Myeloid Sarcoma is a rare tumor composed of myeloblasts occurring at an extramedullary site like bones, or various soft tissues. Myeloid sarcoma may involve the gastrointestinal tract very rarely either solitarily, or occurring simultaneously with acute myeloid leukemia. Its diagnosis is challenging and needs biopsy and immunohistochemical staining. We are describing a case of myeloid sarcoma which presented as a painful anal ulcer mimicking an atypical fissure. Its appearance resembled crohn's disease on sigmoidoscopy. A biopsy of the ulcer along with histochemical staining led to the diagnosis of myeloid sarcoma. Our case demonstrates the need for aggressive evaluation of any common gastrointestinal complaint with an atypical presentation. Myeloid Sarcoma, also know as, “Chloroma,” “Granulocytic Sarcoma,” “Myeloblastoma,” or “extramedullary myeloid tumor,” is a tumor formed by immature myeloid cells or myeloblasts at an extramedullary site. Myeloid Sarcoma can precede the onset of acute myeloid leukemia and can also occur during active phase of leukemia. It may indicate the relapse of leukemia in a previously treated patient, or the blastic transformation of a chronic myeloid leukemia. Head and neck structures, bones, oral cavity, soft tissues, lymph nodes, skin, mediastinum, and reproductive organs are frequent sites of involvement of myeloid sarcoma. The involvement of the gastrointestinal tract is very rare. We report a unique case of myeloid sarcoma presenting as a large painful anorectal ulcer mimicking an anal fissure and Crohn's disease in a young pregnant woman. A 30-year-old pregnant Caucasian female complaining of severe anal pain and an anal bump was referred to the Colorectal Surgery Office by her primary care physician. She was 8-week pregnant. Her pain started a few days earlier and she noticed a small amount of bright red blood on the toilet paper and coating the stools. She had been constipated. She stated the pain was worse during and after defecation. Her medical history was significant for irritable bowel syndrome, recurrent oral ulcers, and anal pruritis. She denied abdominal pain, nausea, or vomiting. Her family history was significant for inflammatory bowel disease in her maternal relatives. She denied any history of anal intercourse or instrumentation. Upon examination there were no significant physical findings with the exception of what appeared to be an atypical fissure with heaped up skin edges and ulceration in the center in the left posterior position. A local block was placed and a steroid injection was performed. Topical lidocaine mixed with diltiazem and high fiber supplementation were prescribed.Her pain transiently improved but upon repeat examination two weeks later she was having recurrent pain and there was no change in the appearance of the fissure. She was admitted for pain control, an examination under anesthesia, and a flexible sigmoidoscopy. The differential diagnosis upon admission was new onset Crohn's Disease or a severe anal fissure. Upon admission, her vitals were normal and anal inspection revealed two large skin tags. A digital rectal exam could not be done secondary to severe tenderness. Labs showed normal serum electrolytes, WBC 3900, platelets 133000, Hb 11 g/dL, Hct 31.5, and MCV 91.She was taken to the operating room and examination demonstrated a large deep anal canal ulcer in the left posterior-lateral position . No obvi In the following few days, the results of the peripheral blood smear and pathology returned. The smear contained 5% blasts. The biopsy from the anal ulcer depicted squamous mucosa with ulceration and submucosal atypical hematopoietic cell infiltrates suggestive of acute leukemia. Similar findings of discrete mucosal and submucosal aggregates of atypical large and medium-sized hematopoietic cells were noted on rectal biopsies. She was immediately readmitted and a bone biopsy was performed. Her results with flow cytometry showed hypercellularity with 53% blast cells. The abnormal cells were positive for CD13 (58%), CD33 (73%), CD15 (59%), CD45, CD34, CD64, CD4, HLA DR, and MPO markers consistent with acute monocytic leukemia (FAB M5b). Refer to Figures She was diagnosed with acute myeloid leukemia and started on high-dose Idarubicin and cytarabine for induction chemotherapy. Intrathecal methotrexate was also given as there was evidence of central nervous system disease. Her pregnancy was terminated therapeutically before the chemotherapy was started. A repeat bone marrow biopsy nine months later after completing chemotherapy did not show any atypical cells. She clinically has no evidence of disease at this time. Myeloid sarcoma is a tumor composed of immature myeloid cells or myeloblasts at an extramedullary site often in close proximity to bone. Myeloid sarcoma can occur as a solitary mass or multiple nodules in different tissues or organs such as skin, bone, head and neck structures, oral cavity, gingiva, salivary glands, lymph nodes, central nervous system, and genital tract, and rarely in the gastrointestinal tract , 2. Myel The involvement of the gastrointestinal tract in different hematologic malignancies occurs at an advanced stage of the disease or as a complication of chemotherapy and radiotherapy. The most common findings on endoscopy includes esophagitis, colitis, gastritis, gastric erosions, duodenitis, proctitis, and polyps . Myeloidt [t [ Myeloid sarcoma poses a significant diagnostic challenge. Biopsy and staining with immunohistochemical markers play a vital role. The immunophenotype is characteristic based on whether myeloid sarcoma is granulocytic , monoblastic , myelomonoblastic , megakaryoblastic , or erythroblastic variant (glycoprotein c+) . It is dt  which out [t  and prest [t , 29. Mye The patient in this report presented with anal pain which was initially presumed to be a fissure and treated accordingly. Her history of irritable bowel syndrome and her family history of inflammatory bowel disease gave the clinical impression of Crohn's Disease with an atypical anal ulcer. An aggressive workup leading to admission and an examination under anesthesia led to the “confirmatory” diagnosis of anorectal Crohn's Disease. The smear and histopathology with immunohistochemical markers diagnosed the myeloid sarcoma. A subsequent bone marrow evaluation, CSF analysis with flow cytometry, and cytogenetic studies confirmed the AML to allow initiation of treatment. We did not find any association of pregnancy with increased incidence of myeloid sarcoma or acute myeloid leukemia in our review. Though, myeloid sarcoma was reported to involve the female genital tract –32 and pIt is unusual to find a diagnosis of myeloid sarcoma and AML with patients who present with common gastrointestinal problems. Our case illustrates the need for aggressive evaluation in any patient presenting with atypical gastrointestinal complaints.


Liposarcomas are malignant tumors derived embryologically from mesodermal tissues. An unusual site of presentation is the spermatic cord, presenting as an inguinal or scrotal mass. We report a rare case of a liposarcoma of the spermatic cord, mimicking a testicular tumor. The patient was operated, and an orchidectomy, including the tumor, was performed. To our knowledge, there are about 185 similar cases reported in the literature. Liposarcomas are malignant tumors derived embryologically from mesodermal tissues. They represent the most common soft-tissue sarcomas, and they can occur in any part of the body that contains fatty tissue. Although lipomas of the inguinal canal, derived from the spermatic cord, are a usual finding in many operations on the inguinal canal or the scrotum, when they are excluded from diagnosis in cases of spermatic cord masses, the possibility of malignancy is as high as 56% . LiposarA 73-year-old Caucasian male presented with a slow-growing left scrotum mass in the Outpatient Department of the Urology Department of Larnaca General Hospital. He had a personal history of hypertension and hypercholesterolaemia treated with medication. The mass was first noticed by the patient 18 months ago, and it was continuously growing ever since, without the patient seeking medical advice before. In physical examination, the mass was large, hard and was interpreted as a testicular tumor. Ultrasonography indicated a left testicular tumor, and further investigation was advised. The patient was admitted, and a surgical intervention for biopsy and possible orchidectomy was decided. In surgery, the mass was found to be not of testicular origin, but it was growing from the spermatic cord, descending into the scrotum, around the testicle. The mass was measuring about 10 cm, it had a yellowish lipoma-like texture, but in the center of the mass, a hard round tumor was palpated. A left orchidectomy was decided and performed, including the mass, which was sent for pathology. The patient's postoperative course was uncomplicated, and he was discharged on the 2nd postoperative day. Pathology report revealed a liposarcoma measuring 10 × 8 × 7 cm, with evidence of myxoid degeneration, with mitotic activity (0–3 mitoses/10 HPF). Immunohistochemical analysis was negative to HMB45, Melan A, CD34, CD31, HHF35, Desmin, SMA, and ceratines AE1/AE3, but was positive to S100.≈25%) [Liposarcomas of the spermatic cord are extremely rare malignant tumors, representing 3%–7% of all paratesticular sarcomas , 3. They≈25%) –13. Well≈25%) . ChemothIn conclusion, liposarcomas of the spermatic cord represent a rare type of tumors, which are often misdiagnosed preoperatively. To our knowledge, there are about 185 cases reported in the literature, and because most of the cases are in the form of case reports or cases series, there is not a clear view regarding the physical course and the proper treatment and prognosis of the disease.


