Composite pheochromocytoma/paragangliomas are very rare tumors composed of ordinary pheochromocytoma paragangliomas associated with neurogenic tumors. Several hereditary susceptibility disorders are known to be associated with pheochromocytoma/paragangliomas such as multiple endocrine neoplasia type 2 (2A or B). To the best of our knowledge, only four cases of composite pheochromocytoma/paragangliomas associated with multiple endocrine neoplasia type 2 have been reported.RET germline mutation.A 40-year-old Arabic woman presented with headache, palpitations, paroxysmal hypertension, and weight loss, which she had had for the last 3 years. She had a familial history of diabetes and multiple endocrine neoplasia type 2. A radiological examination revealed thyroid lesions and bilateral adrenal medulla tumors. Our patient had undergone bilateral adrenalectomy, total thyroidectomy with cervical lymphadenectomy, and parathyroidectomy. A pathological examination confirmed the multiple endocrine neoplasia type 2A consisting of left medullary pheochromocytoma, right medullary composite pheochromocytoma-ganglioneuroma, medullary carcinoma of the thyroid with lymph node metastasis and parathyroid hyperplasia. A genetic analysis also revealed that our patient had a Composite pheochromocytoma/paraganglioma associated with multiple endocrine neoplasia type 2 is a very rare occurrence, as the current literature provides only a few cases. Further reported cases are needed in order to understand the behavior and the pathogenesis of this uncommon entity. In 1998, Matias-Guiu et al. reported another case of CP in a 49-year-old man with MEN 2A. The two reported cases were located in the left adrenal medulla and the neurogenic components were ganglioneuroma and ganglioneuroblastoma, respectively. Cases associated with MEN 2B have also been reported. Charfi et al. reported in 2008 the first case of CP associated with MEN type 2B. More recently, in 2016, Yamasaki et al. reported a case of a composite retroperitoneal paraganglioma in a 59-year-old man.Composite pheochromocytoma/paraganglioma (CPC/PG) is a rare entity composed of classic PC/PG associated with other neurogenic tumors such as neuroblastoma, ganglioneuroblastoma, ganglioneuroma (GN), or malignant peripheral nerve sheath tumors (MPNST) . In the We report here an additional case of CPC in a 40-year-old woman with MEN 2A. We discuss the clinicopathological issues and perform a review of the literature in order to improve understanding of this very rare type of tumor.RET proto-oncogene was performed at our hospital’s medical genetics department. The test was carried out on our patient’s peripheral blood. Her genomic deoxyribonucleic acid (DNA) was amplified by using polymerase chain reaction (PCR) and oligonucleotide primers for exon 11. A direct sequencing analyses showed a RET (rearranged during transfection proto-oncogene) germline mutation at exon 11 (codon 634), with a substitution of tyrosine by a cysteine residue (C1900T→C (C634R).A 40-year-old Arabic woman presented with headache, palpitations, paroxysmal hypertension and weight loss, which she had had for 3 years. She had a history of treated type 2 diabetes. She had also a familial history of type 2 diabetes and MEN 2A. Her sister had undergone thyroidectomy and adrenalectomy for medullary carcinoma of the thyroid and pheochromocytoma. A physical examination showed an enlarged and firm thyroid mass which moved with swallowing, without cervical adenopathy. Our patient had no hypertension on admission. A cervical ultrasound scan found nodular heterogeneous calcified and cystic lesions of the thyroid gland, the larger lesion was located at the left lobe and measured 1.8 × 1.3 cm. An abdominal computed tomography (CT) scan revealed a left adrenal mass and a well-encapsulated nodule of the right adrenal gland  with lymph node metastasis, and parathyroid hyperplasia. Our patient recovered well after surgery and had no signs of disease 3 years after treatment.Grossly, the resected right adrenal specimen measured 9 × 6.5 × 5 cm. The tumor was well-encapsulated, firm, and the cut surface was yellow-brown, with areas of hemorrhage Fig. .Fig. 2ThThe left adrenal specimen measured 13 × 8 cm. The cut surface of the tumor was light tan and yellowish, with hemorrhagic changes Fig. .The left lobe of the resected thyroid gland measured 4.5 × 3.5 × 1.5 cm; the right lobe measured 5 × 3 × 3 cm and the isthmus measured 2 × 1.5 cm. A 1.7 × 1.2 cm nodule was found in the middle third of the left lobe; it was fleshy, grayish-white, and ill-defined. Cystic and fibrous degeneration were seen in the remaining parenchyma. The right lobe was enlarged with multinodular colloid structures with cystic degeneration.The resected parathyroid gland measured 2.4 × 1 × 0.5 cm, with no macroscopic lesion identified.Lymphadenectomy consisted of fibroadipocytic fragments measuring from 0.5 cm to 3 × 2.5 cm, containing several lymph nodes.A histologic examination with routine hematoxylin-eosin staining (HES) of the right adrenal resected specimen