Benign neonatal hemangiomatosis is a condition in which multiple cutaneous hemangiomas appear at birth or shortly thereafter; visceral complications are absent. Here, we report a case of a consumption hypothyroidism in an Egyptian baby with benign neonatal hemangiomatosis., puffy eyes, cold extremities, hypothermia, bradycardia, and abdominal distension. An examination of his skin revealed more than 100 dome-shaped red-purple cutaneous hemangiomas that varied in size from 5 to 10mm on the back, the abdomen and the extremities without mucus membrane involvement. He had low serum free thyroxine concentration and triiodothyronine levels and high thyroid-stimulating hormone and reverse-triiodothyronine levels. A work-up that involved appropriate imaging ruled out visceral involvement. Based on the above mentioned data, a diagnosis of consumptive hypothyroidism due to benign neonatal hemangiomatosis was made. He was started on oral thyroid medication which was gradually increased to 90μg L-thyroxine daily (15μg/kg/day). After three months of treatment, he was able to sit alone without support and he had normal levels of thyroid-stimulating hormone and serum free thyroxine.An 8-month-old Egyptian boy with benign neonatal hemangiomatosis was referred to our institution for evaluation of developmental delay. Initial examination revealed a quiet baby who was able to sit only with support. He had hypotonia, a large anterior fontanelleThyroid function should be assessed periodically in babies with benign neonatal hemangiomatosis, especially if symptoms of hypothyroidism appear or the size and number of hemangiomatosis increase rapidly. Moreover, high doses of L-thyroxine may be needed to achieve euthyroidism during the infancy. Benign neonatal hemangiomatosis (BNH) is a rare, self-limited disease where multiple capillary hemangiomas occur exclusively in the skin. Spontaneous regression occurs usually within the first two years of life. BNH mus. At the age of six months, he was unable to roll over prone to supine or vice versa. An examination revealed a quiet baby who was able to sit only with support but unable to orient to voice. His height and weight were 25th percentile. Vital signs on admission were recorded as follows: temperature, 36°C; blood pressure, 80/60mmHg; and heart rate, regular at 66 beats/minute. A physical examination found generalized hypotonia, a wide anterior fontanelle, puffy eyes, cold extremities, and mild abdominal distension. There were no signs of rickets or thyroid enlargement. An examination of his skin revealed more than 100 dome-shaped red-purple cutaneous hemangiomas that varied in size from 5 to 10mm on the back, the abdomen and the extremities with no mucus membranes involvement  to reduce serum thyrotropin concentrations to normal which are thought to reflect tumor size and type 3 deiodinase activity[Thyroid function should be assessed periodically in a baby with BNH, especially if symptoms of hypothyroidism appear or the size and number of hemangiomatosis increase rapidly. Moreover, high doses of L-thyroxine may be needed to achieve euthyroidism during the infancy.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.BNH: Benign neonatal hemangiomatosis;CT: Computed tomography;DNH: Diffuse neonatal hemangiomatosis;FT4: Serum free thyroxine;rT3: Reverse-T3;T3: Triiodothyronine;TSH: Thyroid-stimulating hormoneThe authors declare that they have no competing interests.KA and HS diagnosed, investigated, followed up, managed the patient and drafted the manuscript. Both authors read and approved the final manuscript.


And it also revealed that the rupture of the cirsoid chorionic vessels had led to IUFD.We report a 32-year-old woman  with a vesicular lesion in her uterus that was pointed out on ultrasound at 8 weeks' gestation. Amniocentesis at 15 weeks' gestation showed a normal female karyotype, 46XX. As the pregnancy advanced, the mole-like lesion became relatively reduced. Throughout gestation, the maternal human chorionic gonadotropin level was normal, but the serum alpha fetoprotein level rose as her pregnancy progressed. Her fetus did not exhibit any remarkable anomalies. The patient visited our hospital complaining of a diminished feeling of fetal movements at 36 weeks 5 days' gestation, and intrauterine fetal death (IUFD) was confirmed. She delivered a 2336-g female without any definite anomalies. A pathological examination led to a diagnosis of placental mesenchymal dysplasia, and androgenetic/biparental mosaicism in the placenta was identified using p57 Placental mesenchymal dysplasia (PMD) is a rare placental anomaly characterized by placentomegaly and grapelike vesicles resembling molar pregnancy. The incidence of PMD is reportedly 0.02% , with a A 32-year old woman, gravid 1, para 1, began visiting our hospital at 4 weeks' gestation. An ultrasound performed at 8 weeks' gestation as part of a routine prenatal checkup detected a vesicular lesion in her uterus Figure . A crosskip2 gene, which encodes a cyclin-dependent kinase inhibitor, is expressed in the maternal genome, whereas the paternal allele is transcriptionally silent [kip2 showed that some villi were composed of stromal cells that were negative for p57kip2 only in the affected lesion (Figure The p57n Figure  and 6b. The main differential diagnoses of PMD are partial hydatidiform mole, dichorionic twins of a normal fetus and complete mole, and confined placental mosaicism. When a prenatal ultrasonographic examination detects a vesicular lesion in the presence of a fetus in uterus, whether a single or twin pregnancy is present should be determined. A twin pregnancy would consist of dichorionic twins of a normal fetus and complete mole. In the present case, ultrasound appeared to reveal a single pregnancy. Unlike partial moles, the majority of PMD are diploid . In 70 -The placenta is usually extremely large for the gestational age and the umbilical cord is relatively long in cases with PMD ,3. The gkip2 protein is a potential marker that may prove helpful in distinguishing PMD from molar pregnancy. The immunohistochemical detection of androgenetic/biparental mosaicism in stromal cells suggests to a diagnosis of PMD, because this mosaicism is unusual in molar pregnancies [Although morphologic gross findings of PMD placentas are so often mimicked by features of partial hydatidiform moles, PMD placentas are markedly enlarged and macroscopic vascular anomalies such as cirsoid dilated chorionic vessels with or without thrombi and umbilical cord anomalies like a long umbilical cord that were observed in our case are often co-existence in PMD placentas, but they are not usual in molar pregnancies ,13. Micrgnancies . DichoriPMD has distinct clinicopathological features. According to Truc et al. , 82% of The underlying cause of PMD is currently unknown. Recently, Kaiser-Rogers et al. proposed androgenetic/biparental mosaicism as the origin of some cases of PMD and suggested that the phenotype of androgenetic mosaicism can presumably range from mild PMD, which may not even be diagnosed, to the typical findings of a complete hydatidiform mole, depending on the extent and distribution of the androgenetic lineage . The autPMD should be included in a differential diagnosis of cystic lesions of the placenta by sonography, especially when a phenotypically normal-appearing fetus can be identified. When the prenatal characteristics of PMD, such as an elevated AFP and normal hCG level in the mother, a normal female karyotype revealed by amniocentesis, and dilated subchorionic vessels revealed by ultrasonography during the third trimester, are detected, termination of the pregnancy at an optimal time could be considered, since sudden IUFD including rupture of the cirsoid chorionic vessels might occur at any time throughout gestation.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.The authors declare that they have no competing interests.TU and SK designed the study, performed clinical investigation, and wrote most of the drafts. KK, FT, AS, TS and TK assisted the clinical investigation, and supported to prepare the manuscript. HI performed the histological and immunohistochemical evaluation, and helped rewrite the manuscript. All authors read and approved the final manuscript.


Anaplastic carcinoma of the pancreas (ACP) is a rare neoplasm of the pancreas. ACPs are aggressive neoplasms with a poorer prognosis than poorly differentiated ductal adenocarcinomas of the pancreas. The 3-year survival rate of patients with ACP is less than 3%, with a life expectancy of 10 to 20 months. We describe here a 64-year-old man with ACP mimicking a submucosal gastric tumor. The patient was found to have a giant mass mimicking a submucosal tumor. Total gastrectomy with splenectomy and partial resection of the tail of the pancreas were performed. The pathological diagnosis was ACP, with immunohistological findings showing pleomorphic-type ACP. Because the surgery was noncurative, the patient received adjuvant chemotherapy with paclitaxel but died of peritoneal dissemination and multiple liver metastases 4 months after surgery.  Anaplastic carcinoma of the pancreas (ACP) is a rare, aggressive type of pancreatic cancer with a short median survival. Because of its rarity, the clinical presentation, surgical results, and management are not well known, although some Japanese patients have reported long-term survival after surgery. In other patients, however, the tumor is not resectable and chemotherapy is required. Adjuvant chemotherapy has yielded poor survival outcomes. We describe here a patient with ACP who underwent surgery followed by adjuvant chemotherapy with paclitaxel.A 64-year-old man was admitted to our hospital with general fatigue and diarrhea. On physical examination, he appeared to be cachexic and a firm mass was palpable on the upper abdomen. Laboratory investigation revealed a slight decline in hemoglobin concentration to 8.2 g/dL and elevated serum carcinoembryonic antigen concentration (67.7 ng/mL). General condition of the patient was poor. Only the upper gastrointestinal endoscopy and plain CT were performed. Upper GI endoscopy revealed a giant lesion within the stomach, located at the posterior wall of the body, showing a submucosal tumor-like appearance with oozing from ulceration . FiberscA plain CT scan showed a mass with central necrosis in the stomach, with suspected infiltration of the spleen and tail of the pancreas , as wellThe patient underwent a total gastrectomy with splenectomy and partial resection of pancreas tail, accompanied by resection of disseminated lesions. Pathological specimens included the stomach, the spleen, part of the pancreas tail, and the disseminated lesions. Gross examination showed an exophytically-growing tumor in the stomach, measuring 20 × 18 cm  and weigαSMA, CD34, C-kit, and S-100. The epithelial component was strongly positive for pancytokeratin, CEA and CA19-9; moderately positive for desmin, and negative for vimentin (The lesion was assayed immunohistochemically using monoclonal antibodies against pancytokeratin, CEA, CA19-9, vimentin, chromogranin, synaptophysin desmin, vimentin . PositivαSMA, CD34, C-kit, and S-100 (In contrast, the sarcomatoid component was strongly positive for CEA and vimentin, moderately positive for CA19-9, desmin, and pancytokeratin, weakly positive for cytokeratin, and negative for nd S-100 .Based on these immunohistological findings, the patient was diagnosed with a pleomorphic-type ACP. Because his surgery was noncurative, he started adjuvant chemotherapy with paclitaxel. However, the patient died of peritoneal dissemination and multiple liver metastases 4 months after the operation.ACPs, also called “undifferentiated carcinomas” with or without osteoclast-like giant cells, “giant-cell tumors,” and “pleomorphic carcinomas” of the pancreas, are rare malignancies of the pancreas, constituting 0.5–7% of nonendocrine malignancies of the pancreas –3.Clinical symptoms associated with ACP include abdominal pain, body weight loss, anorexia, and back pain, resembling the symptoms of adenocarcinoma of the pancreas. Because the diameter of ACP tends to be greater than that of pancreatic adenocarcinoma, ACPs may be palpated as huge abdominal masses at diagnosis , 5. CT fThe pathogenesis of ACP is unclear, although a recent study of the initial steps in the evolution of early undifferentiated pancreatic carcinoma provided evidence for its ductal origin, suggesting an epithelial origin of the mononuclear undifferentiated cells . These fACPs are aggressive neoplasms with a poorer prognosis than poorly differentiated ductal adenocarcinomas of the pancreas. The 3-year survival rate of patients with ACP is less than 3%, with a life expectancy of 10 to 20 months , 8, 9. A


