occurred in a 21-year-old woman with hemibody congenital lymphedema. Surgical biopsies of the tumor mass revealed diffuse epithelioid proliferation of clear atypical cells, for which immunophenotyping highlighted the vascular differentiation. Despite  Cases of angiosarcoma are rare and represent less than 1% of all sarcomas . The presarcoma) . Chronicsarcoma) , 6. ThisA 21-year-old woman with right hemibody congenital lymphedema caused by hypoplasia of the lymphatic channels developed a huge tumor mass in her right groin. Lymphedema was diagnosed when she was 3 months old and progressively increased, affecting the right hemibody . She underwent many surgical interventions such as lymphatic aspiration, node autografts, lymphedema tissue excision, labiaplasty majora, and lymphovenous anastomosis to reduce the lymphedema and to treat complications. Despite compression, manual drainage, and pressure therapy, her lymphedema required several hospital admissions for intensive lymphatic drainage, with relative success. She was referred for a sensitive subcutaneous nodule in her right groin. Ultrasound showed a hypoechogenic heterogeneous tumor with liquid content, the size of which rapidly increased from 1.5 cm to 5.5 cm at the widest point in a few weeks. Tomodensitometry showed a heterogeneous, hypervascularized sub-cutaneous nodule. Skin punch biopsies were performed.Low-power microscopy examination showed a poorly differentiated, dense proliferation of clear cohesive epithelioid cells, infiltrating the dermis and arranged in continuous compact sheets in a thin fibrous stroma . High-poThe tumor measured 13 × 9 × 5.5 cm and was of reddish lobulated appearance, with large areas of necrosis . It ulcec-MYC amplification, known to demonstrate a high level of amplification in secondary angiosarcoma (secondary to irradiation or chronic lymphedema) but not in primary angiosarcoma [in situ hybridization (FISH) study performed on the biopsy material and on the surgical specimen (frozen samples) did not show any c-MYC amplification (data not shown). We investigated osarcoma . The fluTwo months after surgery, the patient developed multiple lung metastases, revealed by significant pleural effusion. The patient died 3 months after diagnosis.  We report a case of cutaneous congenital lymphedema-associated secondary angiosarcoma, the diagnosis of which was rendered difficult by its predominant poorly differentiated epithelioid clear cell morphology. Epithelioid appearance is more common in angiosarcomas arising in deep soft tissues . In the The positivity of a panel of vascular immunohistochemical markers, including CD31, CD34, and ERG, usually confirms the diagnosis. ERG is a highly sensitive marker of endothelial cells and vascular tumors. GLUT1 is mainly helpful in the differential diagnosis between malformative vascular lesions and capillary hemangioma, but its positivity has also recently been described in sarcomas and angiosarcomas exhibiting epithelioid features , 11.  Secondary angiosarcoma may involve the skin, deep soft tissues, or deep organs. Cutaneous secondary angiosarcoma can develop in a context of chronic lymphedema (Stewart-Treves syndrome). More than 200 cases of cutaneous secondary angiosarcoma have been reported in the context of lymphedema of the upper extremity, occurring 10 to 20 years following radical mastectomy . In contc-MYC proto-oncogene. High-level gene amplifications of c-MYC were found in 55% (18/33 cases) of secondary angiosarcoma, including secondary angiosarcoma associated with chronic noncongenital lymphedema (number of cases not specified) and not in the patients with primary angiosarcoma (0/28 cases). The authors concluded that, although these tumors were not morphologically distinguishable, primary and secondary angiosarcomas were genetically different entities. In a recent series of 25 cases, Mentzel et al. confirmed the presence of an amplification of c-MYC in all cases of secondary angiosarcoma studied by FISH and showed that such an abnormality was lacking in patients with atypical vascular proliferation (16 cases studied) [c-MYC has been developed and might help to distinguish angiosarcoma from atypical vascular proliferation in difficult cases [ The molecular pathogenesis and cytogenetics of cutaneous secondary angiosarcoma in the context of chronic lymphedema are poorly characterized. Several authors have hypothesized that chronic lymphedema contributed to local immune disorders and to the development and progression of some angiosarcomas . Manner lt cases .c-MYC amplification may therefore be an additional diagnostic tool in the diagnosis of secondary angiosarcoma. Such amplification was absent in the case reported here. However, as this case of congenital chronic lymphedema was, to the best of our knowledge, the first in which a c-MYC amplification was sought, no conclusion can be drawn. The status of the c-MYC gene should therefore be further evaluated in the specific situation of chronic congenital lymphedema. The presence of