Gastric duplication cyst (GDC) with a pseudostratified columnar ciliated epithelium is an uncommon malformation supposed to originate from a respiratory diverticulum arising from the ventral foregut. Morphologic appearance of GDCs is variable, depending on the density of their contents. GDCs are often misdiagnosed as solid masses by imaging techniques, and as a consequence they may be wrongly overtreated. We report our case of a 56-year-old man with a 5 cm hypoechoic mass of the gastroesophageal junction, incidentally detected by transabdominal ultrasonography. Neither transabdominal ultrasonography nor magnetic resonance clearly outlined the features of the lesion. The patient underwent endoscopic ultrasound (EUS), which showed a hypoechoic mass arising from the fourth layer of the anterior gastric wall, just below the gastroesophageal junction. According to EUS features, a diagnosis of gastrointestinal stromal tumor was suggested. EUS-guided fine-needle aspiration cytology revealed a diagnosis of GDC with pseudostratified columnar ciliated epithelium. We therefore performed an endoscopically-assisted laparoscopic excision of the cyst.In conclusion, whenever a subepithelial gastric mass is found in the upper part of the gastric wall, a duplication cyst, although rare, should be considered. In this case, EUS-guided fine-needle aspiration cytology could provide a cytological diagnosis useful to arrange in advance the more adequate surgical treatment. Duplications of the alimentary tract are relatively rare congenital anomalies. Those located in the stomach are very uncommon, constituting between 4 and 9% of all intestinal duplications . The strDiagnosis of a gastric duplication may be difficult even using the most advanced imaging techniques, including endoscopic ultrasound (EUS) -6. Here A 56-year-old man, with a history of B-related chronic hepatitis under antiretroviral treatment, was referred to our surgical department by the Infectious Diseases Unit, where he was under follow-up. The patient did not complain of any symptom concerning the gastrointestinal tract. During an abdominal ultrasonography, a hypoechoic round-shaped mass 4.7 cm in size, with regular margins, located between the left lobe of the liver and the anterior surface of the pancreatic body, was found. Magnetic resonance imaging confirmed the presence of a cystic mass with complex content, located anteriorly to the gastroesophageal junction.The patient was then submitted to EUS, in order to better define the structure of the lesion and its relationship with the adjacent organs. EUS showed a hypoechoic mass with slightly heterogeneous internal echoes and regular margins, located just below the gastroesophageal junction Figure . The lesLater on, the patient underwent a surgical intervention carried out by an open laparoscopic approach with a transhumbelical Hasson trocar (without a Verres needle to obtain the pneumoperitoneum) and four additional trocars (two of 10 mm and two of 5 mm). Once the lesion was clearly identified, the overlying serosa was cut by a harmonic scalpel . Through a cautious dissection performed under endoscopic control in order to keep the cyst intact, to prevent perforation of the gastric wall, the mass was totally exposed and then completely resected using a linear endoscopic stapler . The surgical procedure was completed by performing a Dor fundoplication. The patient had an uneventful postoperative recovery and was discharged on the seventh postoperative day.Pathologic examination of the surgical specimen revealed macroscopically a cystic lesion 5 cm × 3 cm × 3 cm in size with a mucoid content. Microscopically the cystic wall consisted of mucosa, subepithelial connective tissue, a layer of smooth muscle and an outer fibrous capsule. Focally the mucosa was lined by gastric foveolar epithelium with cardial glands but most of the cystic wall was lined by a pseudostratified columnar ciliated epithelium  are supposed to originate from a respiratory diverticulum, arising from the ventral foregut . This tyDespite advances in imaging, cysts that contain solid secretions can often be misclassified as soft tissue masses. A rate of computed tomography misdiagnosis ranging from 43 to 70% of cases has been reported ,10. MagnOn the basis of EUS morphologic findings alone, a GDC may be misdiagnosed as a GIST, which is the most common gastric subepithelial lesion, as in the case reported by Jiang and colleagues . In the Owing to the report of gastric cancer arising in gastric duplication ,21-23, sIn summary, GDCs – particularly those with respiratory epithelium – represent a rare disease, often misdiagnosed as GISTs, which are more common. Nevertheless, these lesions should be considered in the differential diagnosis of subepithelial gastric masses, especially if located in the upper part of the gastric wall. Using this technique we will not take any chance on treating a FDC of the stomach through unnecessary extended gastric resection.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.EUS: endoscopic ultrasound; FDC: foregut duplication cyst; FNAC: fine-needle aspiration cytology; GDC: gastric duplication cyst; GIST: gastrointestinal stromal tumor.The authors declare that they have no competing interests.


Perforation of the inflamed diverticula is a common diverticulitis complication. It usually leads to the formation of a local abscess. In some rare cases, the inflammatory process may spread towards extra-abdominal sites like the anterior or posterior abdominal wall or the thigh and form an abscess in these sites. We present the case of a 73-year-old man with a history of pain at the lower left quadrant of the abdomen for 20 days and a visible mass in this site. Ultrasonography and computed tomography revealed this mass to be an abscess of the abdominal wall which had been formed by the spread of ruptured sigmoid diverticulitis by continuity of tissue through the lower left abdominal wall. Local drainage of the abscess was performed and the patient was discharged after alleviation of symptoms and an uneventful course. We also discuss causes of abdominal wall abscesses along with the possible pathways by which an intra-abdominal abscess could spread outside the abdominal cavity. Diverticular disease is estimated to have a prevalence of 20% to 60% and an incidence which increases with age and is rather uncommon in people under 40 years of age. Diverticulitis is the most common complication of diverticular disease and commonly results in perforation of the colon, accounting for 60% of all cases or functional obstruction of the bowel. Perforation of the colon can usually form a diffuse phlegmonous infiltration, a local abscess, or a colovesical fistula . In someWe present the case of a patient with an abdominal wall abscess which was caused by perforation of inflamed diverticula of the sigmoid colon along with some other cases of extra-abdominal spread of diverticulitis from the literature.A 73-year-old man presented to the emergency department with a history of pain in the left lower quadrant of the abdomen for 20 days. He denied any bowel habit change or being nauseous.μL) with neutrophilia (77.7%). All other haematological and biochemical indices were within normal range.Abdominal examination at presentation revealed a smooth abdomen with tenderness and a clinically visible and palpable mass in the left lower quadrant. Abdominal auscultation revealed normal bowel sounds. Regarding his past medical history, the patient had diabetes, hypertension, chronic obstructive pulmonary disease and had undergone surgery in the prostate and coronary bypass surgery. Laboratory tests showed an increased white blood cell count .The initial thoughts about the differential diagnosis of this abdominal mass included a haematoma, an abscess of the abdominal wall, or some type of hernia. The patient was referred to Radiology Department for an ultrasound examination of the abdominal cavity and the mass. The internal organs were all normal. The ultrasonography of the left lower quadrant of the abdominal wall demonstrated a relatively well-demarcated, oval-shaped mass with mixed echogenicity (relatively more hyperechogenic) whose dimensions were 6.5 × 2.12 cm . The patContrast-enhanced abdominal multidetector computed tomography (MDCT) was performed the next day of admission and revealed a bilocular abscess inside the lower left lateral abdominal wall. The MDCT also demonstrated inflammation and perforation of multiple sigmoid diverticula with wall thickening of the sigmoid, localised small extraluminal air collection, and fat stranding in the area of the sigmoid. This inflammatory process was located in proximity to the abdominal wall abscess and communicated with it by continuity of tissue. The spread of the inflammation from the abdominal cavity to the abdominal wall was done through the fascia connecting the rectus abdominis with the lateral abdominal muscles. There was also a hiatal hernia containing the stomach and an umbilical hernia Figures , and 4.Right after the abdominal MDCT, pus was drained from the abscess . During There are several causes of abdominal wall abscesses (AWA). AWAs occur frequently postoperatively, in laparotomy incisions. Other causes of AWA found in the literature are rare and include postoperative gallbladder disease, infections like amoebiasis or non-typhi salmonellosis, malignancies of the transverse colon, xanthogranulomatous pyelonephritis, and diverticulitis of the colon complicated by perforation. In general, inflammatory diseases located inside the abdominal cavity do constitute a cause of AWA. Such intra-abdominal diseases include acute appendicitis, gynecological disorders, Crohn's disease, diverticula of the colon, cholecystitis, and perforation of the intestine. Finally, other AWA may also be caused by iatrogenic causes like placement of catheters, foreign bodies, or rudiments of the urachus . To the Perforated diverticulitis may be the cause of extra-abdominal manifestations like abdominal wall abscesses or abscesses in the thigh, hip, and buttock, especially in older patients. Once an abdominal wall abscess is suspected in the differential diagnosis, a CT examination of the abdomen may demonstrate the exact extent of the pathology and possible intra-abdominal diseases accounting for it.Rothenbuehler et al. reported a case series of 5 patients with inflammatory processes of the abdominal wall and thigh, all caused by diverticulitis. This number of patients was observed during 11 years and after 263 patients had been operated for diverticulitis. These numbers show how rare the extraperitoneal spread of diverticulitis is. Four out of the five patients reported by Rothenbuehler et al. had diverticulitis of the sigmoid like our patient and one had the disease localized in the ascending colon. All patients had abdominal pain for 2 to 8 weeks before hospital admission and increased white blood cell count. Local drainage was performed in all patients and was followed by resection of the affected part of the colon [Stahlgren and Thabit reported in 1961 that the presence of superficial gas in the abdominal wall or thighs can be an indication of unsuspected intra-abdominal or retroperitoneal abscess. This association was first established by Rodlaha in 1926 on a patient with subcutaneous emphysema and sub diaphragmatic abscess caused by perforation of a gastric ulcer. Many reports followed his observation during the following years. In their paper, Stahlgren et al. presented six patients with subcutaneous emphysema and intra-abdominal or retroperitoneal abscesses.Stahlgren and Thabit also tried to identify the possible pathways by which a paracolic abscess could spread outside the abdomen. They reported seven anatomic routes: (1) along nerves and vessels penetrating the abdominal wall, (2) along the inguinal ring, (3) along the iliopsoas muscle and the femoral vessels to the anterior thigh, 4) through