Over 200 cases of undifferentiated embryonal sarcoma of the liver (UESL) have been reported since 1978 when this disease was first described. In the present study, we describe a case of UESL in a 7-year-old female, whose initial symptoms included swelling in the upper abdomen and a palpable enormous irregular tumor. A magnetic resonance imaging (MRI) examination revealed a massive focal lesion in the right lobe of the liver. Hepatic malignant tumor with a high possibility of hepatoblastoma was diagnosed. The tumor was surgically removed and confirmed to be UESL by postoperative pathology and immunohistochemical staining analysis. The patient then received chemotherapy consisting of three cycles of epirubicin  and cisplatin . To date, the patient has survived for 22 months, and is currently in a good general condition without evidence of local metastasis or recurrence. Although UESL has a high malignancy and a poor prognosis, cases of long-term survival with improved diagnosis and therapy have recently been reported. Therefore, it has been proposed that UESL should not be considered as an hepatic tumor with a poor prognosis. Total resection with preoperative or postoperative radio-chemotherapy is currently considered to be the key approach to improving the survival rate. Undifferentiated embryonal sarcoma of the liver (UESL), also known as malignant mesenchymoma or embryonic sarcoma, is a rare disease  that rarA 7-year-old female child was admitted to the Department of Hepatobiliary Surgery of the Shandong Cancer Hospital  on December 29 2009, with an augmented swelling in the upper abdomen and a palpable massive irregular tumor that had been identified >10 days previously. A magnetic resonance imaging (MRI) scan revealed a massive focal lesion in the right lobe of the liver. The lesion was diagnosed as a malignant tumor with a high possibility of hepatoblastoma. The patient did not present with fever, bellyache, abdominal distention, diarrhea, jaundice, nausea or vomiting.Physical examination revealed that scleral or systematic mucocutaneous icterus and swollen lymph nodes were not present. The patient had ectasia of the upper abdomen, particularly in the right superior belly. The liver under the rib edge was palpable for 7 cm, and the surface tuberculum crossing the left of the ventral median line was not palpable for 1 cm. No percussion pain was observed in the hepatic renal region.μmol/l; aspartate transaminase (AST), 63.8 μmol/l; albumin, 32.01 g/l; albumin and globulin ratio, 1.25; total bilirubin (Tbil), 19.7 μmol/l; direct bilirubin (Dbil), 2.5 μmol/l; indirect bilirubin (Ibil), 17.2 μmol/l; alkaline phosphatase (ALP), 162 μmol/l; γ-glutamyl transpeptidase (GGT), 80 μmol/l.The laboratory examination results were as follows: α-fetoprotein (AFP), 0.853 ng/ml; carbohydrate-associated antigen (CA) 19-9, 23.39 U/ml; carcinoembryonic antigen (CEA), 0.788 ng/ml; alanine transaminase (ALT), 68.3 The MRI examination revealed a massive tumor (15×13×10 cm) in the right lobe of the liver , which dThe following clinical pathology results were observed by the naked eye. The size of the tumor in the hepatic right lobe was ∼17.0 cm, and the incisal surface was occupied by a gray-yellow/red/white swelling. Additionally, the texture of the swelling was impalpable and soft. Necrosis was observed and the swollen tissue demonstrated the partial stick-slip phenomenon. Further clinicopathological results were obtained using a light microscope. Under the light microscope  and 2, tThe immunohistochemistry results were as follows: Broad spectrum creatine kinase (CK)(−), cytokeratin 8/18(CK8/18)(−), S-100(−), MSA(−), myoglobin(−), SMA foci(+), a-ACT foci(+), desmin foci(+), vimentin foci(+), CD34(+), CD68(+), Ki-67(+) and P53(+).The karyotype analysis revealed that the chromosomal aberrations accounted for the remaining 5% of all chromosomes, which involved chromosomes 6, 11, 12 and 14, as well as the X chromosome. The aberrations included fourcentric chromosomes, deletion, disruption and marker chromosomes.et al in 1978  scan reveals cystic lesions with low density that is reflected as a fluid. Therefore, the difference between CT and ultrasonography results is a critical characteristic of this disease. However, both scans reveal cystic lesions when hemorrhage, necrosis and liquefaction account for the major part of the tumor. An enhanced CT scan of the tumor solid part, septation and pseudo-capsule shows different degrees of reinforcement. However, in the present study, MRI revealed that the T1W1 scan mainly detected a low or equal signal and high signal foci, which were a reflection of a hemorrhage in the tumor. Additionally, the T2W1 scan frequently exhibited a mixed high signal. Positron emission tomography (PET)-CT scans are also used in the diagnosis of UESL, and they have a critical diagnostic value for UESL patients, particularly those with metastasis of an extra-hepatic organ.The immunohistochemistry results reveal the positive expression of SMA, a-ACT, desmin, vimentin and actin in UESL patients, and a minority of cases are positive for PCNA, CK8/18 and p53; while AFP, S-100, CEA, CA 19-9 and cytokeratin have negative expression. The indices of the patient were as follows: SMA foci(+), a-ACT foci(+), desmin foci(+), vimentin foci(+), broad spectrum CK(−), CK8/18(−), AFP(−), CEA(−) and CA19-9(−).Generally, a definite diagnosis of UESL is not able to be determined preoperatively; the diagnosis relies on postoperative pathological analysis and immunohistochemical results. UESL ought to be differentiated from hepatoblastoma, embryonal rhabdomyosarcoma, hepatic mesenchymal hamartoma and hepatic echinococcosis. Hepaoblastoma, which mainly occurs in infants aged <3 years, is composed of primary hepatic parenchymal cells with small cellular heteromorphism, little karyokinesis, increased blood sinus and positive expression of endosomal membrane protein, vimentin and AFP revealed by immunohistochemistry. By contrast, embryonal rhabdomyosarcoma mainly occurs in infants aged <6 years, and the tumor is mainly composed of striated muscle maternal cells in different phases and primary mesenchymal cells. The myosin often exhibits strong positive expression by immunohistochemistry, and transverse striation may be observed by preservative associated transient hyperfluorescence (PATH) staining. With hepatic mesenchymal hamartoma, which mainly occurs in infants aged <1 year, the tumor is composed of a mucus matrix and a large number of star and fusiform primary mesenchymal cells, whereas the tumor cells themselves do not exhibit heteromorphism. By contrast, hepatic echinococcosis is a local parasitic disease that mainly occurs in adults aged 20–40 years. Casoni and complement fixation tests are used to diagnose this disease, and the positive rate is 90–95%, which has a critical diagnostic value. A case history combined with imaging analysis has been demonstrated to be beneficial for the discrimination of UESL. A previous study has revealed that UESL is often misdiagnosed as echinococcosis of the liver , as wellet al described a case of UESL in a child with distant metastases in the lung and adrenal glands (et al suggested that the best method for improving survival is total resection, regardless of whether the tumor is ruptured (et al proposed that surgical resection combined with chemotherapy is the most effective therapy for UESL patients with tumor rupture. Additionally, the authors suggested that chemotherapy containing 3–4 types of drugs, such as adriamycin, cisplatin and others, demonstrated beneficial therapeutic effects and significantly improved survival rates (et al demonstrated that a UESL patient who underwent interventional therapy and surgical resection exhibited a prolonged survival compared with patients who underwent surgical resection only (et al described a case of a pregnant UESL patient who underwent trans-catheter ablation to control the development of the tumor (UESL is generally considered to be a highly invasive malignant tumor in hepatic primary mesenchymal tissue with distant metastasis. Lee l glands . The prol glands . In recel glands . The keyruptured . Uchiyamal rates . Li et ahe tumor . Certainhe tumor .In the present case, blockage of the blood flow in half the liver was performed while the patient was under total anesthesia on December 29, 2009. A massive tumor with a smooth and glossy surface was observed in the right hepatic lobe and the Glisson’s capsule remained intact. Moreover, in the patient’s left hepatic lobe, the first and second porta hepatis had been removed by the pressure, and the inferior vena cava had been distorted. The surgery ran smoothly. The hepatic right lobe and partial left internal lobe were resected. The patient recovered successfully, and a chemotherapy regimen of epirubicin at 20 mg for days 1–2 and cisplatin at 15 mg for days 1–3 was administered at 14-day intervals following surgery. Subsequently, epirubicin at 20 mg for days 1–2 and cisplatin at 15 mg for days 1–3 were administered in the second and third cycle of chemotherapy on July 27, 2010. Currently, the patient’s physical status is normal, and no local recurrence or metastasis has been observed. We conclude that preoperative and/or postoperative interventional therapy, combined with radiotherapy and chemotherapy, may improve survival rates and times in certain cases. The precise timing of the total surgical resection is crucial to prevent invasive growth of the tumor, and a liver transplantation is the most effective therapy for a patient whose tumor cannot be surgically resected.