Inflammatory myofibroblastic tumors (IMTs) are distinct entities with specific characteristics immunohistochemically and molecularly. They are regarded as “intermediate malignancy” tumors of unknown etiology. We report a case of a 64-years-old woman with a fever and abdominal discomfort for 3 months; a computer tomography was performed indicating gastrointestinal stromal tumor. Histologically the lesion proved to be IMT of the abdomen.  Inflammatory myofibroblastic tumors (IMTs) are relatively uncommon neoplasms. They mainly involve the lungs of children, without sex predilection, whereas, extra-pulmonary locations are manifested mostly in the viscera with a slight predominance in females . Their eHerein we report the case of a middle-aged Caucasian female with a soft tissue mass in the abdomen. A combination of clinical history, biochemical findings, and imaging features raised the suspicion of gastrointestinal stromal tumor (GIST) as initial diagnosis. However the histopathological appearance and the immunohistochemical analysis elucidated any ambiguity concerning the diagnosis of IMT. Complete surgical excision was considered sufficient and followup imperative.3/μL). The white blood cell (WBC) was normal. On admission laboratory tests revealed highly distorted inflammatory markers: C-reactive protein (CRP) value was 170 mg/dL and erythrocyte sedimentation rate (ESR) was 120 mm/h. Thereafter the abdominal ultrasound that was performed raised the suspicion of a mass adjusted of the spleen. An abdominal CT scan revealed a large (13.5 × 7.7 × 8.5 cm), heterogeneously enhanced mass with well defined margins. There was a clear plane between the mass and the adjacent spleen. The mass appeared to adhere to the stomach and seemed to compress the pancreas. Thus, the radiologist's initial differential diagnosis was gastrointestinal stromal cell tumor (GIST) or sarcoma  and thrombocytosis  and a sarcoma.According to English literature, IMTs have a predilection for children and adolescents. In some rare cases it may involve any anatomical part and may arise as late as the eighth decade of life , 14, 15.Surgical excision with clear resection margins is proposed as the principle treatment in all cases . Local rIn conclusion, IMTs are uncommon, true neoplasms with biological behavior that range in most cases from benignancy to the rare aggressive variants. Final diagnosis should be based on histomorphological features and immunohistochemical analysis. Morphology is not a reliable parameter to predict the outcome. Complete surgical excision and a long-term multidisciplinary followup is the most indicated therapeutic approach. Appropriate awareness should be exercised by radiologists to abdominal solid tumors in combination with constitutional symptoms and abnormal hematologic and serologic findings, to avoid misdiagnoses.


Tailgut cysts ) are developmental abnormalities consisting of multiloculated cysts lined by squamous, transitional or glandular epithelium which, albeit rarely, may give rise to malignant transformations. Carcinoid tumours arising in the presacral region are extremely rare and usually benign, and only a few are described in the literature. Case 1: A 63-year-old female diagnosed as having bilateral ovarian cysts underwent surgery to remove a right adnexial mass that was histopathologically diagnosed as a well-differentiated carcinoid tumour. She is currently disease free after 18 months of follow-up. Case 2: A 41-year-old-female diagnosed with hepatic metastases and a solid pelvic mass arising from a moderately differentiated neuroendocrine carcinoma is currently alive with disease after having undergone surgical removal of the mass and several medical treatments. We here describe two different clinical histories of well- and moderately differentiated neuroendocrine tumours (NETs) arising from tailgut cysts in the prerectal space together with a review of the relevant literature. Neuroendocrine tumours (NETs) are a relatively rare and heterogeneous group of usually indolent malignancies, and there have been sporadic reports of NETs originating in the presacral region. Tailgut cysts  are very uncommon, generally congenital developmental abnormalities that consist of multiloculated cysts lined with squamous, transitional or glandular epithelium, and are usually incidentally identified in adult life as a result of radiological examinations performed for other reasons. Albeit rarely, they may give rise to malignant transformations, including NETs or adenocarcinomas. Here, we describe the clinical cases of two Italian female patients with NETs which developed within a tailgut cyst.A 63-year-old woman with a negative family history of malignancy had undergone a hysterectomy because of uterine fibromas when she was 45-years-old. She also had a radiologically diagnosed slipped lumbar disc that did not require pain medication. In July 2008, she underwent radiological examinations because of increased lumbar pain and the emergence of a peri-umbilical mass. Spine magnetic resonance imaging was negative for the presence of a neoplasm and/or suspected mass, but abdominal and transvaginal ultrasonography revealed bilateral ovarian cysts. Her blood carcinoembryonic antigen and cancer antigen (CA) 125 levels were normal, but she had above-normal levels of CA 19.9 . There were no other abnormalities.She underwent bilateral adnexectomy with the removal of a 58 × 35 × 43 mm cystic/solid mass located in the retroperitoneal region and loosely connected to the lower sacrum by means of fibrous