the obturator foramen into the ischiorectal fossa, (5) to the gluteal region and hip through the fossa piriformis, (6) into the perineum along the rectum and finally (7) along Denovillier's fascia to the external genitalia. In our case, we speculate that the inflammatory process spreads to the anterior abdominal wall through the fascia connecting the rectus abdominis muscle and the lateral abdominal muscles, possibly along nerves or small blood vessels [ through As Stahlgren and Thabit pointed out, one possible pathway of the inflammatory process of diverticulitis is through the psoas muscle to the thigh. Rao et al. reported a case of such a patient with hip pain and subcutaneous emphysema of the left lower limb. This patient's abdominal CT scan revealed an abscess within the psoas muscle which was the result of retroperitoneal perforation of sigmoid diverticulitis which had spread to the left lower limb . RotsteiApart from the aforementioned pathways, intra-abdominal inflammations may spread outside the abdominal cavity through “locus of minus resistencia” of the abdominal wall. The lumbar triangle of Petit (or inferior lumbar triangle) is such a site of the posterior abdominal wall. Thus, Coulier et al. reported two cases of gastrointestinal perforations extending through the triangle of Petit and forming extraabdominal lumbar abscesses. The first case was caused by perforated appendicitis and the second was caused by left colonic diverticulitis, as in our patient. Abdominal CT posed the diagnosis in both cases. However, as the authors comment, this kind of intra-abdominal inflammation spread is extremely rare. In general, the retroperitoneal space communicates with the leg along the inguinal ligament, the femoral canal, the sciatic foramen, or the obturator foramen and, through these pathways, retroperitoneal inflammations may spread to the leg .The superior lumbar triangle (or Grynfeltt triangle) also constitutes a weak site of the posterior abdominal wall and thus retroperitoneal inflammations may extend through it to the posterior abdominal wall. Ishigami et al. reported a case of a patient with retroperitoneal appendicitis which extended through the Grynfeltt triangle to the posterior abdominal wall .The formation of an abscess is the second most frequent complication of a perforated cancer of the colon and occurs in 0.3 to 0.4% of the cases. Tsai et al. mention that in half of their patients with perforated colonic cancer, ruptured diverticulitis was the initial diagnosis. This is why attention should be paid in patients presenting with intra- or extraabdominal abscesses. Ultrasonography and computed tomography are of great assistance towards the right diagnosis as they can accurately detect the cause of the abscess preoperatively .Ingestion of a foreign body like a chicken bone is one possible cause of diverticular perforation. It occurs in a small percentage of cases and is asymptomatic until the perforation happens. The site of the perforation may be in the oesophagus, stomach, and small or large intestine and peritonitis is its consequence. Elderly patients, alcoholics, patients with psychiatric diseases, fast eaters, prisoners, people attempting suicide, and other groups of patients are more likely to ingest foreign bodies. Imaging will more likely detect the foreign body when it is metallic. Kornprat et al. conclude by suggesting foreign body ingestion and perforation in the differential diagnosis of diverticular abscess, especially in elderly patients .Another report of intestinal perforation due to foreign body ingestion was done by Erichsen and Sommer. Their patient had ingested a toothpick, albeit not being aware of it. This toothpick caused inflammation in small intestinal loops and formed an abscess in the nearby abdominal wall .When dealing with patients with suspected gastrointestinal perforation, ultrasonography may be helpful. Namely, there are direct imaging findings like an increased echogenicity in the prehepatic space along with comet-tail sign. Indirect signs include fluid collections, thickening of the bowel wall, and ileus. Although the diagnostic accuracy of ultrasonography varies among different studies, some authors conclude that it can be even more sensitive than radiography in detecting gastrointestinal perforation .In a recently published study, the role of MDCT in diagnosing gastrointestinal perforation was evaluated. The authors reviewed CT examinations of patients with surgically proven gastrointestinal perforation and searched for signs of perforation. These signs included the presence of free air, the leakage of oral contrast medium, thickened intestinal wall, wall discontinuity, the formation of abscess, the presence of free fluid collections, and the presence of phlegmon. With the help of these findings, an assumption for the site of perforation was done and was correlated with the surgical findings. The site of perforation was correctly found in a percentage of patients which depended on the part of the gastrointestinal system which was perforated. The overall percentage of correct diagnosis was 82.9%. Free fluid collections and free air were the two most frequent CT findings of gastrointestinal perforation .Perforation of inflamed sigmoid diverticula is a common complication of diverticular disease which may lead to the formation of a localised abscess. In some rare cases like the one presented, this intra-abdominal inflammatory process may spread outside the abdominal cavity and cause the formation of extra-abdominal abscesses.


Case Summary. 35- and 41-year-old female patients presented with obesity and BMI of 102 and 46 kg/m2, respectively. Both patients underwent upper gastrointestinal endoscopy as part of presurgical evaluation. Multiple polyps were indentified in both patients, and biopsy was taken. Histological examination revealed tumors that were formed by nests of epithelial cells. The cells have eosinophilic cytoplasm and monomorphic nuclei, typical morphology of neuroendocrine tumors. Conclusions. (1) Upper gastrointestinal endoscopy is an important procedure for prebariatric surgery evaluation. (2) Gastric carcinoid is a rare tumor with higher incidence among obese patients.Bariatric surgery is a prevalent procedure due to the high incidence of obesity and comorbidities. Upper gastrointestinal endoscopy is one of the procedures used to evaluate the patient before surgery. However, its role is questionable. The incidental findings during endoscopy are variable including inflammatory diseases, and ulcers, and epithelial and stromal tumors. Herein a report of two obese sisters with incidental gastric carcinoids was diagnosed in prebariatric surgery endoscopy.  Laparoscopic sleeve gastrectomy is a procedure that became more prevalent as a treatment for morbid obesity due to marked improvement in surgical techniques. However, patients going for this procedure need proper preoperative assessment including medical, laboratory, and psychological evaluation . Upper gGastric carcinoid tumor is one of the rare tumors that were discovered during prebariatric UGIE. In a series of 426 patients who underwent bariatric operation, carcinoid tumor was diagnosed preoperatively in only one patient –7. In th A 35-year-old female with body mass index (BMI) of weight 40.8 kg/m² with no history of any comorbidity underwent UGIE as a workup prior to laparoscopic sleeve gastrectomy (LSG). Endoscopy revealed a gastric body polyp that measured 3.0 × 2.0 cm with central ulceration. Two smaller satellite polyps were seen near the main lesion, and biopsy was taken. Histopathological examination of all lesions was the same and revealed tumor cells that were arranged in small nests with abundant granular cytoplasm and round to oval nuclei . The tumHistopathology of liver lesion again showed preserved architecture with microsteatosis and no tumor deposit. The gastric specimen showed well-differentiated neuroendocrine tumor with lymphatic permeation. The surrounding gastric tissue shows variable degree of G-cell hyperplasia. There were tumor deposits in the serosa, and two out of nine lymph nodes showed metastasis with perinodal fat infiltration. Postoperative octreotide scan showed features in keeping with liver metastasis. MRI was performed and revealed a small lesion 1.7 × 1.4 cm in segment 6 of the liver suggestive of metastasis. Another focal lesion in segment 8 was noted, which was peripherally located that measured 2.1 × 1.9 cm probably site of previous biopsy. Based on imaging suspicion of liver metastasis, the patient underwent segmental liver resection in the cancer center, and histopathological examination proofed the presence of metastatic neuroendocrine tumor in the liver.Three months later, her elder sister, 41 year old with BMI 46 m²/kg, was subjected to UGIE as preoperative assessment for sleeve gastrectomy. The UGIE showed multiple tiny nodules and sessile polyps at the gastric body and antrum. Histopathological examination revealed tumor nests with typical morphology of well-differentiated neuroendocrine tumor that expressed chromogranin and synaptophysin. Subsequently, the patient underwent CT scan of the abdomen and chest that uncovered incidental multiple small polyps in the sigmoid colon about 6 mm in diameter. Colonoscopy was done, and the polyps were biopsied which did not reveal any significant pathological changes. At this point the patients decided to think of surgical intervention due to her psychological stress. Two months later, the patient underwent an UGIE which revealed no gross abnormality. Therefore, the patient decided to undergo sleeve gastrectomy. The specimen received demonstrated granular mucosa with no definite tumor nodules. Histological examination revealed multiple foci of microscopic neuroendocrine tumor with the largest focus that measured 0.4 cm in a background of G-cell hyperplasia. Both patients were investigated for possibility of other associated tumors and were negative.As communities go into more civilization and change thier the life style, the population pays taxes of increasing morbidities including diabetes, hypertension, and obesity. As a result, bariatric surgery becomes more prevalent especially with the presence of advanced surgical techniques. Patients going for such procedures need special evaluation. One of the investigations commonly performed for such patients is UGIE. However, the significance of such procedure is debatable and depends on the significance of the findings that can be diagnosed by such procedure. Schirmer and his colleagues  publisheGastric carcinoid tumor is a rare epithelial neoplasm of the stomach. It comprises less than 1% of the all gastric neoplasms. Gastric carcinoids are subclassified into three types: those associated with atrophic gastritis and pernicious anemia, those associated with Zollinger-Ellison syndrome with multiple endocrine neoplasia (MEN I), and sporadic cases. The tumors associated with atrophic gastritis are the most common and accounted for 70–80% of gastric carcinoids . Type 1 Carcinoid tumor is a rare tumor in general population with incidence variable between 1 and 2.5 per 100,000 population. However, an analysis of large series of cases of such tumors demonstrated increasing the incidence of carcinoid tumors in all sites particularly gastric and rectal carcinoids . There wIn conclusion, prebariatric surgery upper gastrointestinal endoscopy is an important tool to evaluate the patients as abnormal pathology can be diagnosed and will change the management plan. Carcinoid tumor is rare in general population; however, observations showed higher incidence in obese patients. There is no definite explanation to this association. But the observation of previously reported cases and animal study raised the possibility of abnormal dietary behavior, and hyperphagia has a role in the association. Therefore, we need more studies on human to see these associations.