We describe the treatment of a 46-year-old Saudi man with advanced stage liver metastatic neuroendocrine rectal cancer. The patient presented with a large liver lesion and rectal bleeding. He was cachectic, with a firm tender mass 20 mm above the anal verge. Computed tomography (CT) showed a mass 9.5 × 13 cm in size in the right hemi-liver, abutting the middle hepatic vein. The patient refused treatment, and consulted another hospital. After 3 months, he presented with the same symptoms in addition to delirium. Colonoscopy showed an ulcerating anorectal mass, from which a biopsy was taken. Repeat CT showed an increase in the size of the liver lesion to 17 cm and no change in the pelvis. The final histopathology report identified anaplastic small cell carcinoma. The patient underwent extended right liver resection followed by abdominoperineal resection, then 13 cycles of chemotherapy and monthly somatostatin injections. At the most recent follow-up, the patient had been disease-free for 48 months. Surgical resection (R0) of the primary and secondary tumor, followed by platinum-based chemotherapy can result in good survival in cases of small cell carcinoma with large liver metastasis, irrespective of whether the primary or secondary tumor is resected first. Neuroendocrine tumors include a wide spectrum of lesions including microcarcinoid, carcinoid, and mixed endocrine/exocrine tumors, and small cell carcinoma (SCC). SCC most commonly originates in the lungs, but it can originate anywhere in the body, including the esophagus , large bGastrointestinal (GI) SCC is a type of extrapulmonary carcinoma, and the GI tract is known to have the largest population of neuroendocrine cells . ApproxiStaging  and manaUnfortunately, patients with SCC of the GI tract have a dismal prognosis, with a median survival of only 6 to 20 months ,13. MoreWe therefore describe the first such case, to our knowledge, of successful surgical treatment of SCC of the rectum with advanced stage liver metastasis, followed by combination chemotherapy achieving long-term survival.A 46-year-old Saudi man was referred to Al-Hada Military Hospital, Taif, Saudi Arabia; he reported bleeding from the rectum for 6 months, along with decreased appetite, weight loss, and pain in the right upper quadrant of his abdomen. On examination, he was conscious and cachectic, with a mildly distended abdomen and a large hepatomegaly. Upon digital rectal examination, a firm and tender mass was found, 2 cm above the anal verge.Laboratory tests including complete blood count, renal panel, liver function test, carcinoembryonic antigen, and α-feto-protein were all normal. A CT scan of the abdomen showed a huge mass (13.5 × 8 cm) in the right liver reaching down to the right iliac fossa and pushing the abdominal viscera away.Unfortunately, the patient refused to undergo colonoscopy, and was discharged against medical advice, because he wished to get a second opinion. Three months later, he returned to our hospital. He presented to the emergency department with delirium and impaired liver function , alkaline phosphatase 203 μ/l 40 to 150 μ/l), aspartate aminotransferase 598 μ/l (5 to 35 μ/l), and alanine aminotransferase 373 (0 to 55 μ/l])). A repeat CT scan showed a dramatic increase in the size of the liver mass  (N0) , which was followed by monthly treatment with intrasmuscular somatostatin 20 mg, which is continuing. Positron emission tomography in May 2012 and CT in September 2012 did not show signs of recurrence. To date, the patient has been disease-free for 48 months.et al. [et al. reported a median overall survival of 15.3 months in a retrospective study of 24 patients [Only a few cases of patients with limited rectal SCC with long-term survival have been published. While only three cases -16 with et al.  reportedpatients . In the et al. [et al. [Only three cases of SCC of the rectum with liver metastasis have been published, and the patients were not treated surgically in any of these cases. In a report by Robidoux et al. , a patie [et al.  reported [et al. , in whicAfter reviewing the literature, we decided to attempt surgery on our patient because we could find no apparent significant advantages of chemotherapy or a combination of chemotherapy and radiotherapy in such cases (extensive disease), although this was based only on the three existing cases reported in the literature. Our treatment strategy was unique in that we started with the secondary tumor because the patient presented with deterioration of liver function, recent onset of delirium, and significant rapid growth of the liver with no change in the rectal tumor during the 3 months that elapsed between the patient deciding to seek another opinion and the time he re-presented to us. Surgery was followed by the established chemotherapy protocol (cisplatin and etoposide) to achieve the maximum result, followed by somatostatin therapy. This case is the first in which the patient had a huge liver metastasis of rectal SCC, and was successfully treated with surgery followed by chemotherapy. This treatment has so far resulted in long-term disease-free survival (48 months).Surgical resection (R0) of rectal SCC (limited or extensive disease), starting with the primary or secondary tumor, depending on the patient’s presentation, followed by (platinum-based) chemotherapy may result in better outcome and survival.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.BOA-J was the primary treating surgeon, followed up the patient, and wrote the paper. OAM: collected all the data, helped in writing the paper, and followed up the patient in the ward during his treatment process. All authors read and approved the final manuscript.


Splenogonadal fusion (SGF) is a rare congenital abnormality that affects children of both genders. Very few cases of SGF have been diagnosed preoperatively. In this study, the surgical findings and laparoscopic treatment of four children with SGF associated with intra-abdominal cryptorchidism are described. Laparoscopy was demonstrated to be the only accurate exploratory procedure for the diagnosis and surgical treatment of SGF with non-palpable testis. Splenogonadal fusion (SGF) is a rare congenital anomaly in which close proximity of spleen and gonad during early embryological development facilitates fusion. SGF may be divided into continuous or discontinuous types. The continuous type of SGF describes the gonad attached to the spleen. The discontinuous type consists of gonadal fusion with an accessory spleen or ectopic splenic tissue. The diagnosis of this uncommon anomaly is rare and difficult to diagnose preoperatively. Certain patients with SGF have undergone unnecessary orchidectomy due to the presence of a testicular lump . Therefo3 in size, of medium hardness without any tenderness was palpable in the left scrotum. Surgery was performed under general anesthesia. During the surgery, a reddish-brown mass attached to the upper pole of the left testis was identified. The mass and testis were free from other intra-abdominal structures. The mass was confined to an intact capsule on the upper pole of the testis and occupied one-third of the volume of the testis. Postoperative examination of the specimen under the microscopic revealed it was spleen tissue that was separated from the surrounding compressed testicular tissue (A male, 6 years old, presented with a painless mass in the left scrotum. Upon physical examination, a swollen testicle ~2.5×2.5×4.5 cmr tissue .A male, 7 years old, presented with a left inguinal mass. It was a soft mass with a clear boundary, which was palpable from the left groin area to the scrotum, but was not able to be completely pushed back into the abdominal cavity. During laparoscopy, a congenital hernia was confirmed by the red cord-like tissue in the left inguinal region that connected to the testicle, entered into the left internal inguinal ring and were fused to the hilum of the spleen. The diameter of the cord-like tissue was ~1.0 cm. The proximal cord-like tissue was ligatured prior to being resectioned  and 3. P3 (A male, 2-years-old, presented with bilateral undescendent testes. The penis was bent downwards, while the urethra was located at the junction of the penis and scrotum. The testis were not palpable in bilateral scrotums and bilateral inguinal region. Ultrasound demonstrated there was no testicular-like mass in the bilateral inguinal and abdominal cavities. During laparoscopy, a testicle-like mass was located in the left iliac fossa. It was dark red, with clear fixed boundaries and measured 2.0×3.0×4.0 cm3 . The rem2 in size with a uniform signal and clear boundary. The location and shape of the spleen was noted to be normal during the laparoscopy. A testis-like reddish brown mass was observed that was attached to the lower pole of the left kidney, with clear, fixed boundaries and measuring 2.0×2.5×4.0 cm3. The right testicle was pulled down and fixed, while the left, testis-like mass was removed. Pathological examination of the specimen obtained from the left side revealed SGF.A male, 12-years-old, presented with bilateral undescendent testes. The penis had a postoperative appearance, a bend in it having been corrected. The urethra was located in the glands. The bilateral scrota were underdeveloped, without the testis. Surgical scars were visible at the bilateral inguinal region, but there was no testis-like mass. An oval-shaped mass shadow on the lower pole of the left kidney was confirmed by MRI. It was ~2.3×4.0 cmSGF is a rare congenital anomaly, between the spleen and a gonad or mesonephric derivatives, almost always presenting on the left side in males. The male preponderance may be due to the fact that the male sex gland is located superficially and is easily located. Female gonads are inside the body and have fewer complications than their male counterparts. SGF is often discovered accidentally during gynecological surgery, with a higher prevalence in Caucasian individuals, followed by that in African descent and other ethnicity ,3. SGF iSGF may be classified into two types, continuous and discontinuous, but clinically there are no significant differences between the two types . Le Rouxet al(Cryptorchidism and inguinal hernias are the most common malformations associated with SGF. In total, 31% of SGF patients are diagnosed with cryptorchidism or inguinal hernias, and in 59% of cases the cryptorchidism is diagnosed as bilateral . In caseet al reviewedet al reported99TCm spleen scanning, aid with the diagnosis of SGF (The diagnosis of SGF prior to surgery is challenging. SGF typically presents as an asymptomatic testicular mass and other manifestations may include acute testicular pain and swelling caused by ectopic splenic tissue infections , but thes of SGF –15. Howes of SGF . In the The decision to resection the entire splenogonadal tissue was based upon the anomalous appearance of the testicle and its fusion to ectopic splenic tissue. Once an accurate diagnosis has been achieved without significant complications, particularly in the scrotum, no surgery is required. Even if surgery is performed, in most cases the testis may be preserved. Performing laparoscopy and testicle-sparing surgery is advised. Spleen tissue is easily separated from the gonad and as a result the testis may be retained, unless there is a high degree of cryptorchidism. If a tumor is suspected, frozen sectioning may aid with the diagnosis. Currently, to the best of our knowledge, there have been no reports of malignant SGF.In conclusion, the diagnostic evaluation of patients with an abnormal gonad is complex due to multifactorial etiopathogenesis and the rarity the condition. The primary aim of diagnosis is to rule out malignancy. We suggest three steps that should be considered when diagnosing and treating SGF. Firstly, a mass found at birth growing slowly for several years in a benign condition should be considered. Secondly, various imaging techniques should be used to investigate the nature of the mass. Thirdly, in doubtful cases, a biopsy should performed during surgery or, preferably prior to making an incision, for example, by needle biopsy, punch biopsy or a classical bivalve biopsy and regional node evaluation. If the mass proves to be malignant, a radical resection should be performed immediately. If the malignancy is unconfirmed, an orchiectomy is sufficient for further pathological study. In cases where the mass has been identified to be benign, but the organ has been opened, removal of splenic tissue may be performed. With SGF, there may be no need for surgery. However, diagnosis and surgical treatment by a diagnostic laparoscopy are suggested. Laparoscopy is a safe and reliable approach that is highly accurate in the diagnosis and treatment of nonpalpable testis.