showed tumor cells arranged in trabecular and anastomosing cords. The cells were polygonal, spindle or oval-shaped with a moderate amphophilic cytoplasm. The nuclei were round to spindle-shaped with inconspicuous nucleoli. Some bizarre nuclei were found with pseudoinclusion Figs.  and 4. BThe microscopic aspect of the left adrenal resected specimen was similar to the pheochromocytoma component seen in the right adrenal specimen.At immunohistochemistry, the cells of the pheochromocytoma component were strongly positive for synaptophysin and chromogranin, but negative for neurofilament Fig. . The ganMicroscopically, the nodule of the left lobe of the resected thyroid gland showed sheets of polygonal cells with amphophilic cytoplasm and oval nuclei with granular chromatin and inconspicuous nucleoli. Amyloid deposits were seen as pink amorphous globules within the tumor sheets Fig. .Fig. 7ThTumor cells stained positive for synaptophysin and chromogranin, but negative for thyroid transcription factor 1 (TTF1) Fig. .The histologic examination of the parathyroid gland showed hyperplastic chief cells with reduced adipose tissue Fig. .The lymphadenectomy consisted of 26 lymph nodes with two metastatic lymph nodes from medullary carcinoma of the thyroid. The tumor was classified as pT1bN1b (AJCC 2009).Composite pheochromocytoma/paragangliomas (CPC/PG) are uncommon tumors combining the histologic features of the ordinary PC/PG with those of ganglioneuroma, ganglioneuroblastoma, neuroblastoma or peripheral nerve sheath tumors . Paraganet al. was composed of approximately equal parts of PC and PG [et al. reported the absence of N-myc amplification in four cases of CPC, and concluded that CPC does not have adverse prognostic significance conferred by the neuroblastic elements [Macroscopically, CPC/PG have similar features of ordinary pheochromocytoma, presenting generally, as solid and firm tumors, the cut section appearing gray-white, light tan, or dusky red , 12. TheC and PG . The PC C and PG , 7. The C and PG , 12. Theelements .et al., CPC/PG are similar clinically and radiologically, and their management should be so [Usually, CPC/PG are functional tumors, clinical symptoms are related to the type of hormones produced by the component of the tumor. Symptoms range from palpitations, headache, hypertension, and excessive sweat. These signs are due to excessive production of epinephrine or norepinephrine. Cases presenting with watery diarrhea have been described attributable to increased production of vasoactive intestinal peptide (VIP) . Accordild be so .RET proto-oncogene, von Hippel–Lindau disease tumor suppressor (VHL), neurofibromatosis type 1 tumor suppressor (NF1), genes encoding the succinate dehydrogenase (SDH) complex subunits, TMEM127, and MYC-associated factor X (MAX) [PC/PG, ordinary or composite types, are usually associated with certain hereditary disorders: von Hippel-Lindau syndrome (vHL), multiple endocrine neoplasia syndrome type 2A and 2B (MEN 2A and 2B), NF1 and the recently described familial paraganglioma syndrome . Several X (MAX) .et al. was in fact a postmortem diagnosis on autopsy as the patient had died after an orthopedic surgery [RET germline mutation on the exon 11 at the codon 634, like our patient. In fact the vast majority of patients with MEN 2A carry a RET germline mutation on exon 10 or 11 [RET proto-oncogene [et al. had a composite retroperitoneal paraganglioma with a M918T germline mutation of the RET proto-oncogene [et al. had a retroperitoneal CPG. Our patient had bilateral pheochromocytomas with a CP at the right adrenal medulla. The neurogenic components of CPC/PG are, in decreasing frequency: ganglioneuroma, ganglioneuroblastoma, neuroblastoma, malignant peripheral nerve sheath tumors (MPNST), and neuroendocrine carcinoma [To the best of our knowledge, only four cases of CPC/PG associated with MEN 2 have been reported –9 , like our current patient, should be treated by surgery and their first-degree relatives should be offered genetic counseling and genetic testing for RET germline mutations [The clinical course of CPC/PG is not clearly defined because of the rarity of these tumors. Patients usually present with clinical signs related to hormone hyperproduction, such as headache, palpitations or hypertension. It has been reported in the literature that the prognosis of composite tumors with neuroblastoma (NB), ganglioneuroblastoma (GNB) and MPNST is worse, and the clinical behavior is determined by these components . The behfrom MCT , 9. In gfrom MCT . Recentlfrom MCT . In factutations . In factCPC/PGs are very rare tumors and are supposed to have similar clinical behavior of ordinary pheochromocytoma/paraganglioma. The association with MEN 2 is uncommon. We have described here a case of a patient with CPC associated with MEN 2A, together with a review of the literature. Less than 100 cases of CPC/PG have been reported, emphasizing the need for further studies in order to know more about the epidemiological, diagnostic, and prognostic aspects of these rare tumors.