Ovarian tumors are the least common cause of sexual precocity in girls. Mixed germ cell-sex cord-stromal tumors associated with a yolk sac tumor of the ovary are rare neoplasms, of which only a small number of well-documented cases have been described so far. Here, we report precocious puberty in a four-year-old Egyptian girl caused by a mixed germ cell-sex cord-stromal tumor associated with a yolk sac tumor of the ovary.A four-year-old Egyptian girl was referred to our pediatric endocrinology unit for evaluation of bilateral breast budding, pubic hair and vaginal bleeding. On examination, we found that her breast enlargement and pubic hair were compatible with Tanner III. A thorough workup revealed a large mass in her right ovary. Magnetic resonance imaging ofher brain showed that her pituitary gland was normal. A hormonal assay revealed high levels of estradiol, 280 to 375pmol/L; progesterone, 5.3 nmol/L; testosterone 38.9 pg/mL; and androstenedione, 4.1 ng/mL. Her basal and stimulated levels of luteinizing hormone and follicle-stimulating hormone were low. Tumor markers levels were high, with a total inhibin of 1,069U/L and an alpha-fetoprotein of 987 μg/L. Her chromosomes were normal (46XX). Our patient underwent an explorative laparotomy and a solid tumor localized to her right ovary was identified. A right salpingo-oophorectomy was performed and the histopathological diagnosis was a mixed germ cell-sex cord-stromal tumorwith a yolk sac tumor of the ovary. Postoperatively, she was started on treatment with chemotherapy. Our patient is doing well without evidence of tumor recurrence or metastasis during eight months of postoperative follow-up.Although a mixed germ cell-sex cord-stromal tumor associated with a yolk sac tumor of the ovary is a rare occurrence, it should be considered in the differential diagnosis for a prepubescent girl with an abdominal mass and precocious puberty. Precocious puberty in girls is defined by the development of sexual characters before the age of eight years. It is usually due to the premature activation of the hypothalamic-pituitary-ovarian axis, defined as central precocious puberty (CPP) . It is rA four-year-old Egyptian girl was referred to our pediatric endocrinology unit for evaluation of bilateral breast budding and growth of pubic hair of four months duration followed by two episodes of vaginal spotting, initially noticed two weeks prior to her presentation. Her previous medical history and birth history were unremarkable; there was no family history of endocrinological disorders or precocious puberty. At presentation, our patient was an alert, irritable child. Her height was 115 cm (>97th percentile) and weight 20 kg (90th percentile). Her vital signs were normal. She had normal ocular, thyroid and cardiorespiratory examinations and no lymphadenopathy. Her breast tissue was palpable and the contour of her nipples and areolae was equivalent to Tanner stage III breast development. Her pubic hair was coarse, pigmented and distributed over her labia majora, equivalent to Tanner stage III [An initial ultrasonographic study of her abdomen and pelvis revealed an enlarged uterus with prominent endometrium and 7.8 cm × 5.2 cm irregular, inhomogeneous mass in the right adnexal region lying above her uterus and crossing the midline. Her left ovary, adrenals, liver, spleen and kidneys were normal. Computed tomography imaging of her abdomen and pelvis confirmed the above mentioned findings Figure . SubsequLaboratory values of a complete blood count and thyroid function tests were within the normal limits. Serum concentrations of estradiol were in the range of 280 to 375 pmol/L ; progesterone, 5.3 nmol/L ; testosterone, 38.9 pg/mL ; androstenedione, 4.1 ng/mL ; and prolactin, 233 mIU/L . Basal levels of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) were low , with no change in response to stimulation by 100 mg of LH-releasing hormone intravenously. Regarding tumor markers, the total inhibin level was 1,069 U/L  and the alpha fetoprotein levelwas 987 μg/L  while B human chorionic gonadotropin, lactic dehydrogenase and alkaline phosphatase were negative. Her chromosomes were normal (46XX). Our patient underwent explorative laparotomy one week after her initial presentation to our department; a solid tumor localized to her right ovary was identified. A right salpingo-oophorectomy was performed. Gross examination showed that the tumor mass measured about 10 × 7 cm in diameter. The mass was capsulated and its outer surface was nodular with dilated congested blood vessels. The cut section was solid and with multiple cysts containing transparent thick material. The mass was firm in consistency with friable areas. Its color was yellow grayish with small dark areas of necrosis  every three weeks for four cycles. Eight months after surgery and chemotherapy, she was tumor free on clinical examination and radiography; her estradiol and testosterone levels had decreased to 61 pmol/L and 3.1 pg/mL, respectively. Our plan is to follow her up every three months for two years then every six months for five years.Children with precocious puberty may be stressed because of physical and hormonal changes they are too young to understand. Going through puberty early can also be difficult for a child emotionally and socially. Girls with precocious puberty may be confused or embarrassed about physical changes, such as getting their periods or having enlarged breasts well before any of their peers. Not only that, but precocious puberty could cause the child to become an object of adult sexual interest . IdiopatOur index case presented with a typical picture suggestive of idiopathic CPP with maintenance of the harmony (consonance) of normal puberty, that is, breast and pubic hair development, vaginal bleeding, growth acceleration and normal magnetic resonance imaging of the brain. But, the presence of an ovarian mass that was confirmed by computed tomography and low levels of FSH and LH pointed to an ovarian tumor as the sole cause of her precocious puberty. Her advanced bone age and accelerated height were seen because of tumor-derived estradiol. Advanced bone age, especially if it has progressed beyond the height age, serves as a nonspecific biomarker of abnormal sex steroid production .The endocrine effects of the tumor on our patient were probably dependent upon the stromal components and proportions of different elements. Tumors containing both Sertoli and Leydig cells have variable effects, depending partly upon the proportion of these tumor elements and the degree of histologic differentiation. Signs of androgen excess or overt virilization occur in about half of patients with Sertoli-Leydig cell tumors. As with the pure Leydig cell tumors, estrogenic manifestations also occur. Patients with Sertoli-Leydig cell tumors who have defeminizing or virilizing clinical signs invariably have elevated blood testosterone levels . In addiAn MGCCST with a yolk sac tumor of the ovary should be differentiated clinically and pathologically from a gonadoblastoma, described by Talerman in 1972 ,18. An MAlthough in most of girls with precocious puberty, the etiology is idiopathic, MGCCST with a yolk sac tumor of the ovary should be considered in the differential diagnosis for a prepubescent girl with an abdominal mass.Written informed consent was obtained from the patient’s parents 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.CPP, central precocious puberty; FSH, follicle-stimulating hormone; LH, luteinizing hormone; MGCCST, mixed germ cell-sex cord-stromal tumor.The authors declare that they have no competing interests.KA carried out the patient diagnosis, investigation, follow-up, management and writing of the manuscript. DS carried out the histopathological diagnosis and writing of the manuscript. HS carried out the patient diagnosis, follow-up, management and writing of the manuscript. HA carried out the patient diagnosis and follow-up. MA carried out the excision of the tumor. All authors read and approved the final manuscript.


A 50-year-old male patient presented with erectile failure and loss of libido. In the physical examination, there were stone-hard indurations in his bilateral testes. The ultrasonographic study demonstrated multiple hypoechoic areas in the testes and normal epididymis. Since the lesion was presumed as malignancy, bilateral inguinal exploration was performed and intraoperative frozen biopsies were studied and diagnosed as inflammatory process. Nevertheless, we decided to perform left orchiectomy to a deeper histopathologic analysis which revealed granulomatous orchitis, mastocytosis, and severe depletion of Leydig cells at the testicular interstitium. Differential diagnosis between testicular tumor and granulomatous orchitis is very difficult in any examination except by histological findings. Bilateral cases of this pathology are relatively rare, but it is necessary to distinguish them from the testicular tumor before surgical intervention to avoid an unnecessary orchiectomy. Inflammatory pathology is rare in the testis, and usually, when it is detected, the involvement of the Leydig cells in the process is so infrequent . Most ofImagenological analysis such as ultrasonography and colour doppler sonography is not always conclusively in the differential diagnosis between tumoral and non tumoral testicular lesions. Since these lesions are presumed as malignancy, inguinal exploration of the testis is necessary.α-fetoprotein and β-human chorionic gonadotrophin were normal, but levels of testicular axis hormones were altered .A 50-year-old man presented with erectile failure and loss of libido. There are not antecedents of autoimmune or rheumatoid illness. In the physical examination, there were stone-hard indurations in his bilateral testes. The ultrasonographic study demonstrated multiple hypoechoic areas in the testes and normal epididymis . The valSince the lesion was presumed as malignancy, bilateral inguinal exploration was performed and intraoperative frozen biopsies were studied and diagnosed as inflammatory process. The later histopathology from the biopsy of the left testis revealed histoarchitecture altered with seminiferous tubules hyalinized and atrophic. A severe stromal fibrosis was seen with the presence of large infiltrates of lymphocytes, macrophages, and eosinophils. Also, we observed several giant cell clusters and a severe Leydig cell depletion at the interstitium Figures . By histThe histopathologic diagnosis was bilateral granulomatous orchitis.We resolved to conserve the right testis in order to preserve the endocrinologic testicular function. Further controls were normal. The patient was under replacement hormonal therapy with testosterone enanthate, and he recovery sexual function and libido.The idiopathic granulomatous orchitis is an entity of unknown etiology, clinically or ultrasonographically not distinguishable from testicular tumors. Usually, the differential diagnosis is done by the histological findings.Bilateral cases of this entity are relatively rare, and, in our knowledge, it is the first report describing this entity as a cause of primary hypogonadism and Leydig cell loss.Testicular inflammatory processes were described in animal models of experimental autoimmune orchitis (EAO)  and in oIn the human testis, inflammatory cells have been found in some viral conditions as mumps orchitis  and in bLymphocytes, macrophages, and mast cell infiltrates have been found in association with degenerative seminiferous tubules, and this process is explained as an alteration of the blood testis barrier that permits the passage of antigens from the adluminal tubular compartment to the testicular interstitium , 8.Leydig cell loss is infrequent in the testicular inflammation, and it has not been described in models of EAO. The role of mast cells in normal and pathological testis is not fully understood; however, the number of these cells increases in EAO  and in tWe conclude that bilateral granulomatous orchitis is a rare pathology, but it should be considered in the differential diagnosis of diffuse testicular hypo echoic lesions. Histopathological findings suggest that it could be an autoimmune condition, and inflammatory cells, mainly macrophages and mast cells, could play an important role triggering germ cell damage and also being involved in the induction of fibrosis of the wall of seminiferous tubules, as it has been suggested for other testicular pathologies .A correct preoperative diagnosis is necessary to preserve the remainder endocrine function of the testis.