Synovial sarcoma presenting in the mediastinum is exceedingly rare. Furthermore, data addressing optimal therapy is limited. Herein we present a case where an attempt to downsize the tumor to a resectable state with chemotherapy was employed.A 32 year female presented with massive pericardial effusion and unresectable huge mediastinal mass. Computed axial tomography scan - guided biopsy with adjunctive immunostains and molecular studies confirmed a diagnosis of synovial sarcoma. Following three cycles of combination Ifosfamide and doxorubicin chemotherapy, no response was demonstrated. The patient refused further therapy and had progression of her disease 4 months following the last cycle.Synovial sarcoma presenting with unresectable mediastinal mass carry a poor prognosis. Up to the best of our knowledge there are only four previous reports where primary chemotherapy was employed, unfortunately; none of these cases had subsequent complete surgical resection. Identification of the best treatment strategy for patients with unresectable disease is warranted. Our case can be of benefit to medical oncologists and thoracic surgeons who might be faced with this unique and exceedingly rare clinical scenario. Soft tissue sarcomas (STS) are rare malignant tumors that comprise less than 1% of malignant neoplasms. In the thorax, where the majority of malignant tumors are carcinomas, the proportion of STS is even much less; probably less than 0.01% of all malignant thoracic neoplasms . These tThe mediastinum as a primary site of occurrence of synovial sarcoma is exceedingly rare. Although complete resection had been the only therapy associated with long term survival, uncertainty exists regarding the best therapeutic strategy for patients with unresectable disease. External beam radiotherapy (EBRT) is the most commonly employed primary therapy when the mass is unresectable ,4. ChemoA 35 year old female presented with bilateral lower limb swelling and exertional shortness of breath that had developed over few weeks. She was found to have pericardial effusion that was aspirated at another hospital. The patient was discharged two days later on non-steroidal anti-inflammatory medication after showing subjective improvement. Two weeks later, she had recurrence of her symptoms due to recurrent pericardial effusion with signs of impending tamponade that was managed with a pericardial window. A computed axial tomography (CT) scan of the thorax demonstrated a huge mediastinal mass, for which a CT guided biopsy was carried out. The patient was referred to us later for further evaluation. On presentation she was asymptomatic and her physical examination disclosed mild bilateral pitting edema, but was otherwise noncontributory. A review of her CT scan showed a huge mass in the anterior mediastinum and no evidence of distant metastases. Review of her pathology specimen showed proliferation of spindle cells with oval normochromatic nuclei, scattered mitotic figures and no evidence of necrosis. Prominent thin- walled blood vessels were seen with branching embarking a hemangiopericytoma-like vascular pattern Figure A. No epiThe mass was unresectable according to the thoracic surgeons’ assessment because it was invading the pericardium and engulfing the major vessels. We elected to proceed with combination chemotherapy in an attempt to downsize her tumor for possible subsequent resection. The option of EBRT was discussed, however; given the huge size of her mediastinal mass, a sufficient and definitive dose of radiation would not be feasible without severe toxicity. She received three cycles of Ifosfamide 2g/m2 every 12 hours D1-D3, Doxorubicin 75 mg/m2, Mesna and Granulocyte-colony stimulating factor (GCSF) starting 24 hours following completion of chemotherapy and continued until neutrophil recovery. She had recurrent neutropenic fever following the first two cycles in addition to severe thrombocytopenia and epistaxis following the second cycle which required 25% dose reduction of both agents in the third cycle. Her chest CT scan following the third cycle showed slight increase in tumor size. The patient refused further therapy and was referred to palliative care. Three months later, she started to suffer from progressive shortness of breath and chest discomfort secondary to mass effect, A CT scan of her chest and abdomen showed progression of the size of the mass without distant metastasis. The patient is still alive with her disease 11 months after diagnosis.Primary thoracic sarcomas are rare group of neoplasms that may arise in mediastinum, heart, lung, and pleura, and includes a wide variety of possible histologic subtypes. Although angiosarcoma, leiomyosarcoma, sarcomatoid mesothelioma, and rhabdomyosarcoma are the most commonly encountered histologies, other subtypes including synovial sarcoma, fibrosarcoma, malignant fibrous histiocytoma, chondrosarcoma, and primitive neuroectodermal tumors may arise in these locations. Apart from rhabdomyosarcoma which has a bimodal occurrence , almost all of these tumors affect middle aged and elderly patients, and there occurrence in children is exceedingly rare .Morphologically, synovial sarcoma can show several growth patterns and is basically divided into monophasic and biphasic subtypes. The better recognized variant is the biphasic synovial sarcoma which consists of proliferation of bland looking spindle-shaped cells in a collagenous background and hemangiopericytomatous vascular growth pattern, along with evidence of epithelial differentiation ranging from well-formed gland-like structure to aggregates of cudoidal cells. Monophasic tumors on the other hand can show either spindle cells only or occasionally might consist of epithelial component only . A poorlAlthough the majority arises in extremities , synoviaBecause the mediastinum is a host for a variety of primary and secondary neoplasms, differentiating synovial sarcomas from variety of other, more commonly encountered diagnoses such as lymphoma, mesothelioma, thymoma, metastatic carcinomas, as well as other sarcoma subtypes is of paramount importance. Careful assessment of biopsy specimen, with adjunctive imunostains is necessary for appropriate diagnosis. The availability of molecular genetic identification (FISH or polymerase chain reaction) of the t has improved diagnostic specificity for synovial sarcomas as this translocation is found in over 90% of cases . TestingFollowing establishment of diagnosis of a mediastinal or cardiac sarcoma, accurate staging to identify the extent of disease and to rule out distant metastases is an important and essential step for subsequent management.Apart from rhabdomyosarcoma and primitive neuroectodermal tumors, which require histology-specific chemotherapy regimens as integral part of treatment, management of other histologic subtypes, regardless of age and location, share same general principles. Surgical resection is the cornerstone of therapy of thoracic STS including those arising in the mediastinum if the mass is resectable; in fact, the ability to completely resect the mass is the most important factor associated with long term survival .Although there are no randomized phase III studies to define the most effective modality, patients with unresectable sarcomas are usually treated with systemic chemotherapy and/ or EBRT ,15. BecaFor patients presenting with unresectable mediastinal synovial sarcomas, very limited data exists regarding the optimal therapy that can effectively downsize the tumor to resectable state. A review of literature identified only 34 cases of primary synovial sarcoma of the mediastinum, 16 of them presented as unresectable disease (Table Unlike patients with unresectable disease, patients whose’ disease can be completely resected had a better chance for long term survival; Gerry Van der Mieren et al. , reporteIn patients with resectable mediastinal disease, the prognosis seems to be worse than patients with resectable extremity synovial sarcomas, as most of the reported cases of mediastinal synovial sarcomas had local or distant recurrences following complete surgical resection ,18. ThisSynovial sarcoma arising in the mediastinum is a rare disease entity. Complete surgical resection is the only known factor associated with possible long term survival. When arising as unresectable mediastinal mass, the prognosis is extremely poor. Reports of effective downsizing strategies and studies addressing the best integration of chemotherapy and radiotherapy in patients with unresectable mediastinal disease are warranted. Our case can be of benefit to medical oncologists and thoracic surgeons who might be faced with this unique and exceedingly rare clinical scenario.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 axial tomography; EBRT: External beam radiotherapy; FISH: Fluorescence in situ hybridization; PET: Positron emission tomography; SUVmax: maximum standardized uptake value; GCSF: Granulocyte-colony stimulating factor; STS: Soft tissue sarcomas.The authors declare that they have no competing interests.SS wrote the paper, reviewed the literature, constructed the table, cared for the patients, and approved the final version of the manuscript. AA interpreted the imaging studies, assisted in writing the paper, and provided the radiology image. AD performed the FISH study and provided an image and a descriptive legend for the FISH study. MA reviewed the pathology, performed the immunostains, assisted in writing the paper, and provided the pathology image and a descriptive legend. All authors read and approved the final manuscript.