The patient underwent a pancreaticoduodenectomy with lymph node dissection including all cellular adipose tissues of the hepatic pedicle from the hepatic common artery and of the retroportal lamina. Histological findings were suggestive of a duodenal embryonal carcinoma with pancreatic infiltration. This is the second published case highlighting the duodenal primitive localisation of an embryonal carcinoma with pancreatic infiltration.We report here the case of a young man suffering from a rare germ cell tumour. The patient was a 25-year-old man who was referred to our centre for asthenia, stinging epigastric pain, and an iron deficiency anaemia. Gastroscopy revealed a circumferential vegetating lesion on the second portion of the duodenum. The lesion was indurated at the third portion of the duodenum, responsible for a tight stenosis. A computerized tomography-scan of the chest, abdomen and pelvis, and a pancreatic MRI showed a circumferential lesion with a bi-ductal dilatation ( Core tip: Duodenal embryonal carcinoma is a rare germ cell localisation. This lesion may be revealed by a chronic or acute haemorrhage. Our patient presented with an iron deficiency anaemia associated with asthenia and epigastric pain. Imaging studies and endoscopy showed a tight stenosis of the third portion of the duodenum with a circumferential lesion responsible for a common bile duct and Wirsung’s duct dilatation without any metastatic localisation. The patient underwent a pancreaticoduodenectomy and histological findings helped to identify a duodenal embryonal carcinoma with pancreatic infiltration.In young men, aged between 15 and 35, testicular cancer is the leading cause of neoplasia, with an incidence rate of 2.1 for 100000[Here, we reported the case of an embryonal duodenal carcinoma with pancreatic infiltration.A 25-year-old Bulgarian man was referred to our centre by his regular medical doctor for a strong asthenia, which lasted for the past 3 wk, and stinging epigastric pain, which was paroxysmal with dorsal irradiation responsible for nocturnal awakening getting worse since 1 mo.The patient had neither lost weight recently nor did he present with anorexia.Apart from a moderate active smoking, he did not have any significant surgical and medical history.Clinical examination showed that the patient has a body mass index of 19. Examination of the abdomen revealed an epigastric sensitivity without any abdominal mass. Bowel movements were regular; however, dark stools were noted for the last week. A digital rectal exam revealed neither mass nor blood. The patient was afebrile and presented with a marked skin pallor.Biologically, the patient presented with haemoglobin at 6.8 g/dL in relation with an iron deficiency anaemia . For this reason he was transfused 4 units of packed red blood cells as soon as he was admitted to our department. There was no inflammatory syndrome (leukocytes: 8.88 Giga/L and C-reactive protein: 22.3 mg/L). Liver function test results showed a cytolysis  as well as an anicteric cholestasis . Lipase was at 2591 U/L and quickly decreased. Tumoural markers were measured: carcinoembryonic antigen: < 1 μg/L; carbohydrate antigen 19-9: 17.2 kU/L; alpha foetoprotein: 2.1 μg/L; β human chorionic gonadotropin < 3 UI/L; lactate dehydrogenase: 117 U/L.Rectoscopy performed until 40 cm from the anal margin did not show anything specific. Gastroscopy has revealed a circumferential vegetating lesion with a villous appearance on the second portion of the duodenum Figure . This leA computerized tomography (CT) of the chest, abdomen and pelvis showed a circumferential lesion thickening of up to 2 cm at the level of the second and third portions of the duodenum with a bi-ductal dilatation (of the common bile duct and main pancreatic duct (Figure After a multidisciplinary meeting, pancreaticoduodenectomy was decided. Surgery was performed by RM. Intraoperatively, examination of the abdominal cavity did not find any peritoneal carcinomatosis or any other tumoural lesions. Picking analysis of para-aortic lymph nodes and two frozen section examination at the level of the upper part of the mesenteric pedicle did not help to identify any sign of malignancy. A pancreaticoduodenectomy was performed with a pancreaticogastric anastomosis and a hepatico-jejunal anastomosis. Lymph node dissection included all cellular adipose tissues of the hepatic hilum from the common hepatic artery and the retroportal lamina Figure . HistoloIn order not to undermine a primitive cancer, a testicular ultrasound and CT-scan of the head and neck were performed without any repeat