Intermittent pelvic pain caused by ovarian cysts in adolescence may be due to torsion or partial torsion of the ovary. We present a case of 18-year old adolescent with symptomatic left ovarian torsion with calcifications demonstrated by pelvic MRI and ultrasonography prior to surgery. The pathologic investigation demonstrated dystrophic calcifications. We speculated that the pattern of the intermittent pain in the story of the patient and the dystrophic calcifications in pathologic investigation which is thought that it might have been potentially developed as a result of chronic hypoxia due to intermittent partial torsions over a period of two years. Adnexal torsion should always be considered in the differential diagnosis of adnexal mass in an adolescent. We presented a chronic adnexal torsion case with calcifications. The pattern of the intermittent pain in the story of the patient and the dystrophic calcifications in pathologic investigation which is thought that it might have been potentially developed as a result of chronic hypoxia due to intermittent partial torsions over a period of two years.An 18-year-old girl presented to the outpatient clinic with an intermittent pelvic pain for the last two years with worsening pattern over the last 6-weeks. Her past medical history was unremarkable. Family history was remarkable for breast cancer in her mother. Physical examination revealed that there was a 10 cm soft mass in front of the uterus.3, serum hematocrit level of 26%, and serum Ca-125 level of 52.2 U/mL. Serum LDH, Alpha fetoprotein, CA 19-9, and beta-HCG levels were within normal limits. Pelvic MRI showed diffuse hypointensity of necrotic left ovary with small hyperintense peripheral area corresponding to hemorrhage and calcification at the periphery of left ovary as a hypointense focus. Pelvic ultrasonography with colour Doppler showed a 63∗56∗66 mm solid-cystic mass with no arterial perfusion.Laboratory findings were as follows: white blood cell count of 7.6 × 10In the presence of the possibility of torsion, she was immediately taken to the operating room. On surgical exploration, the right ovary was seen in front of the uterus as a biloculated cystic mass (8 × 9 cm in diameter). Torsioned left adnexa (9.5 × 7 cm in diameter) was obliterated to Douglas pouch completely with adhesions. Cystectomy was made in the right ovary. The left adnexa were mobilized with blunt dissection. Both left tuba and ovary were seen three times tostioned in the presence of a cystic mass. Both of the tube and ovary were firm and full of coagulum. Total salphingo-oopherectomy was performed because of the diffuse tissue necrosis. The result of pathology from the right ovary was follicular cysts and corpus luteum, and from the left salphingo-oopherectomy material, was diffuse hemorrhagic necrosis and dystrophic calcifications in both ovary and tube .Clinical approach to the adnexal mass in adolescence differs from the adulthood counterparts in terms of the incidence of tumor types and the relative importance of the reproductive and endocrinological properties. The majority of adnexal masses in adolescents are asymptomatic and pathologically benign and often diagnosed as a result of complications like rupture or torsion. Adnexal torsion is reported to be the fifth most common gynecologic emergency condition encountered, with a prevalence of 2.7% . AlthougProper diagnosis is possible by using appropriate imaging modalities. Pelvic ultrasonography with color Doppler flow imaging is an excellent imaging technique especially in the evaluation of patients with acute pelvic pain .Underlying ovarian tissue necrosis and type of surgery  in these patients can be determined in part by the demonstration of calcifications by imaging modalities like ultrasonography and MRI .We have limited knowledge about chronic torsion or intermittent partial torsions in pediatric and adolescent population. It has been demonstrated that partial torsion of the adnexa or the early phase of the torsion can cause massive ovarian edema .Calcifications in cases with ovarian torsion have been rarely reported on either imaging modalities and/or pathologic examinations . There a


Dear SirIntussusception is referred to as recurrent when after being spontaneously reduced it is re-diagnosed clinically and on ultrasound, later confirmed at operation; or it is a post-operative intussusception in a patient where it was reduced at an open surgery or non-operatively either with hydrostatic or pneumatic method , 2, 3.Most of the recurrent intussusceptions present within 6 months of initial episode and have been observed with almost all the treatment modalities. These are frequently reported after non-operative reduction (hydrostatic/pneumatic), whereas their incidence is negligible after operative management. This is especially true where the resection of the involved part of intestine is performed. The recurrence may occur once to as many as 8 times or more , 2, 3.There is ongoing debate as to the management of recurrence. The recommended management is non-operative reduction, however, a significant number of recurrent intussusceptions do have pathological leads point (PLP); therefore operative management is required in these cases . The purCase 1: A 3-month-old male infant presented with abdominal pain, two episodes of bilious vomiting, irritability, and passage of currant jelly stool for two days. There was a preceding history of diarrhea. Clinically a sausage shaped mass was present in the lower abdomen. Ultrasound of the abdomen confirmed it as intussusception. At operation, no intussusception was found and appeared to be spontaneously reduced as indicated by hyperemia of a portion of distal ileum and collapse of the ileum distal to edematous segment.On the third post-operative day the infant again developed similar features with toxic features. Patient underwent re-operation and resection of gangrenous ileum with primary end to end ileo-ileal anastomosis performed for ileo-ileal intussusception. This time, thePost-operative course remained uneventful. There was no recurrence during a follow up of 15 months.Case 2: A 6-m-old male infant presented with abdominal pain, vomiting, and passage of currant jelly stool. The sausage shaped mass was palpable in the right upper quadrant. Ultrasound abdomen also revealed a positive pseudo-kidney sign. At operation ileo-colic intussusception found that was reduced manually. No PLP was detected. On 4th post operative day the clinical features of intestinal obstruction appeared. A diagnosis of recurrent intussusceptions was made and hydrostatic reduction with saline enema under ultrasound guidance was attempted. The intussusception was successfully reduced and the post-operative course remained uneventful. The patient had no further recurrence and doing well at 18 months of follow up.Case 3: A 7-year-old boy presented with colicky abdominal pain, bilious vomiting, and bleeding per rectum for 3 days. There was no history of any preceding illness. Ultrasound done a day earlier suggested intussusception as suspected on clinical examination which revealed a sausage shaped mass in the right iliac and lumbar regions. The mass was spontaneously reduced within an hour of admission to our hospital. The symptoms again developed on the next day with the mass detectable clinically and demonstrated on ultrasound. The patient was planned for exploratory laparotomy with a strong suspicion of PLP. On operation table under general anesthesia the mass was not palpable. It was decided to proceed with laparotomy. Operative signs of spontaneous reduction of ileo-colic intussusception were found but no PLP demonstrated. Appendectomy was added with a view of probable PLP. The post operative course was uneventful. The patient remained asymptomatic at 3 months follow up.The incidence of recurrent intussusceptions is 8-15% in most of the big series. The recurrence is higher with hydrostatic and pneumatic reduction (10-15%) and lower with operative reduction (1-3%). Post operative adhesions play a vital role in preventing recurrence. The recurrence is almost nil after resection of the involved portion of intestine which indicates that the involved part of intestine, even in absence of an obvious PLP, has a key role in development of recurrence [Niramis et al have reported that about 70% of patients with recurrent intussusceptions were infants with a maximum incidence within 6 months of the initial episode. Frequently recurrent intussusceptions may have PLP as a cause of recurrence. Previously we have reported a case of recurrent colocolic intussusception in a 10 year old boy having inflammatory fibroid polyp as a PLP. In contrary to that, Niramis et al found maximum of 5 recurrences in two of their patients but neither of them had PLP when explored for it .The management of recurrent intussusception is somewhat tricky. One cannot exactly anticipate the future episodes of recurrences. Many authors are in favor of operative interventions in case of more than one episodes of recurrence in patients older than 2 years. As the recurrence is also reported after operative reduction therefore some authors recommend ileocolopexy for prevention of recurrence however others do not recommend it. Some authors suggested resection of involved portion of intestine in case of multiple recurrences , 5,6.In summary, recurrent intussusceptions may pose difficulties as to the management. The selection of the treatment modality should be individualized as per case requirement.Source of Support: NilConflict of Interest: None declared


Paragangliomas are extra-adrenal tumors of the autonomic nervous system and may be found within the skull base, neck, mediastinum and periaortic region. Paragangliomas of the urinary bladder are rare, and non-functioning bladder paraganglioma is even rarer and not easily recognized. Histological examination is often key in leading to a definitive diagnosis. The current report presents a case of a 28-year-old female with urinary bladder paraganglioma. The patient presented with no classical signs and symptoms, and these were only appreciated following histological examination of a transurethral resection specimen that elucidated the correct diagnosis. In the present report, the clinical features, diagnosis, management and pathological observations of paraganglioma of the urinary bladder are discussed. Paraganglioma is a neoplasm that develops from the chromaffin tissue of the sympathetic nervous system situated outside the adrenal medulla . It is aA neoplasm of the urinary bladder was identified during a routine examination of a 28-year-old female patient, three days later the patient was admitted to the Peking University Shenzhen Hospital . The patient had no abdominal symptoms, had been in good health, with no previous medical problems, and had no specific family past medical or drug history. The patient did not complain of headache and had no history of hypertension. Routine hematological examination and biochemical tests were within normal limits and physical examination showed no evidence of hypertensive disease. Ultrasonography revealed an abnormal mass on the right lateral wall of the urinary bladder and computed tomography (CT) of the abdomen showed a solitary tumor protruding into the bladder. This was confirmed by cystoscopy demonstrating a protruding tumor on the right wall of the bladder with normal mucosa, measuring 2.0×2.0 cm . No signOn histopathological evaluation, the tumor cells were arranged in a nested pattern . The hisPheochromocytoma is a rare tumor with an estimated 800 cases diagnosed yearly in the USA, of which ~10% are extra-adrenal or paragangliomas . ParaganPreoperative location and qualitative analysis are extremely important in confirming the diagnosis. B ultrasound, CT and magnetic resonance imaging may be of great use in localizing the tumor –9. UltraFor the majority of asymptomatic bladder paragangliomas, a definitive diagnosis may be reached only by histology. The tumors show histological features similar to adrenal pheochromocytomas and the cells usually grow in a characteristic nested Zellballen pattern. Immunohistochemical staining is required for a definitive diagnosis. Chromogranin, synaptophysin and NSE may aid the identification of neural tissue and neuroendocrine cells . A positThe majority of paragangliomas are sporadic in nature, but ~10% of these tumors may be associated with genetic disorders, such as familial paraganglioma, neurofibromatosis type 1, von Hippel-Lindau, Carney triad and multiple endocrine neoplasia type 2 . TherefoThe most effective management is surgical resection, including transurethral resection and partial or total cystectomy. The perioperative preparation and treatment may not be simplified, particularly for patients who exhibit characteristic paroxysmal hypertension during micturition. It is necessary to stabilize hypertension prior to surgery, using α-blocking agents (phenoxybenzamine) or calcium channel blockers for two weeks to inhibit the release of catecholamine and expand the blood volume, which is similar to the management for adrenal pheochromocytoma. Occasionally, it is difficult to determine a definitive preoperative diagnosis, resulting in insufficient preparation, which complicates the transurethral resection since unexpected intraoperative hypertensive crisis and bleeding may occur . With thRegular follow-up is necessary to detect late recurrences . It mustNon-functioning bladder paraganglioma is easily misdiagnosed. Transurethral resection for bladder paraganglioma may be a treatment of choice, offering several advantages, including reduced invasion, rapid recovery and early discharge from the hospital, but the optimal management mode remains uncertain. A definitive diagnosis may be reached only by histology and no histological criteria have been established to distinguish between benign and cancerous tumors. Long-term annual follow-up is recommended in all paragangliomas.