Congenital or true colonic diverticulosis is a rare condition typified by the preservation of the colonic wall architecture within the diverticular outpouching. Cases of multiple jejunal diverticula have been reported as well as cases of solitary giant diverticula of the colon. There have been no reports in the literature of pancolonic congenital diverticulosis. Congenital or true colonic diverticulosis is a rare condition typified by preservation of the colonic wall architecture within the diverticular outpouching. Cases of multiple jejunal diverticula have been reported and are probably secondary to points of focal weakness from the insertion points of vessels into the wall of the colon . Other cA 26-year-old man of Bangladeshi origin presented with a two-day history of generalised abdominal pain, loss of appetite, and nausea. His past medical history includes hypertension, type 1 diabetes mellitus, and autosomal dominant polycystic kidney disease. He is the product of a consanguineous marriage and suffered from intrauterine growth retardation and failure to thrive with persistent short stature and microcephaly. Previous investigations for chromosomal disorders at Great Ormond Street Hospital were negative, and the karyotype was confirmed as 46XY.On examination, he was afebrile and physiologically stable with tenderness in the lower abdomen and a BMI of 14 (height 135.5 cm and weight 25.56 kg). Inflammatory markers were mildly raised . He was admitted for observation, intravenous fluids and antibiotics. A computed tomography scan of the abdomen and pelvis revealed mild thickening of the descending colon but no focal inflammatory changes, free air, or free fluid.The patient deteriorated clinically and underwent a diagnostic laparoscopy, which identified a thickened inflamed loop bowel adherent to the anterior abdominal wall. A laparotomy was performed. There was extensive diverticular disease and an associated inflammation of the entire colon with an inflammatory mass around the transverse colon adherent to the anterior abdominal wall. The diverticula were large and broad based, resembling jejunal diverticulosis macroscopically. Malignancy could not be excluded, and the tissues were extremely friable, so a subtotal colectomy and end ileostomy with abdominal washout were performed. Our patient's postoperative course was complicated by an episode of aspiration pneumonia from which he made a full recovery. Histopathological examination supported the diagnosis of true pancolonic diverticulosis. The entire specimen showed numerous large diverticula and associated inflammation/abscess formation. The majority of the diverticula had a colonic type muscularis propria and can therefore be considered as true diverticula resulting from congenital malformation . Lymph nHe was discharged 13 days postoperatively, with a view to restoration of intestinal continuity in 6 months.Although uncommon, isolated congenital diverticula of the colon have been described in only a small number of case reports , but notWe report the first documented case of generalised congenital colonic diverticulosis affecting the entire colon resulting in an acute presentation.


Plasma cell granuloma, also known as inflammatory myofibroblastic tumour or inflammatory pseudotumour, is a nonneoplastic process characterized by an unregulated growth of inflammatory cells. It most commonly occurs in the lung and upper respiratory tract, and only six other cases of gastric plasma cell granuloma exist. There are no other cases of intragastric laparoscopic resection of this type of lesion. Here, we present a case of a 60-year-old gentleman who had gradual onset epigastric discomfort and was thought to have a gastrointestinal stromal tumour on gastroscopy. Subsequent imaging and laparoscopic transgastric resection of the lesion confirmed the presence of a plasma cell granuloma. We discuss the aetiologies, presentation, investigation, and treatment of this rare disorder and make recommendations on the management. A 60-year-old gentleman presented with gradually worsening epigastric discomfort and weight loss over a six-month period. Due to continuing symptoms following antireflux therapy, an urgent oesophagogastroduodenoscopy was arranged. An ulcerated lesion was demonstrated in the proximal lesser curvature of the stomach, which was thought to be a Gastrointestinal Stromal Tumour due to its appearance (GIST).An urgent double-contrast CT scan of the abdomen was arranged . This deThe patient was discussed in the local oesophagogastric multidisciplinary team meeting, and along with the patients' wishes, primary surgery was suggested as the best option, due to the likelihood of a diagnosis of GIST.An intragastric laparoscopic resection was performed within four weeks of the initial presentation. This was chosen over a proximal gastrectomy in order to preserve the antireflux system of the patient. A 70 × 40 × 20 mm specimen was excised and sent for histopathology Figures . The patThe histopatholgy report demonstrated a good resection margin of over 1 mm. The specimen consisted of mature chronic inflammatory cells, mainly plasma cells and reactive-type lymphoid follicles. Alk-1 immunostaining identified occasional scattered larger mononuclear cells with open nuclei and occasional nucleoli. This was consistent with a plasma cell granuloma, a nonneoplastic polyclonal proliferation of mature plasma cells.The patient is now symptom-free and has had regular follow-up CT scans of his abdomen, and a gastroscopy, which has not demonstrated any recurrence within 18 months of the procedure.Plasma cell granuloma is referred to as several other names in the literature, including inflammatory myofibroblastic tumour, inflammatory pseudotumour, fibrohistiocytoma, and fibroxanthoma. It is a rare disease that is characterised by a nonneoplastic proliferation of mature plasma cells in a fibrovascular background . Tada etThere have been very few cases of gastric plasma cell granuloma, and there are no other cases that have been primarily treated with laparoscopic surgery. It is generally considered a benign condition, with an inflammatory component, but has been reported in association with gastric cancer . Kim et There is very little data on the aetiology, pathogenesis and the most effective treatment for this disorder; therefore, the prognosis of the condition is difficult to predict. The most common treatment for these lesions is complete excision although this is not always possible. Radiotherapy  and sterHere, we present a case of a 60-year-old gentleman with a six-month history of worsening epigastric pain. Due to the possibility of the presence of a malignant lesion, such as a GIST, it was urgently and thoroughly investigated using a combination of noninvasive imaging (double-contrast CT scan of the abdomen) and invasive investigations (gastroscopy and diagnostic laparoscopy). Due to the continuing diagnostic doubt regarding the exact nature of the lesion, an intragastric laparoscopic procedure was undertaken in order to remove the lesion with minimal disruption to organ function. The surprising diagnosis was of a rare gastric plasma cell granuloma. The patient recovered well, with a resolution of symptoms, and no recurrence was found at 18-month follow-up.Nonneoplastic tumours of the stomach are rare and can present with very similar symptoms to malignant lesions, such as epigastric discomfort and microcytic anaemia. The diagnosis should be kept in mind, especially for solitary lesions with no evidence of metastases. Due to the rarity of gastric plasma cell granuloma, each new case should be recorded to allow a better understanding of the condition. We advocate the use of intragastric laparoscopic resection of these lesions in order to provide a diagnosis and avoid complications associated with more extensive surgery, such as bleeding, loss of organ function, and acid reflux disease.


Acute mastoiditis in the newborn is a very rare disease. Herein we report a case of a 28-day-old child with right aural atresia and ipsilateral mastoiditis requiring mastoidectomy. To our knowledge, this is the youngest case reported in the literature. Issues on diagnosis and management of mastoiditis in cases of aural atresia are further discussed. Based on our experience and on previous reported cases we conclude that mastoiditis is difficult to diagnose in a child with aural atresia. Moreover, mastoidectomy may be necessary, although identification of the facial nerve and consequent treatment of the atresia are usually too difficult to perform simultaneously. Congenital aural atresia occurs approximately one in every 20 000 live births. Unilateral atresia is four times more common than bilateral atresia. Abnormalities of the middle and inner ear are associated with atresia in 20–30% of cases , 2.Diagnosis of otitis media in children with congenital atresia is difficult to detect especially when the symptoms mimic very common infections of the upper respiratory system since no otoscopy is possible. In these cases, otitis complication, such as acute mastoiditis, usually reveals the real ear problem.Although acute mastoiditis remains a relatively common clinical problem, it is extremely rare in the newborn. What is more, aural atresia represents a surgically challenging problem, both in technical terms and in relation to the appropriate treatment option, especially in cases of complications. This paper details the rare case of a four-week-old child with acute coalescent mastoiditis in an atretic ear. A 28-day-old female newborn with right congenital aural atresia was referred to our emergency department with postauricular erythema and edema, accompanied by irritability, anorexia, and insomnia, without fever, since two days. On initial examination, the newborn had a small, deformed auricular tag on the right side with no external auditory meatus. She presented with edema, erythema, and tenderness over the right mastoid expanding to the zygomatic bone area . Her lefμL and CRP: 111 mg/L). MRI and high-resolution CT scans (The child had increased inflammation markers (white blood cells: 19.900/CT scans  of the hThe newborn was started immediately on meropenem and vancomycin i.v. Two days later, her condition remained unchanced, and a mastoidectomy was performed. Right temporomantidular joint, zygomatic process of the temporal bone, and the mastoid tip were palpated and used as landmarks for a curvilinear incision over the mastoid bone. Abnormal bone was drilled or curetted away. Mastoid cells filled with granulation tissue were exenterated and about 2 mL of pus were collected for culture Figures . Pus andThe antibiotic scheme continued for a total of four weeks. Her postoperative course was unremarkable, with progressive resolution of signs of inflammation on mastoid area. The baby girl remained afebrile and her alimentation as well as the inflammation markers improved gradually. She left the hospital in a very good condition upon completion of her treatment. The baby has no problem two months after her operation, and her weight is 5.100 gr.The presence of inflammation in the middle ear of atretic ears has been well documented since 1912 by Krampitz  who was In our case, an even younger child required surgical intervention. Despite current reports , 9 indicSurgical treatment is challenging because of the anatomy, the presence of inflammation, and the very young age. Thus, identification of the facial nerve course in the inflamed and underdeveloped temporal bone may not be possible as in our case or in previous cases . In conclusion, whether atresia is related to an increased incidence of ear infection is a subject of controversy and speculation. There are relatively large atresia series showing both increased  and norm