finding of the tumoural lesion. The case was presented to the oncology staff in order to put forward an adjuvant chemotherapy after cryogenic sperm preservation.Melanoma, renal, mammary, bronchopulmonary, gastric or testicular neoplasia represenA primitive duodenal tumour is a hypothesis which must be discussed because clinical and imaging examinations have not led to the identification of another primitive lesion. A duodenal primitive embryonal carcinoma was already described in a young man. The hypRegarding tumour markers, high rates of β human chorionic gonadotropin are frequently described with non-seminomatous tumours, particularly choriocarcinomas. The alpha foetoprotein seems rather ascending in case of a non-seminomatous lesion, particularly embryonal carcinomas with a yolk sac differentiation. We did In conclusion, the onset of a strong anaemia in a young man requires endoscopic and imaging explorations in order to search for an embryonal tumoural lesion on the digestive tract since they are very frequently revealed by a chronic or acute haemorrhage.A 25-year-old Bulgarian man was referred to our centre for a strong asthenia, and stinging epigastric pain getting worse since 1 mo.Clinical examination showed that the patient has a marked skin pallor with epigastric sensitivity without any abdominal mass nor recent weight loss or anorexia.A slightly differentiated adenocarcinoma of biliopancreatic origin or another germ cell tumour.The patient presented with haemoglobin of 6.8 g/dL in relation with an iron deficiency anaemia, associated with a cytolysis as well as an anicteric cholestasis.Radiological exams showed a circumferential lesion thickening of up to 2 cm at the level of the second and third portions of the duodenum with a bi-ductal dilatation without any secondary lesion.Histological findings were suggestive of a duodenal embryonal carcinoma with pancreatic infiltration associated with, in immunohistochemical staining, a specimen cells’ expression of cytokeratin 7, CD30 and SALL4.After a multidisciplinary meeting, pancreaticoduodenectomy was decided and performed.This is the second published case highlighting the duodenal primitive localisation of an embryonal carcinoma with pancreatic infiltration.A pancreatic tumour in a young man may not be of biliopancreatic origin and requires extensive investigations to not ignore the uncommon entities as a germ cell tumour.This is an important case report of a most unusual embryonal tumour involving the duodenum and adjacent pancreas and needs publication.


Intrahepatic biliary cystadenomas are rare hepatic neoplasms, which are usually cystic. These tumors are often misdiagnosed as simple liver cysts and hydatid cysts clinically and radiologically owing to nonspecific clinical and radiologic features. These tumors require complete resection, as recurrence and malignant transformation can occur following incomplete excision. It is essential that these tumors be diagnosed accurately so that they can be adequately excised.Clinical and radiological features of six cases of biliary cystadenoma are described.All of these cases were resected with the clinical and/or radiological impression of simple liver cysts and/or hydatid cysts. Out of the six patients, five were female and one was male. Ages of the patients ranged from 28 to 60 years (mean 45 years). The patients presented with nonspecific symptoms. Internal septations were seen on preoperative imaging (when available). On gross examination, all tumors were cystic; their sizes varied from 5.5 to 14 cm, mean size was 9.0 cm. On histopathologic examination, cystic spaces were lined by cuboidal to columnar mucin-secreting epithelium with underlying ovarian-type stroma. In one case, ovarian-type stroma was not seen. Recurrence was seen in three cases at 1 to 5 years of follow up.Owing to their malignant potential and high recurrence rate following incomplete resection, an aggressive surgical approach is recommended. Prognosis is excellent after complete resection. Intrahepatic biliary cystadenomas are rare cysts forming hepatic neoplasms, which are usually multilocular. Clinical, radiological, and histological features are nonspecific and often lead to misdiagnosis. Some tumors may be unilocular. Cysts are usually thin walled and filled with thin watery fluid. In some cases, the cyst wall may be thickened, filled with thicker mucinous fluid and may show papillary projections. They range in size from 2.5 to 28.0 cm. On histopathologic examination, the cystic spaces are lined by cuboidal to columnar mucin-secreting epithelium and ovarian-type