attachments, leading to complete functional recovery. After being emptied, the cystic mass measured 40 × 30 × 20 mm. Macroscopically, the external wall was red and smooth, but the internal wall was multiloculated. A brown-red nodule with a diameter of 15 mm arising from the external wall was microscopically described as a NET (carcinoid) mainly with a trabecular architecture probably arising from a retro-rectal tailgut cyst. The histological material was reviewed by a pathologist (GP) at the European Institute of Oncology in Milan, who confirmed the diagnosis of a well-differentiated NET (based on the.2000 WHO classification). The trabecular architecture was compatible with a retro-rectal tail cyst origin. The immunophenotype was positive for cytokeratin AE1–AE3, synaptophysin, pancreatic polypeptide (PP), acid phosphatase and, focally, chromogranin A. The proliferation index (Ki-67) was <2%. Peritoneal washing did not reveal malignant cells. The patient received no medical therapy and after a follow-up of 25 months, is in good clinical condition with no clinical, biochemical or radiological signs of recurrence .In111-Octreotide scintigraphy showed several regions of uptake in the liver and hypogastric region. Blood chromogranin A and neuron-specific enolase levels were normal.This is the case of a 41-year-old woman who gave birth to twins in March 2003. In April of the same year, she underwent abdominal ultrasonography because of persistent rectal pain during evacuation; a solid mass was found close to the rectum. Subsequent colonoscopy excluded the presence of inherent lesions. A chest-abdomen computed tomography (CT) scan revealed a thickened rectal wall, the solid mass and multiple liver metastases. A total body The patient underwent posterior surgery with intersphincteric and pera-sacrococcygeal excisions. The surgical specimen reviewed by a pathologist (GP) at the European Institute of Oncology in Milan indicated a moderately differentiated (Ki-67 = 18%) neuroendocrine carcinoma (on the basis of the 2000 WHO classification) associated with residual epithelial cystic material. The immunophenotype was positive for chromogranin A, synaptophysin, cytokeratin AE1–AE3, and somatostatin receptor subtype 2. On the basis of the clinical, morphological and immunohistochemical findings, a clinically malignant, presacral carcinoid with liver metastases was diagnosed .In111-Octreotide scintigraphy scan revealed multiple suspected bone metastases. The patient received long-acting somatostatin analogue therapy and experienced stable disease until October 2009, when total body CT revealed the recurrence of multiple nodes. She is currently receiving PRRT with radiological stable disease.Between November 2003 and April 2004, the patient received six cycles of chemotherapy (carboplatin plus etoposide) because of CT evidence of progression in the number and size of the liver lesions. Subsequently, she received peptide receptor radionuclide therapy (PRRT) from June 2004 to November 2005 because of increasing liver lesions, which led to a partial tumour response. After the appearance of pleural lesions and a solid right adnexal mass, she underwent hysterectomy and bilateral adnexectomy, and the diagnosis of a neuroendocrine carcinoma was histologically confirmed only in the left adnexa. Simultaneously, a second total body Published reports of carcinoid tumours of the presacral region are rare and mainly involve direct extensions of a rectal mass . They arThe close association of presacral carcinoid tumours with tailgut cysts suggests that both probably originate in the hindgut and remnants of the tailgut or neuroenteric cord may be the origin of tailgut or presacral space carcinoid tumours. The cysts infrequently extend from the presacral space to involve the rectal wall and rarely extend laterally from the mid-line or into the postsacral space, or laterally and anteriorly to the rectum.To the best of our knowledge, only 28 cases of carcinoid tumours of the presacral region are described in the medical literature and of these 14 were associated with tailgut cysts. The rest were either associated with teratomas or with no other peculiar anomalies . AlthougHere, we describe the different behaviours of two well-differentiated NETs arising from RCHs. In the first case, despite the absence of residual epithelial material, the anatomic location of the NET and its benign behaviour make it very likely that it originated from a tailgut cyst. The second case demonstrates that not all NETs arising from tailgut cysts are benign. The second patient showed metastatic dissemination at the time of initial diagnosis and evidence of clinical recurrence over a 7-year observation period. The treatment of these malignancies may therefore vary from case to case. The time of the diagnosis, the radical nature of surgical resection and a histopathological diagnosis are the most important prognostic factors.Although NETs arising from tailgut cysts in the retro-rectal area are extremely rare and generally benign, they should be considered in the differential diagnosis of an adnexal mass evidenced by radiological and pathological means. The behaviour of our two cases are in line with their Ki-67 index values, thus indicating this as reliable predictors in NETs.