A 61-year-old female was referred to The First Affiliated Hospital, College of Medicine, Zhejiang University  due to a right adrenal tumor. A pre-operative transcutaneous fine-needle aspiration biopsy and right adrenalectomy were performed, and pathological analysis resulted in the diagnosis of pleomorphic rhabdomyosarcoma (RMS). Primary pleomorphic RMS of the adrenal gland in an adult is a rare condition. To the best of our knowledge, this is the first case of pleomorphic RMS of the adrenal gland in an adult diagnosed by light microscopy and immunohistochemical stains. Rhabdomyosarcoma (RMS) is a malignant soft-tissue sarcoma that is believed to develop from primitive totipotent embryonic mesenchyme. RMS is a highly aggressive tumor with a tendency for advanced and disseminated disease early in its course. The condition is the most common soft tissue sarcoma in children. However, RMS in adults is an uncommon tumor that arises mainly in the large skeletal muscles –4. PleomA 61-year-old female was referred to the urological ward of The First Affiliated Hospital, College of Medicine, Zhejiang University for a right adrenal mass that had been detected incidentally by ultrasound examination two weeks previously. The patient had no underlying disease and the physical examination was unremarkable. Abdominal ultrasound revealed a large adrenal tumor. A computed tomography scan revealed a right adrenal tumor measuring 6.0×4.0 cm . The patA pre-operative transcutaneous fine-needle aspiration biopsy was performed and the cytological diagnosis was consistent with a malignant neoplasm. Right adrenalectomy was performed. The tumor was observed to have invaded into the right lobe of the liver, and was well-demarcated from the hepatic parenchyma by a thick fibrous capsule. The total operating time was 3 h. The estimated blood loss was 200 ml . The patient tolerated the procedure well and there were no post-operative complications. The drainage tube was removed at 48 h following the surgery. The patient was discharged on the fifth post-operative day, tolerating a regular diet. Pathological examination of the surgical specimen was pleomorphic RMS containing spindle cells . Immunohet al(et al(et al(In the current World Health Organization Classification of Soft Tissue and Bone Neoplasms, RMS is divided into three distinct subtypes, embryonic, alveolar and pleomorphic . RMS is et al suggest  al(et al reported al(et al reportedIn the present case, light microscopic examination revealed a malignant pleomorphic mesenchymal neoplasm, characterized mainly by the proliferation of atypical spindle cells and few epithelioid cells. Immunohistochemistry revealed positive staining for MyoD1, desmin, vimentin and CD56. By contrast, no expression of SMA, SYN or S100 protein was identified in the tumor tissue. A diagnosis of pleomorphic RMS was confirmed according to the clinical and pathological findings.In conclusion, the present study described a rare case of pleomorphic RMS in the right adrenal region based on the histopathology and immunohistochemistry results. Due to the small number of described cases of adrenal gland RMS, inadequate information is available for evaluating the treatment procedure and the final prognosis of the patient. An accumulation of such cases and an improved understanding of the molecular biology driving RMS tumor behavior are required for further evaluation and research to identify the histogenesis of the condition. Primary pleomorphic RMS of the adrenal gland in an adult is a rare condition. To the best of our knowledge, this is the first case of pleomorphic RMS of the adrenal gland in an adult diagnosed by light microscopy and immunohistochemical staining.


Paratesticular mesotheliomas are very rare tumors. In this paper, we present the management of a 38-year-old male patient with paratesticular malignant mesothelioma who was initially misdiagnosed and treated as recurrent epididymitis. After the final pathology report defining paratesticular mesothelioma during scrotal exploration, he underwent radical orchiectomy and hemiscrotal excision as a complementary, secondary procedure. His metastatic workup did not show any dissemination. Therefore, he did not receive any adjuvant treatment and remained disease-free for more than 2 years. Only a subset of the tumors within the scrotal sac is extratesticular. The paratesticular region, including the spermatic cord, testicular tunics, epididymis and vestigial remnants, is composed of a mixture of epithelial, mesothelial, and mesenchymal elements . With thHerein, we give the clinical details of a patient with paratesticular malignant mesothelioma, who presented with a longstanding history of scrotal pain and swelling and received many medications due to the presumptive misdiagnosis of epididymitis before the final diagnosis of mesothelioma. A 38-year-old male patient presented with left testicular pain and swelling. His past medical history was unremarkable. However, he had similar complaints which also included intermittent swelling for almost 1.5 years, and during this period he received many medications for recurrent epididymitis. Previous imaging findings and serum tumor marker values obtained elsewhere were within normal limits. Physical examination was completely normal on his initial visit in our department, upon which he was recommended to present while he has the scrotal swelling again. The patient represented 1 month later with a significant, soft, noninfectious swelling on the left hemiscrotum, suggesting reactive hydrocele formation. Left epididymis was minimally dilated and sensitive upon palpation. Scrotal ultrasonography demonstrated findings related to hydrocele and left epididymitis. Testicular parenchyma was devoid of any ultrasonographic abnormality. Blood and urine tests, including serum tumor markers, were again within normal limits. Based on these findings, he was scheduled for left hemiscrotal exploration, during which, inflammatory tunical layers together with caput epididymis, which seemed dilated and inflamed, were excised and sent to pathology. Postoperative course was uneventful. He was discharged the following day. Histopathological examination of the excised specimen revealed normal findings for epididymis but epitheloid-type paratesticular mesothelioma  originat After consulting the case with medical oncology and radiation oncology departments, we decided to perform left radical orchiectomy and hemiscrotectomy as a complementary procedure. Chest and abdominal computed tomographies (CT) as well as positron-emission tomography (PET) scans were all negative. After the second operation, he was hospitalized for 6 days due to the development of left hemiscrotal hematoma, which was managed conservatively. Pathological examination of the specimens removed during the second operation confirmed the diagnosis of malignant paratesticular mesothelioma originating from the tunica vaginalis. Although he was offered active surveillance, he asked for a second consultation in an urooncology center elsewhere, at which he was recommended to have a diagnostic laparoscopy to exclude any lymphatic spread after having confirmed the histopathologic diagnosis of paratesticular malignant mesothelioma by second opinion. He had the procedure where all biopsies taken from pelvic lymph nodes were found to be negative. He was then given no additional treatment. The patient is still under our close followup, and he is disease-free for 26 months. Mesotheliomas are relatively rare tumors that arise from the serosal surface of the pleura, peritoneum, and pericardium. On rare occasions, they originate from the tunica vaginalis of the testis in which case they manifest as a paratesticular mass . Since tApart from a past medical history consistent with exposure to asbestos or other natural fibers, preoperative clinical findings usually could not point out the diagnosis of malignant mesothelioma. Firm, painless scrotal mass accompanied by a reactive hydrocele is the usual presentation. The hydrocele or the mass itself may gradually increase in size . The diaSuspected diagnosis should be confirmed with imaging modalities such as ultrasonography and computerized tomography. On ultrasonography, malignant mesothelioma is usually characterized by a hypoechoic hydrocele with heterogeneous masses of increased echogenicity at the periphery . HoweverRadical inguinal orchiectomy is accepted as the optimal treatment for paratesticular malignant mesothelioma . However Although the followup in published series is often short and variable, almost half of the patients have been reported to develop recurrence or metastasis after radical orchiectomy for paratesticular malignant mesothelioma. Local recurrence after orchiectomy is reported in approximately 10% of the patients . MoreoveIn conclusion, paratesticular neoplasms should always be kept in mind while evaluating chronic, recurrent hemiscrotal symptoms. These tumors may not be distinguishable from testicular counterparts based on preoperative findings. However once suspected, interventional measures should be prompted since delays in diagnosing paratesticular tumors may have prognostic implications.


Intraspinal mature teratomas rarely occur in adults. The present study describes an unusual case of adult intradural mature teratoma, which was completely resected. A 22-year-old female presented with an intermittent pinching pain in the lower right shank that had lasted for three months. Magnetic resonance imaging (MRI) results indicated a multicystic mass extending from the T12 to L2 vertebrae, and the tumors were certified as teratomas by a histopathological examination. The level of pain experienced by the patient was improved following the surgery. The present study also compared the literature concerning adult intradural mature teratoma, summarized the basic clinical characteristics and theory of origin of adult intradural mature teratoma and reviewed the available treatments for this disease. Teratomas are a type of multipotential cell tumor that contain a mixture of multiple germinal layers formed by normal organogenesis and reproductive tissues. Based on the degree of differentiation, teratomas may be classified as mature, immature or malignant . The incUnlike in intraspinal teratomas in infants and children, the symptoms of these tumors in adult patients typically lack specific clinical features that, upon diagnosis, may cause confusion with other spinal tumors, such as schwannomas, which are more commonly observed in adult patients . At presA 22-year-old previously healthy female presented with an intermittent pinching pain in the lower right shank that had lasted for three months, progressive lower right extremity weakness and instability while standing. The onset of the shank pain and weakness occurred without any obvious cause. After one month, the patient experienced a shooting pain from the right shank, which traveled towards the right groin, foot and thigh. The patient had not received spinal surgery or any other spinal procedures, and did not complain of lower extremity numbness or urinary incontinence. The physical examinations revealed that there were no motor or sensory deficits in either extremity, and no palpable midline spinal displacement. Upon neurological examination, the patient demonstrated normal physical reflexes and the pathological examinations revealed no cutaneous abnormalities or dermal sinus tracts. In addition, the routine laboratory examinations were normal.The magnetic resonance imaging (MRI) results revealed a lobulated, intradural, heterogeneous, 6.0×1.5×1.7-cm mass between T12 and L2 levels of the lumbosacral spine . The lesThe patient underwent a total resection of the tumor by means of a T12-L1 laminectomy performed under a surgical microscope. Through an incision into the dura, three connected cystic tumors were observed. A portion of the mass was in contact with the medullary and conus medullaries, and a yellow, oval-shaped, fatty cyst extruded to the cauda equina where it had become inseparable. Following the incision into the tumor cyst wall located in the conus medullaris, a white fluid containing hair follicles and gray soft tumor tissue was observed. A histopathological examination of the excised mass revealed the presence of elements from multiple germ cell layers. Under a light microscope, numerous fatty cysts consisting of neuroepithelial and epithelial tissues were observed . The finThe prognosis of the patient, following surgery, was good. No further neurological deterioration was observed during the three-month follow-up period and the leg pain symptoms were relieved subsequent to the surgery.Mature teratomas are a type of benign germ cell tumor, rarely observed in adult patients. The literature concerning adult intradural mature teratoma was reviewed from 1928 to the present date –25, and The incidence of mature intraspinal teratomas in adults is rare, however, certain common features may be noted. Adult patients with mature intraspinal teratomas typically present with a delitescent onset. The literature revealed that, unlike in infants and children, intraspinal mature teratomas in adult patients were rarely observed with vertebral body anomalies or thoracolumbar spinal bifida ,20,26–29There are two dominant theories regarding the origin of intraspinal teratomas. The first is the dysembryogenic theory, and the second is the misplaced germ cell theory ,34. The There is evidence to support the rationale of each theory. To the best of our knowledge, dysraphic malformations are considered to support the dysembryogenic theory. The tridermal anomaly is the primary event of the disordered development of pluripotent cells in the spine, which is likely to further affect the spinal closure . OccurreIn adult intraspinal teratomas, which rarely present with significant dysraphism, the misplaced germ cell theory is likely to be more feasible ,33,38,39The primary treatment for teratomas is surgery, which may also be applied to mature intraspinal teratomas. An epidemiological study of spinal teratomas revealed that the recurrence rates for complete and gross resection were extremely similar  , and thaMature intradural teratomas in adults are rare, with few accompanying spinal anomalies. The currently preferred theory of origin of the disease is the misplaced germ cell theory. A resection of the tumor is the primary treatment methodology for adult patients, as the nature of the tumor is relatively benign and the recurrence ratio is low, even following gross resection. However, radiosurgery is not recommended.