Primary pulmonary mucinous cystadenocarcinoma (PMCAC) is an extremely rare cystic neoplasm. A case of a 56-year-old male with a cystic lesion of the right lower lobe is described. Preoperative fine needle aspiration cytology and bronchoscopy were inconclusive. The patient underwent a formal right lower lobectomy and mediastinal lymph node dissection. Diagnosis was established intraoperatively. The biological behavior of primary PMCAC is unknown. Therefore, careful long-term follow-up is considered necessary because of lack of experience globally. Primary pulmonary mucinous cystadenocarcinoma (primary PMCAC) belongs to a group of cystic neoplasms characterized by extracellular mucin secretion. These mucinous neoplasms are histologically similar to well-recognized mucinous tumors of the ovary, breast, and pancreas . AlthougA 56-year-old male presented with a complaint of chest pain. His medical record was clear, except that he was a heavy smoker (60 cigarettes/day for 38 years). Clinical examination revealed diminished breath sounds in the right hemithorax. A chest X-ray showed a large mass in the right lung . ComputeBronchoscopy was negative for endobronchial growth, and CT-guided FNA cytology failed to establish a tissue diagnosis. The patient underwent a right posterolateral thoracotomy, and a right lower lobectomy was performed. Frozen sections of the specimen revealed a cystic mucinous mass with fibrous walls containing abundant mucinous material. Cells suspicious for malignancy were detected inside the mucin. The operation, therefore, was completed with a radical mediastinal lymph node dissection. The patient was discharged on the 11th postoperative day. No complications were observed. The excised lobe contained a cystic tumor filled with mucin, 9.3 cm in greater diameter. Microscopic examination revealed a well-differentiated mucinous cystadenocarcinoma. The tumor consisted of a large cystic space filled with mucin, and many smaller cysts along its wall . The cysThe patient is currently been followed up closely. No signs of local or distal recurrence were observed during 6-months period.Primary PMCAC is a very rare entity. It was first described in 1978 by Sambrook Gowar . It is cRadiologically, some authors mention CT characteristics which can raise suspicion for the diagnosis. These include a uniform low attenuation cystic lesion with focal thickening and enhancement of the walls and septa . CT imagThe normal bronchial mucus is 95–98% water and 2–5% solids, and the majority of the solid component is formed by glycoproteins. Due to its high water content, mucus has CT attenuation and Magnetic Resonance Imaging (MRI) signal similar to those of water and do not enhance after injection of intravenous contrast medium; however, a chronic entrapped mucus collection can undergo a gradual transformation. The increased production of mucus protein is accompanied by a gradual reabsorption of water with a decrease in free water content and an increase in protein concentration. Protein concentration influences the T1 relaxation time, and an elevation in protein concentration may cause impressive shortening of the T1 relaxation time. Mucus with a protein level >9,000 mg/dL has high signal intensity on T1-weighted images. The detection of mucus within a mediastinal or pulmonary lesion is important because it permits shortening of the list of differential diagnoses [It has been well known that most malignant tumors have a higher rate of glucose metabolism than normal tissue. However, 18Fluoro-2-deoxy-D-glucose (FDG) uptake has a wide variation in tumor glucose metabolic rate, depending on the histologic type and aggressiveness of the tumor. Because FDG behaves as an analog of glucose, its distribution closely follows that of glucose-metabolizing cells and organs. Most malignant tumors have a higher rate of glucose metabolism than normal tissue, therefore, exhibit a higher FDG uptake than background tissue. Using FDG Positron Emission Tomography (PET) a positive correlation between tumor FDG uptake and cellularity can be proved, but a negative correlation with the amount of mucin is also expected. In case of PMCAC hypocellular lesion with abundant mucin, it is possible that little FDG uptake on integrated PET/CT be shown . In addiThe histopathological differential diagnosis of primary PMCAC includes primary lesions of the lung and metastatic tumors from other organs, especially the ovary, breast, and pancreas . Well-diNonneoplastic lesions that enter the differential diagnosis include developmental bronchogenic cysts and congenital adenomatoid malformations. Both can have foci of mucinous epithelium but more commonly are lined by ciliated columnar epithelium, in contrast with the purely mucinous epithelium of mucinous cystic tumors. Developmental bronchogenic cysts are extrapulmonary, located in the midline, and have a fibromuscular wall containing cartilage and seromucinous glands . Preoperative diagnosis is very difficult to establish. Both FNA cytology and transbronchial lung biopsy seem inadequate, mainly because the malignant cells are floating inside the mucin. Only in two cases were the authors able to reach preoperative diagnosis by using FNA cytology , 3. In oThe biological behavior of primary PMCAC is generally unknown. Although it is considered a malignant tumor, it seems not to have malignant potential of high degree. In the only case where recurrence occurred, margin negative wedge resection was performed at first. Due to the small number of reported cases in the literature, the tumor's developing pattern is not yet completely clarified. It seems, however, that local extension is more likely than distal metastases. Therefore, formal anatomic lobectomy for early stage tumors, in those patients who can physiologically withstand it, according to GN Mann et al, is probably the correct surgical approach . The rolWritten informed consent was obtained from the patient for publication of this paper and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare no conflict of interests.


Gallstone ileus is a rare cause of bowel obstruction and results from the passage of gallstones into the bowel.We present the case of an 83-year-old Caucasian woman who had three episodes of gallstone ileus, each of which was managed with simple enterotomy. This sequence is one of the first reported in the medical literature and may be seen to challenge the traditional surgical approach of enterotomy alone.The available evidence comparing enterotomy alone with combined enterotomy, cholecystectomy, and fistula closure in the management of gallstone ileus is reviewed. Neither approach is clearly identified as superior, but available series suggest that simple enterotomy may be safer than a combined approach and does not result in a higher rate of recurrent biliary disease. Gallstone ileus occurs when gallstones migrate from the gallbladder to the bowel through a cholecysto-enteric fistula, causing a mechanical obstruction. Investigation of suspected cases with abdominal computed tomography (CT) will generally show gallstones in the bowel lumen with proximal dilatation. Patients are often frail and elderly, and so surgical treatment is generally limited to stone retrieval through an incision in the bowel wall known as an enterotomy . Owing tAn 83-year-old Caucasian woman with rheumatoid arthritis and a large hiatus hernia was admitted with a one-day history of severe abdominal pain and vomiting. She was tachycardic, tachypnoeic, and pyrexial. Abdominal examination revealed tenderness and peritonism to her right iliac fossa. Her bowel sounds were diminished. Serology revealed a white blood cell count of 16.1g/dL, and plain abdominal radiographs revealed no obvious perforation or obstruction. A differential diagnosis of appendicitis and cecal carcinoma was considered, and the decision to operate was made. At laparotomy through a lower midline incision, palpation of the small bowel revealed that a large impacted gallstone in the mid-ileum was causing patchy mesenteric necrosis. There was free fluid in the right iliac fossa but no perforation. A 12cm length of ileum was resected, and a primary stapled anastomosis was performed. The gallbladder was found to be packed with stones. No fistula was identified, but the dissection was limited by dense fibrosis.Clostridium difficile infection, but our patient returned home to independent living four weeks after the second procedure.After initial signs of recovery, our patient developed worsening abdominal pain on the fourth post-operative day, and abdominal CT was undertaken Figure . The scaC. difficile infection in addition to a prolonged period of respiratory failure managed with a tracheostomy in critical care. She was discharged to her own home six weeks after surgery and made a good recovery. Two months after discharge, magnetic resonance cholangiopancreatography did not visualize any residual gallbladder stones.Twelve months later, she presented with a further episode of central abdominal pain associated with vomiting and distension. An ultrasound scan revealed a contracted gallbladder with a 10mm common bile duct, and intravenous antibiotics were commenced for a presumed cholecystitis. She failed to settle, and a CT scan revealed free intraperitoneal gas and fluid, a persistent cholecysto-duodenal fistula, and a further gallstone obstructing the small bowel. This gallstone corresponded in size and shape to that seen in the gallbladder 12 months earlier (Figure Gallstone ileus accounts for around 25% of mechanical small bowel obstruction in patients over 65 years of age . A histoOperative management is challenging. Simple enterotomy risks further stones and the potential complications of cholangitis, pancreatitis, and recurrent gallstone ileus. Conversely, cholecystectomy and fistula repair within dense adhesions risk duodenal injury and biliary leak. Both options have an associated mortality in the population in question.In the case described, it is unclear whether the stone removed at the second laparotomy was in the gallbladder or the bowel lumen at the time of the first operation. Twelve months later, the CT images suggest that a further stone had migrated into the bowel. Our experience demonstrates the importance of inspecting the entirety of the small bowel at the time of the initial laparotomy and illustrates that late recurrence of gallstone ileus, albeit rare, can occur .To the best of our knowledge, this is only the third reported case of twice recurrent gallstone ileus ,7. The rTo date, the largest review of reported cases of gallstone ileus, which consisted of 1001 cases, was published by Reisner and Cohen in 1994 [et al. [In their combined series, Reisner and Cohen  publisheet al. . There aet al. ,9.These studies all describe small, retrospective, non-randomized series without predetermined follow-up. Given the rarity of the presentation, the inconsistency of pre-operative diagnosis, and the frailty of many patients, formal randomized analysis is unlikely to provide a more definitive set of answers in the future.Gallstone ileus is a surgical emergency affecting a high-risk population. Although there remains no strong evidence in favor of enterotomy alone or one-stage repair, the available data suggest that enterotomy is safe and not associated with increased long-term biliary complications.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.CT: Computed tomography.The authors declare that they have no competing interests.DB collated patient data relevant to the case report. RJ reviewed the medical history, wrote the manuscript, and performed the literature review. RH reviewed the radiology and contributed to the writing of the manuscript. DC contributed to the writing of the manuscript. All authors read and approved the final manuscript.