Colonoscopy is a widely used diagnostic and curative procedure. Extraperitoneal perforation with pneumoretroperitoneum, pneumomediastinum and subcutaneous emphysema combined with intraperitoneal perforation is an extremely rare complication. We report a case of a 78-year-old woman presented to the emergency department with abdominal pain and diffuse abdominal, chest, neck, and facial swelling appeared after a diagnostic colonoscopy. Diagnostic and therapeutic modalities are discussed. Colonoscopy is a widely used diagnostic and curative procedure. However, bleeding, perforation, and postpolypectomy coagulation syndrome may variably occur. The incidence of colonic perforation after colonoscopy is very rare and estimated in 0.19–0.21% [3), anemia (Hb: 9 g/dL), and minimally elevated C-reactive protein (2.32 mg/dL). Temperature was slightly elevated (37.8°C). Vital parameters were within normal limits and no signs of respiratory distress were observed (oxygen saturation 97%). Additional information was obtained from the gastroenterologist who performed the colonoscopy. He mentioned extensive diverticular disease of the sigmoid colon and insufficient mechanical preparation of the colon with solid stools. He attributed the perforation to the effect of the air insufflations in connection with the observed diverticula. The patient underwent chest radiograph and chest and abdominal computed tomography (CT) scan. Right subdiaphragmatic air and diffuse subcutaneous emphysema were observed  and elevated C-reactive protein . Clinical examination revealed intense abdominal pain with rebound tenderness. We initially decided a conservative treatment. Fluids and antibiotics (ciprofloxacin and metronidazole) were administered. 24 hours later, the patient's clinical condition was worsening. Temperature was elevated (38.8°C) while additional laboratory findings revealed leukocytosis . This complication rarely occurs after diagnostic colonoscopy (0.06–0.17%) [ Perforation may be caused by different mechanisms. Pneumatic perforation is the consequence of high pressure insufflations resulting in excessive distension of the bowel wall and rupture. Mechanical rupture should be attributed to the pressure performed by the endoscope against the colonic wall. Perforation commonly occurs at points of previous colonic lesions or weakness such as diverticula with or without diverticulitis, inflammatory, or neoplastic processes and recent colonic operations . Forcibl The retroperitoneal perforations are uncommon. Cirt et al.  reviewed On the other hand, combined intra- and extraperitoneal paerforation during colonoscopy is extremely rare. In the study of Cirt et al. , only el In our case, the patient had a simultaneous intra- and extraperitoneal perforation. Diagnosis of retroperitoneal involvement was posted in relation to the subcutaneous emphysema. Intraperitoneal perforation was clinically suspected and confirmed through radiologic examination. We initially opted for the non-operative management. The important changes of the patient's clinical condition with evident signs of peritonitis made us consider operation in the means of infection source control. Minimal fecal contamination and timing in diagnosis  allowed us to perform resection with anastomosis. Intra- and extraperitoneal colonic perforation following diagnostic colonoscopy is extremely rare. Non-operative management may be achieved when no signs of peritonitis or hemodynamic instability exist. The choice of operative management should be strongly related with clinical, laboratory, and radiologic changes during non-operative management, with diffuse peritonitis and with colonic pathology that requires surgery.