Parotid gland involvement is extremely rare, even in countries in which tuberculosis is endemic. Clinically, it usually presents as a slow-growing mass indistinguishable from a malignancy. On imaging too, tuberculosis of the parotid may mimic neoplasm. The diagnosis of parotid tuberculosis needs a high degree of clinical suspicion. This paper highlights the clinical presentation, imaging findings, and importance of FNAC in diagnosis of this rare entity. A 15-year-old male presented with a gradually enlarging swelling in the left parotid region of one year duration. It was associated with mild pain in this region. He had caries of the upper molar teeth on the ipsilateral side. There was no history of fever or night sweats on presentation. His past history was unremarkable. He gave no history of personal or family history of tuberculosis. Physical examination revealed a mobile lobulated swelling in the region of the left parotid gland. There were no overlying secondary skin changes. There were no visible fistulae or sinuses. Facial nerve function was intact. On palpation, a firm, mobile and nonfluctuant 3 cm area of mass-like enlargement was noted involving the left parotid gland. There was palpable cervical lymphadenopathy. The right parotid area was clinically normal. On investigation, the hemogram including erythrocyte sedimentation rate and chest radiograph were normal.An ultrasound (US) of the left parotid area was advised. On US, the left parotid gland appeared enlarged in size. Few well-defined, hypoechoic oval mass lesions were noted within the substance of the left parotid gland. These lesions were quite obvious against the relatively hyperechoic appearance of the normal parotid parenchyma . The larFine needle aspiration cytology was performed under US guidance. Cytology showed granulomas made up of epithelioid cells and occasional giant cells surrounding caseous type of necrosis. Scattered abscesses were also seen. No malignant cells were detected. The FNAC opinion was chronic caseating granulomatous inflammation of salivary gland consistent with tuberculosis. Tuberculin test was done which turned out to be strongly positive. The patient was advised prolonged antitubercular medication. Tuberculosis is a necrotizing granulomatous disease with varied clinical presentations and a wide distribution. The lungs are most commonly involved. Extra thoracic forms of the disease account for approximately 20% of overall active tuberculosis and can be seen in the kidneys, bones, meninges, and lymph nodes . AlthougInvolvement of the parotid gland and lymph nodes may develop in two ways. First, a focus of mycobacterial infection in the oral cavity liberates the mycobacterium that ascends into the salivary gland via its duct or pass to its associated lymph nodes via lymphatic drainage. The second pathway involves haematogenous or lymphatic spread from a distant primary lung focus . TubercuTuberculosis of the parotid gland presents with difficulties in diagnosis because of the similarity of the presentation to that of a neoplasm. Diagnosis mainly relies on the treating physician having a high index of suspicion. Kant and associates in 1977 reported 35 cases ; Yaniv aRadiological investigations including US, CT, and MRI are sensitive in detecting intraparotid tubercular lesions, however, findings are not specific. Most of the imaging findings mimic neoplasm. A chest radiograph may be helpful in cases of associated pulmonary tuberculosis. The chest radiograph of our patient was normal. US may well delineate the lesion however the echotexture has been variably described in literature most commonly being hypoechoic as in our case. The colour doppler findings within the lesions are also not specific and may range from avascular to highly vascular lesions. CT and MRI are useful to define the extent of the lesion and detect any concomitant deeper lesions. Again the findings on CT and MRI are highly sensitive however not specific and may be indistinguishable from neoplasm. In patients presenting with unilateral parotid nodules, tuberculosis should be considered when linearly arranged enhancing nodules are demonstrated in the superficial lobes of the glands on CT scan . On MRI,Establishment of diagnosis of tuberculosis requires histological confirmation since imaging findings are not specific. Tuberculous disease is impossible to distinguish clinically from other diffuse inflammatory disease of the salivary glands if a culture of the glandular secretions from the Stenson's duct or saliva are negative for AFB . In paroIn our patient, the most probable source of infection for the parotid gland was via the Stenson's duct from the oral cavity since he had molar caries in the ipsilateral side and no other tubercular focus could be established elsewhere in the body. The diagnosis of tuberculosis of the parotid was clinched due to FNAC of the intraparotid lesions under US guidance. CT or MRI was not indicated in this case. Our case is unique as most previously described case reports of tuberculosis of the parotid have been achieved post surgery on parotidectomy specimens where initial FNAC had failed to achieve diagnosis. In our case since the FNAC was conclusive, surgery was avoided, and management was conservative. This highlights the importance of ancillary investigations like FNAC in such cases. Although rare, tuberculosis should be kept in mind and considered in the differential diagnosis of any patient presenting with a solitary swelling in the parotid gland to avoid unnecessary surgery .


Leiomyomatosis peritonealis disseminata (LPD) are multiple nodules adherent to and superficially invading the peritoneum, mimicking metastatic ovarian carcinoma. Their origin would be metaplastic mesenchymal cells (MC), which in susceptible women MC could originate from surgical myoma fragments . EstrogeWe report the first case of successful management of LPD with ulipristal, a selective progesterone receptor modulator (SPRM), registered for preoperative management of uterine myoma , 6. All A 21-year-old nulliparous woman presented with abdominal discomfort coinciding with a pelvic mass stretching beyond the umbilicus. MRI revealed multiple nodules, some with central necrosis, all over the abdomen, apparently not originating from any abdominal organs. She had a hysteroscopic FIGO1 myoma (3-cm diameter) resection 6 months earlier, and 3 years before, a breast fibroadenoma had been removed. Laparotomy disclosed multiple implants over the peritoneal surface and bowels and their meso. Pathology of several biopsies revealed leiomyomatosis, strongly expressing progesterone, but not estrogen receptors. She was first given goserelin acetate 3.6 mg/month (Zoladex®) and tibolone 2.5 mg/day  with an objective response on MRI after 3 months. This was continued for 2 years while also on a contraceptive Cu-IUD. The therapy was discontinued because of reassuring images, but she developed menorrhagia and a symptomatic benign ovarian cysts (diameter = 6 cm). Two months of nomegestrol acetate 5 mg/day (Lutenyl®) rested the menorrhagia; however, LPD seemed progressive on MRI (Fig. We believe that this first use of ulipristal for LPD was successful because lesions were expressing progesterone receptors. The subjective and objective responses were striking. Its effect is underscored by a proven reduction in size of lesions and their regrowth following abortion of ulipristal. Though asymptomatic, the disappearance of LPD was incomplete. In addition, she wishes to conceive, so debulking surgery was offered, though the patient reports no side effects, which sharply contrasts with her previous use of GnRH agonists. There is little known on prolonged ulipristal administration. Endometrial atypical hyperplasia may be a concern, though never described for ulipristal. Endometrial biopsy after 12-month exposure is however reassuring. In conclusion, ulipristal acetate may be considered as an adjunct in the management of women with LPD.


Background: Primary intra cardiac tumors are rare. In this article, we present papillary fibroelastoma of mitral valve chordae.Case Presentation: A 35-year old man presented with atypical chest pain and palpitation. Physical examination and electrocardiogram were normal. Transesophageal echocardiography (TEE) revealed a mass of 1015 mm attached to chordae of anteromedial papillary muscle of mitral valve. The tumor was completely resected and the mitral valve chordae tendineae was preserved successfully. The pathological diagnosis was papillary fibroelastoma.Conclusion: In any patient with atypical chest pain and palpitation, valvular tumor should be considered in differential diagnosis. Primary intra cardiac tumors are rare with a prevalence ranging from 0.0017 to 0.28% in various autopsy series and account for an only 0.3% of all open heart operations . PapillaA 35-year old man was referred to our department for the evaluation of atypical chest pain and palpitation. The physical exam and electrocardiogram were normal and routine blood laboratory tests were unremarkable. Transthoracic echocardiography showed a mobile mass on mitral valve. However, the aortic valve and cardiac chambers were normal. There was no regurgitation of the mitral valve, and ejection fraction was normal. Transesophageal echocardiography (TEE) revealed a mass of 1015 mm attached to chordae of anteromedial papillary muscle of mitral valve . Blood culture, sedimentation rate, and testing for thrombophilia disorders were not remarkable. Based on its place and appearance, the working diagnosis of a cardiac papillary fibroelastoma was made. The patient underwent surgical resection of this mass. The heart was exposed through a median sternotomy. After appropriate cannulation, cardiopulmonary bypass was started, and then the mitral valve was reached through a trans-septal approach. The multilobular yellowish mass was seen attached to chordae of the anteromedial pappilary muscle of the mitral valve . Tumor wThe tumor was attached to three chordae tendinaes and was not pedanculated. The patient had a good weaning from the cardiopulmonary bypass and recovered well in the post-operative period. The postoperative TEE showed absence of residual tumor and normal mitral valvular function and there were no findings of mitral valvular regurgitation. The histological findings of the tumor were compatible with papillary fibroelastomas . After 7Primary intra-cardiac tumors are rare with a prevalence ranging from 0.0017 to 0.28% in various autopsy series -14. TherGenerali et al. reported pulmonary valve papillary fibroelastoma (Hakim et al. presented papillary fibroelastoma of the pulmonary valve . Zhang eelastoma . Surgicaelastoma . In this paper, we report the uncommon location of a PFE without valvular involvement. Resection of these tumors might lead to mitral regurgitation, and heart failure is unavoidable in some valvular and chordae tendinaes involvement as in some previous cases, therefore, valvular repair or replacement may be required. In our case, although the tumor was attached to three chordae tendinaes and was not pedanculated, the tumor was completely resected from mitral valve chordae with sharp dissection. Fortunately, chordae tendinae were preserved successfully without destruction. After 7 months follow-up, our patient had no symptoms and at last TTE showed normal chambers with normal mitral valve without regurgitation or recurrence of tumor. In conclusion, our patient was presented with atypical chest pain and palpitation. We suggest more attention and echocardiographic assessment for evaluating these common symptoms especially in young patients, although these signs and symptoms occurred more often after common ischemic heart diseases.