Ectopic livers are infrequently reported in the literature. The reported size for ectopic livers range from a few millimeters up to several centimeters. They are often clinically silent and incidentally discovered during imaging of the hepatobiliary tract, regional surgical procedures or autopsy. They are predestined for benign liver diseases otherwise observed in normal livers like fatty change or develop malignancies such as hepatocellular carcinoma, in a manner analogous to the parent orthotopic liver. The presence of abnormal alpha 1-antitrypsin retention in an ectopic liver has, to our knowledge, not been reported in the literature. Hereby, we present the first reported case featuring alpha 1-antitrypsin retention in an ectopic liver attached to the fundus of the gallbladder and present the clinical, radiological and pathological findings in a caucasian woman undergoing cholecystectomy for acute cholecystitis. Special liver stains showed an alpha 1-antitrypsin retention which was confirmed immunohistochemically. Although ectopic livers are rare and usually an incidental finding, the radiologist and the surgeon should take this into the differential diagnosis of a mass attached to the gall bladder. A secondary disease should be considered by the pathologist in such a specimen and alpha 1-antitrypsin retention should be ruled out by special liver stains. Finally, such a finding should prompt the managing clinician to exclude systemic alpha-1 antitrypsin deficiency in the patient through further appropriate tests. Ectopic liver is a rare finding in routine clinical imaging, during surgery or as a pathological specimen. The first case of an ectopic liver attached to the gallbladder was reported more than fifty years ago by Eisert and colleagues . OccasioA 91 year old previously healthy female patient underwent laparoscopic cholecystectomy four weeks after presenting with symptoms suggestive of acute cholecystitis. A preoperative abdominal computer tomography (CT) scan showed gall stones and a small mass of approximately 1.5 cm size attached to the fundus of the gall bladder Figure  using thEctopic livers attached to the gallbladder are very rare in clinical practice, and have been estimated to occur in 0.23 to 0.47% of excised gallbladders. It was first described by Eisert in 1940 with an incidence of 0.23%; in another more recent laparoscopic case series, an incidence of 0.47% was noted [The increasing incidence of ectopic liver excisions is most likely the result of more thorough clinical and pathological work-up -6.Several pathogenetic mechanisms for the development of liver heterotopias have been postulated. During human embryogenesis, there is a very proximate relationship between the developing liver, the duodenum and the gall bladder. A migration or displacement of a cranial part of the liver bud during embryogenesis seems the most likely explanation for ectopic livers and would explain their localization within structures associated with organogenesis of the liver, including the gall bladder, the gastrohepatic ligament, the adrenal glands, pancreas and the splenic capsule. Ectopic livers are rarely found in an intrathoracic location, such as attached to the surface of the heart or the pericardium. Others hypotheses of origin include the possibility that they occur as developmental anomalies due to atrophy or regression of the connecting tissue to the parent liver ,10-17. Ibona fide liver tissue or may show identical pathological changes seen as normal orthotopic liver. Fatty infiltration, alcoholic cirrhosis, haemosiderosis and primary hepatocellular carcinoma have been reported in ectopic liver, along with secondary malignancy [The surgeon and the radiologist would be advised to consider the possibility of ectopic liver in addition to edema, inflammation, cholesterol polyps, adenomyomatosis, adenoma and primary or secondary malignancies in the differential diagnosis of a patient presenting with thickening of the gall bladder wall or a suspicious mass lesion ,19. On alignancy ,8,20,21.Clinically, alpha 1-antitrypsin deficiency usually presents with dyspnea that may resemble an asthmatic exacerbation, which does not respond to standard anti asthmatic treatment. Patients often develop severe emphysema by their early thirties and forties, in the absence of a history of smoking. Liver impairment progressing to liver failure or cirrhosis has been attributed to the toxic accumulation of synthesized mutant alpha 1-antitrypsin peptide within the cytoplasm of the hepatocytes. The disease may be frequently under recognized and underdiagnosed, as many patients may be clinically silent . TypicalIn this case, the patient did not have any history of liver or lung disease, nor did she display any symptoms related to alpha 1-antitrypsin deficiency. The patient declined further confimatory blood tests for alpha 1-antitrypsin in view of her advanced age and this matter was not further pursued by the managing clinician.Nonetheless, we strongly believe that alpha 1-antitrypsin accumulation diagnosed histologically with PAS/D-PAS and confirmed by immunohistochemical staining for alpha 1 anti-trypsin should prompt the treating clinician to perform a work-up for alpha 1-antitrypsin deficiency. In fact, a case of clinically silent alpha 1-antitrypsin deficiency, diagnosed in an orthotopic liver has been described in a previous case report .However, ectopic livers are prone to all kinds of pathologic changes. This could account for the possibility that the observed intracellular alpha-1 antitrypsin retention might be unrelated to a genuine alpha 1 antitrypsin deficiency genotype and represent only a local phenomen.The possibility of ectopic liver has to be considered in the differential diagnosis of a gall bladder wall thickening or mass. A thorough examination of ectopic liver tissue by the pathologist may uncover clues to a systemic disease, such alpha 1 antitrypsin deficiency.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of Diagnostic Pathology.The authors declare that they have no competing interests.All authors contributed to this article. All authors read and approved the final manuscript.


Papillary intralymphatic angioendothelioma (PILA) or Dabska tumor is extremely rare, and often affects the skin and subcutaneous tissues of children. Since its first description by Dabska, only a few intraosseous cases have been described in the literature and none of them presents with multifocal osteolytic lesion of bones. We present a case of unusual multifocal intraosseous PILA in facial bones occurring in a 1 year 3 month old male child. Computed tomography (CT) scan revealed multifocal osteolytic lesions were located at the left zygoma, left orbital bone and right maxillary. Histologically, the lesions were ill-defined and composed of multiple delicate interconnecting vascular channels with papillae formation which projected into the lumen lined by atypical plumped endothelial cells. The vascular channels were also lined by plump cuboidal endothelial cells with focal hobnailed or “match-head” appearance. In some areas, endothelial cells formed solid-appearing aggregates with vessel lumens. By immunohistochemistry, the tumor cells were positive for CD31, CD34 and D2-40 at varying intensity. A final diagnosis of intraosseous PILA was made. To the best of our knowledge, this case is the first case of primary multifocal osseous PILA.http://www.diagnosticpathology.diagnomx.eu/vs/1919488629100787The virtual slide(s) for this article can be found here:  Papillary intralymphatic angioendothelioma (PILA) or Dabska tumor is a locally aggressive, rarely metastasizing vascular lesion characterized by lymphatic- or vascular-like channels and papillary endothelial proliferation. The tumor is extremely rare, and often affects the skin and subcutaneous tissues of children . Since iA 1 year 3 month old Chinese male child was referred to our pediatric department for pain and swelling on his left side of face for past 2 weeks. In the past two weeks, the baby was suffering from a gradually severe soft tissues swelling on his left face. Two days before admission to our hospital, the pain and tenderness of left face became worse. As a result, the patient was referred to our hospital for examination and treatment. There was no history of any trauma to head and neck. Physical examination showed the patient had a mild soft tissues edema on his left upper face and severe pain was elicited upon pressure. There was no fever, weight loss and no palpable lymphadenopathy or organomegaly. The laboratory results, including blood count, differential, liver and renal function, were within the normal range. A computed tomography (CT) scan of the head revealed multifocal osteolytic lesions in the facial bones, including left zygomatic bone (measuring 1.5 × 1.0 × 1.0 cm in size), left orbital bone (measuring 0.5 cm in diameter) and right maxillary bone (measuring 1.0 cm in diameter). The most of left zygoma was observed to be destroyed and associated soft tissue mass was also noted . The antibodies used in this study included a broad panel of antibodies against cytokeratin (AE1/AE3), epithelial membrane antigen (EMA), vimentin, desmin, smooth muscle actin (SMA), CD31, CD34, D2-40, CD1a, CD68, S100 protein, Langerin, and Ki-67. The antibodies were obtained from Dako Cytomation  and Santa Cruz Biotechnology .Under microscopic examination, both zygomatic and maxillary lesion showed an ill-defined neoplasm within the osseous fragments. The lesions were composed of multiple delicate interconnecting vascular channels with papillae formation which projected into the lumen lined by atypical plumped endothelial cells. Some of those papillae contained hyalinized core. The vascular channels were also lined by plump cuboidal endothelial cells with focal hobnailed or “match-head” appearance. In some areas, endothelial cells formed solid-appearing aggregates with vessel lumens. A variable number of lymphocytes are seen within and around the vascular channels. Mitotic figures were rare and necrosis was not observed in the lesions Figure A-C.Immunohistochemistry stains done on these lesions were positivity for CD34 and CD31. Staining for CD31 was stronger than that for CD34. Endothelial cells of lumens expressed CD31, CD34 and D2-40, but the intraluminal tumor cells were negative for D2-40 (F). These lesions did not express CD1a, S-100 protein, cytokeratin (AE1/AE3), EMA, langerin, CD68 and SMA. The Ki-67 labeling index of the lesions was low, accounting for 2% of the tumor  revised tumor description to “rarely metastasizing lymphatic vascular neoplasm”, which further supports lymphatic origin or differentiation of this tumor [Papillary intralymphatic angioendothelioma (PILA), originally termed as endovascular papillary angioendothelioma (EPA), or Dabska tumor, was first presented in 1969 by Maria Dabska where she presented cases occurring in 6 children. In her originally reported cases, EPA was regarded as malignant and termed “malignant endovascular papillary angioendothelioma” because two of six patients had lymph node metastases . Since tSince its first description by Dabska et al. only approximately 33, predominantly pediatric cases have been described in the literature. Of these 19 were children and 14 were adults without sex predilection. It appears that PILA may affect a wider age range than originally described by Dabska. The age range for all reported cases is from birth to 83 years. PILA does not show a preference for a specific