This paper describes a case of ovarian metastasis from lung carcinoma along with its diagnostic challenges, clinical management, and review of the literature. A 49-year-old woman was admitted to our emergency department with complaints of abdominal pain and vomiting. A laparoscopic appendectomy was performed due to acute appendicitis, and a unilateral oophorectomy (left side) via laparoscopy was performed due to the detection of an ovarian mass. Immunohistochemical staining of the ovarian mass revealed that it was reactive to cytokeratin-7 (CK-7) but negative for CK-20. The immunohistochemical and pathological features of the tumor indicated an ovarian metastasis of non-small-cell lung cancer. The patient underwent chemotherapy and was followed up by the oncology department. Her postoperative regular followup of 6 months showed that her condition was stable with no recurrence. The management of female patients with acute abdominal pain and pelvic masses should consist of a multidisciplinary approach to include the diagnosis of any distant organ metastasis. The majority of adnexal masses are functional cysts, endometriomas, teratomas, cystadenomas, leiomyomas, and malignant tumors . HoweverThe present paper is the first to describe an incidental diagnosis of bronchoalveolar carcinoma in a patient with adnexal mass presenting with acute abdominal pain.A 49-year-old woman was admitted to our emergency department with complaints of abdominal pain and vomiting. She had no vaginal bleeding or discharge, diarrhea, fever, or constipation, and her medical history was unremarkable. Physical examination revealed tenderness and guarding at the right lower abdominal quadrant. An abdominal ultrasonography revealed a multiloculated 3 × 4 cm complex cyst in the left ovary and was positive for acute appendicitis. Subsequently, a laparoscopic appendectomy was performed by general surgeons; the appendix was found to be acutely inflamed and nonperforated. After an intraoperative gynecological consultation, a laparoscopic left oophorectomy was performed, and the tumor mass was removed using an endobag. To identify any possible ovarian malignancy, tumor markers were evaluated, and the results were as follows: CEA, 15.1 ng/mL ; CA 125, 49.5 ng/mL (0–35 ng/mL); CA15-3, 42.1 ng/mL (0–31 ng/mL); and CA 19-9, 76 U/mL (0–35 U/mL). On the basis of the pathology report, the tumor mass was diagnosed to be a well-differentiated adenocarcinoma of the left ovary Figures , but wern = 3), synchronously with (n = 3), or less than 1 year after (n = 1) the diagnosis of primary lung cancer. Young and Scully also pointed out that the clinical characteristics and pathological features of the disease may be useful in the diagnosis of ovarian metastasis from lung cancer. Irving and Young [Malignancy is one of the most common causes of death, and most patients die of complications of the metastatic disease. Bronchogenic carcinoma is the leading cause of tumor-related deaths in developed countries . Adenocand Young  reportednd Young . In our case, the patient was 49 years old and was admitted to our emergency department with acute abdominal pain. None of her symptoms reflected any pulmonary disease. In addition, accurate preoperative evaluation could not be performed due to the emergency conditions. However, laparoscopic surgery and incidental diagnosis of the primary disease sets this case apart from the rest. In recent years, immunohistochemistry studies with CK-7 and CK-20 staining have been widely used to differentiate between primary and secondary ovarian malignancies. Yeh et al. were the first to point out the importance of immunohistochemical staining . In the The management of abdominal masses should include a multidisciplinary approach. Female patients likely to have a pelvic mass should be referred to a gynecologist before deciding on any abdominal surgeries. Radiological findings correlating with those of pathological and immunohistochemical staining are required for the diagnosis of metastatic ovarian tumors.


Cystadenolymphomas (Warthin's tumors) are the second most frequent lesions of the parotid gland. Due to their benign clinical behavior, the low rates of recurrence and malignant transformation they were classified as tumor-like lesions. In addition, a polyclonal growth of the epithelial components of the tumor could be detected. Warthin's tumors occur bilateral in 7-10%, whereas a multifocal appearance is extremely rare. Even if the pathogenesis is still unclear a heterotopia of salivary tissue during embryogenesis is the most likely explanation for the origin of these tumors in the upper neck and periparotideal region. Here we present a rare case of bilateral, multifocal, extraglandular Warthin's tumors in lymph nodes of the upper neck and give a brief review of the literature. If a primary malignancy can be excluded by a careful staging procedure prior to the operation an isolated excision of the lesions of the neck is the adequate treatment. Bilath decade ,5. Histoh decade . Although decade  these leh decade ,5,8. Theh decade ,9.There are several theories to explain the origin and the development of the tumors. Only two of the theories remain, namely the hypothesis that the tumor is an adenoma with concomitant lymphocytic infiltration , and secThere are only a few reports on the occurrence of extraglandular Whartin tumors, especially with a bilateral and multifocal appearance ,14-16. HA 85-year-old Caucasian man presented to our department in 2011 with a 4-year history of swelling on the mandibular angle of both sides. After initial slow and continuous growth, the masses now showed an accelerated enlargement in the last months, he indicated.In 2004 the patient had suffered from a carcinoma in situ of the right auricle that was treated by photodynamic therapy alio loco. At that time neither ultrasound nor a CT-scan showed any cervical masses. Follow-up care did not reveal any local recurrence of the carcinoma. In 2007, there had already been detected two enlarged lymph nodes of unknown dignity on the left (2.4 cm) and right 1.4 cm) angle of the mandible by ultrasound examination. The performed CT-scan classified the lymph nodes unspecific with a diameter of 1.6 cm on both angles of the mandible. During follow up, ultrasound was performed twice a year and showed no further enlargement of the lymph nodes. In the year 2011 ultrasound detected a mass on the left side of 3.5 cm and two masses of 1.5 and 1.6 cm on the right side, respectively. Due to their appearance they were classified as suspect lymph nodes. CT-scan paralleled these findings of suspect lymph nodes on both sides of the mandible . The masses showed a defined border and an uptake of the contrast medium (Figure .4 cm angThe patient was a non-smoker. He reported no pain, facial or trigeminal disorders. B-symptoms, a stay abroad and the presence of domestic animals were denied. Furthermore, the patient suffered from coronary heart disease, cardiac arrhythmia, barrett esophagus and hypertension.Physical examination in our department showed soft, painless solid masses at the left and right angle of the mandible which were moveable to the skin and the deeper tissue. Ultrasound revealed two round-ovoid-shaped nearly fused lymph nodes in the left regio IIA cranial to the bifurcation of 16 and 15 mm in the maximum diameter, respectively. No vessels could be detected inside the lymph nodes, the regular lymph-node anatomy with hilum and cortex was absent. The internal echo was increased with interspersed hypoechoic areas. The borders of the lymph nodes were widely well-defined and they displayed a dorsal sound-enhancement. A very similar lymph node was found in the right regio IIA which measured 18 mm in the maximum diameter Figure . Thus, tTherefore, the patient underwent a selective lymph node excision on both sides in general anesthesia without any complications. Histological examination revealed oncocytic benign epithelial cells and mixed lymphoid cells consistent with Warthin's tumors in three cervical lymph nodes Figure . On the The patient recovered from the operation very soon and showed no signs of recurrence during follow up so far.The first description of adenolymphoma was done by Hildebrand in 1895  and in 1Beside the epithelial component these tumors consist of a stromal component with lymphocytic infiltrates and lymph follicles. One hypothesis about the origin of these lymphatic components is the proposal of Low et al., namely that the lymphatic component is a result of an immunological reaction to the epithelial growth . This suSince Honda et al. were able to prove that the epithelial components are polyclonal cell populations this lesion cannot be classified as a true neoplasia anymore . AccordiThe sonographic diagnosis of neck masses originating from heterotopic salivary gland tissue remains challenging due to their rarity. In the present case, first of all, metastastic tumor growth had to be taken into account, because the whole lymphatic tissue was depleted of the lymph nodes. Moreover, the simultaneousness of the occurrence of three similar bilateral lesions suggested the presence of a metastasizing malignant tumor. The absence of detectable intra- or perinodal vessels and the hyperechoic appearance of the presumptive metastatic tissue rather argued against this assumption, but histopathological investigation of the entire lesions was the only way to establish the correct diagnosis.The management of neck masses secondary to heterotopic salivary tissue is complex and depends among other things on the dignity of the lesion. Due to the fact that the definitive diagnosis can finally be achieved by a histological examination it is still a challenge for the surgeon to balance the surgery appropriate. Unnecessary surgery of the salivary glands with its associated morbidity or missing a primary malignancy should be avoided. Therefore a careful search for any primary cancer should be performed prior to the operation as recommended by Ferlito et al. . In the We presented a rare case of a multifocal bilateral cystadenolymphoma in upper cervical lymph nodes, the most frequent lesion of heterotopic salivary gland tissue. Even if there are several hypotheses the pathogenesis is still unclear. Recent studies were able to proof that these tumors are no true neoplasia and consequently were classified as tumor-like-lesions. The preoperative diagnosis to exclude primary malignancies and consequently the definition of a balanced surgery is still a major challenge for the surgeon. Careful clinical examination, radiography, ultrasound and in some cases fine-needle aspiration cytology should be performed. If a primary malignancy can be excluded the adequate therapy of these lesions is an isolated excision of the masses. In these cases surgery of the salivary glands with its morbidity should be avoided. Afterwards a close follow up is recommended.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.CN and CS wrote the paper, CS und DDS performed the operation, CS performed the ultrasound examination, SH did the histopathologic examinations, DDS collected the clinical data, RD, NRK and JH corrected and improved the paper. We confirm that no prior or duplicate publication or submission elsewhere of any part of the work has taken place. All authors have read and approved the final manuscript.