anatomic site, but most of tumors involve the dermis and subcutaneous tissues of extremities . The tumor has also been described in other deeper locations, including spleen , tongue Since cases of intraosseous PILA are so rare, the diagnosis should be only made by strict histological and clinical manifestation. According to the WHO criteria, PILA appears similar to cavernous lymphangiomas. The cuboidal or hobnail endothelial cells lining the vascular structures are characterized by a high nuclear cytoplasmic ratio and an apically placed nucleus that produces a surface bulge, accounting for the term "hobnail" or "matchstick". Immunohistochemically, vascular endothelial markers such as Von-Willebrand factor, CD34, CD31 and Fli-1 are commonly positive in most of tumors at varying intensities in each case, which help identify and diagnose the tumor as a type of vascular neoplasms. Of these markers, CD31 has been the best marker for this tumor because of its high sensitivity and specificity for vascular endothelial cells. CD31 usually has more immunohistochemical intensity than CD34 . MoreoveIntraosseous PILA usually show osteolysis in radiological examination , it is often misdiagnosed as eosinophilic granuloma of bone preoperatively. However, eosinophilic granuloma of bone shows clonal neoplastic proliferation of Langerhans cells that express CD1a, S-100 and Langerin. The absence of those specific Langerhans cell markers will rule out the diagnosis of eosinophilic granuloma. Importantly, two particular types of vascular lesions, retiform hemangioendothelioma and papillary endothelial hyperplasia (Masson's lesion), may also be confused with the PILA, because of presence of “hobnail” cells or irregular papillary projections in vascular channels. Retiform hemangioendothelioma is a locally aggressive vascular tumor and characterized by distinctive arborizing blood vessels lined by endothelial cell with “hobnail” morphology. The elongated, branched vessels impart a pattern reminiscent of rete testis. That histological appearance is not consistent to the PILA. Papillary endothelial hyperplasia, also known as Masson's lesion, is a reactive endothelial proliferation secondary to vascular thrombosis. The lesion shows branching papillary projections, many of which are free-floating in the vascular lumens. Thrombotic material is usually present in the vascular lumens. Moreover, these histologically similar vascular lesions do not express D2-40 and VEGFR3, which are specific markers for PILA. In addition, epithelioid hemangioendothelioma of bone has recently been reported in literature , and lymAlthough PILA was identified initially as a “low-grade angiosarcoma”, following-up in more recent series demonstrated no local recurrence or metastasis . Most ofBecause of its rarity and histological complexity, there exist diagnostic challenges for pathologists to differentiate PILA from other benign, intermediate or malignant vascular lesions. It is important to be able recognize this tumor in order to avoid potential misdiagnosis and improper management of afflicted patients. Our additive case is also presented for its rarity of site. It is the first case of intraosseous PILA with multiple lesions of facial bones. The diagnosis of intraosseous PILA is difficult and should be made cautiously. Besides confirmation by strict morphological criteria, immunohistochemical analysis is helpful for demonstrating the lymphatic phenotype of tumor.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 we have no competing interests.BL and YL made contributions to acquisition of clinical data, and analysis of the histological features by H&E staining and immunoassays. They are joint first co-authors and made an equal contribution to this work. XYT drafted the manuscript. ZL revised manuscript critically for important intellectual content and had given final approval of the version to be published. All authors read and approved the final manuscript.


We present a case of a twin pregnancy in which one fetus developed a rapidly growing unilateral intrathoracic tumor. While a cystic adenomatoid malformation was suspected in the ultrasound scan, the magnetic resonance scan suggested a pulmonary blastoma or a bronchioalveolar carcinoma. Postnatal chest radiography and contrast-enhanced computed tomography of the affected newborn were performed, and it was ruled out the possibility of malignant origin. Finally, the anatomopathologic exam revealed the presence of a mesenchymal hamartoma in the chest wall. Nevertheless, parents refused any treatment for the newborn. Fetal tumors are heterogeneous group of neoplasias that are unique as far as their histological characteristics, anatomical distribution, and physiopathology are concerned. The biological behavior of tumors in the fetus may differ drastically if compared with an adult . Congenital thoracic malformations, which comprise a wide spectrum of abnormalities, are often detected by routine prenatal ultrasound. However, the diagnosis usually cannot be established with absolute certainty before birth. Their presence involves having to rule out if there is a pulmonary issue or if its origin lies in the costal wall and does not affect the lung since the diagnosis impression, the prognosis, and treatment differ . UltrasoMesenchymal hamartomata in the chest wall are extremely infrequent benign tumors that tend to appear during infancy, and their presence in the prenatal period is exceptionally rare . ClinicaThe congenital cystic adenomatoid malformation of the lung is a rare fetal affection with an incidence of 1 : 25000 to 1 : 35000 pregnancies . When a We present a case of a twin pregnancy with one fetus with a rapidly growing unilateral intrathoracic tumor. From the ultrasound scan, a cystic adenomatoid malformation was suspected, while the magnetic resonance scan suggested a pulmonary blastoma or a bronchioalveolar carcinoma. Finally, the anatomopathologic exam revealed the presence of a mesenchymal hamartoma in the chest wall. The literature includes very few cases of mesenchymal hamartoma with accelerated growth complicated with hydrops fetalis in one twin during a bichorial biamniotic twin pregnancy. A 31-year-old nulliparous woman presented a bichorial biamniotic twin pregnancy, accomplished by artificial insemination due to primary sterility problems. The patient had no outstanding medical or surgical background. The fetal morphology ultrasound scan, performed at 20-week gestation, showed that both fetuses were apparently normal. Pregnancy developed normally until 23 weeks of gestation. An ultrasound scan showed supradiaphragmatic avascular cystic formation in the right hemithorax of the first fetus of 34 × 26 mm, suggesting a type I cystic adenomatoid malformation (CAM). Serial ultrasounds were performed by the maternal-fetal medicine service every two weeks, starting at 23 weeks, 3 days of gestation. As part of the diagnostic imaging protocol, fetal magnetic resonance (MR) was performed at 23 + 5 weeks of gestation. Sagital, coronal and axial fetal MR revealed a lesion with several cysts (hyperintensive at T2) occupying a space in the right inferior lobe. A more caudal cyst was noted, measuring 25 × 20 mm, with a second cranial cyst of 11 × 22 mm with a liquid level . Given the discovery of more thickened cystic walls than usually found in a CAM, and since the chest wall was apparently affected, the radiologist suggested considering a pulmonary blastoma or bronchioalveolar carcinoma, tumors described in patients with a previous CAM. However, since CAM-associated tumors have been described to appear postnatally and given the ultrasound scan findings, the first diagnostic suspicion was a type I CAM in the first twin. The amniocentesis at 25 + 1 weeks of gestation proved normal XY karyotypes for both fetuses. The ultrasound scan done at the time of this invasive technique showed an image that was compatible with a CAM in the first fetus' right hemithorax. A subsequent ultrasound scan at 28 weeks of gestation evidenced moderate ascites and hydrops fetalis. At 30 + 5 weeks of gestation, despite tocolytic treatment to prevent preterm delivery, a cesarean section had to be performed because of podalic presentation of the first fetus. The first male newborn weigh was 1700 g, Apgar score 8/10, umbilical arterial pH (ApH) 7.27 , umbilical venous pH (VpH) 7.39 , and he needed assisted ventilation. Second twin weighed 1400 g, Apgar score 9/10, ApH 7.28, VpH 7.36, and a favourable outcome. Postnatal chest X-ray revealed right hemithorax opacification due to a large-sized mass with calcium density and destruction/erosion of the 4th–7th right costal arches, plus leftward tracheal deviation and centralized intestinal luminogram, probably because of ascites . A contrThe mother had an uncomplicated immediate puerperium and was discharged 72 hours after the cesarean section. Mesenchymal hamartoma of the newborn was confirmed by anatomopathological study made after the autopsy. Congenital thoracic malformations are often detected by prenatal ultrasound. Their presence involves having to rule out if there is a pulmonary issue or if its origin lies in the costal wall and does not affect the lung because the prognosis and treatment differ . UltrasoFetal thoracic malformations are examined by color Doppler images and nuclear magnetic resonance (NMR) scans, and the perinatal anatomopathological study proves definitive to associate tumors. The NMR scan allowed a better definition of the main characteristics than the ultrasound scan and also a better spatial valuation with orthogonal tridimensional views . When a We present a case of a twin pregnancy with one fetus with a rapidly growing unilateral intrathoracic tumor. From the ultrasound scan, a cystic adenomatoid malformation was suspected. A NMR scan was done at 23 + 5 weeks gestation, which revealed a lesion with several cysts (hyperintensive at T2) occupying a space in the right inferior lobe. A more caudal cyst was noted, measuring 25 × 20 mm, with a second cranial cyst of 11 × 22 mm with a liquid level . There wMesenchymal hamartomata in the chest wall are extremely infrequent benign tumors that tend to appear during early infancy, and their presence in the prenatal period is exceptionally rare . They inMesenchymal hamartomata are frequently located to the rear, tend to affect many adjacent ribs, and may be multifocal and bilateral. Hamartomata in the bilateral chest wall are an infrequent occurrence and tend to be confused with a malignant lesion. They may involve distortion and cardiac and lung displacement . In ultrA correct mesenchymal hamartoma diagnosis is crucial because most masses located in the chest wall of infants are malignant. As previously mentioned, a biopsy provides a definite diagnosis. Macroscopically, the hamartoma has a bloody appearance and contains small cysts. Microscopically, mature adipose tissue mixed with a large number of veins with various diameters and with collagen tissue is observed, and primitive mesenchymal elements with groups of lymphoid cells can be focally visualized . HistoloSeveral forms of treatment have been described, including the surgical excision of the mass and thermal radioablation. It is believed that the hamartoma becomes smaller in size after the first two years of life . TherefoIn conclusion, mesenchymal hamartomata have to be beared in mind in the differential diagnosis of fetal thoracic lesions. The encouraging prognosis of this benign tumour should be carefully reported to parents.