stroma is seen subepithelially , 2. HoweHere we present six cases of biliary cystadenoma diagnosed in the Section of Histopathology, Aga Khan University Hospital (AKUH), Karachi, over a 7-year period from 2008 to 2015. All of these cases were resected with the clinical and radiological impression of simple liver cysts or hydatid cysts.We searched the Surgical Pathology files of the Department of Pathology and Laboratory Medicine, AKUH, for cases reported as intrahepatic biliary cystadenomas. The principal authors (NU and ZA) reviewed the slides of all six cases reported as intrahepatic biliary cystadenomas. The clinical and follow-up data were obtained from medical records, hospital discharge summaries, and by telephone inquiry. All available hematoxylin and eosin (H&E), histochemical stains, and immunohistochemical (IHC) stained slides were reviewed and reassessed.A total of six cases of intrahepatic biliary cystadenomas were reported between 2008 and 2015. Five patients were from Pakistan  and one patient was Afghan from Afghanistan. Out of the six cases, five were diagnosed in females and one was diagnosed in a male. Ages of the patients ranged from 28 to 60 years. Mean and median ages were 45 and 43 years respectively. All patients presented with abdominal pain and swelling. In five out of six cases, clinical suspicion was that of a hydatid cyst. In all cases, the lesions had been excised and sent for histopathologic examination.On gross examination, all tumors were cystic, sizes varied from 5.5 to 14.0 cm with a mean size of 9.0 cm. In two cases, the specimens were received in pieces. The cysts were multiloculated in four and uniloculated in two cases. Cyst wall thickness ranged from 0.3 to 0.7 cm. In all six cases, outer surfaces were smooth, gray white to tan brown in color, shiny and glistening to dull, and firm in consistency. No solid or nodular areas were seen in the wall in any of the cases. Cysts were already opened when they were received in the histopathology laboratory.A 45-year-old Sindhi woman from Pakistan presented with epigastric and right hypochondrial pain of 2 months’ duration. The clinical impression was that of a hydatid cyst. Radiological films were not available for correlation. She underwent excision of liver cyst in September 2008. The specimen was received as multiple, irregular dull, gray brown pieces measuring 11.0 × 8.0 cm in aggregate. On sectioning, the wall was 0.7 cm in thickness; the cut surface was firm in consistency and gray with patchy areas of hemorrhage. Solid or nodular areas were not seen. Histopathologic examination revealed a cyst wall lined by a single layer of tall columnar epithelial cells with basally placed uniform nuclei and apical mucin, highlighted on periodic acid–Schiff +/− Alcian blue (PAS+/−AB). Dysplasia was not seen. Stroma was composed of plump spindle cells resembling ovarian stroma. The epithelial cells showed positivity for IHC stain cytokeratin (CK) AE1/AE3. A diagnosis of biliary cystadenoma was made. As the specimen was received in pieces, comments on excision margins could not be made. The patient was lost to follow up.A 43-year-old Punjabi woman from Pakistan presented with abdominal pain and fullness of several months’ duration. Clinical diagnosis was hydatid cyst of the liver. Radiological films were not available for correlation. The cyst was excised in December 2010. The specimen was received as multiple flattened pieces, tan in color, and firm in consistency, measuring 5.5 × 2.0 cm in aggregate. The cut surface was firm, tan brown in color, and 0.5 cm in thickness. Solid or nodular areas were not seen. The microscopic sections showed a cyst wall lined by a single layer of tall columnar epithelium with basally placed nuclei and apical mucin highlighted by special stain (PAS+/−AB). Dysplasia was not seen. Epithelial cells were positive for CK AE1/AE3 and CK 7. Stroma was compact and spindly resembling ovarian stroma and showed positivity for the stain anti-smooth muscle actin (ASMA). A diagnosis of intrahepatic biliary cystadenoma was made. As the specimen was received in pieces, comments on excision margins could not be made. The patient developed recurrence in late 2015, almost 5 years after the initial surgery. She underwent repeat resection in December 2015.A 50-year-old Afghan woman from Afghanistan presented with abdominal pain and swelling in her right hypochondrium. The clinical differential diagnosis included simple liver cyst and hydatid cyst. A CT scan of her abdomen and pelvis showed a large cystic lesion in her right upper abdomen measuring 17.0 × 17.0 × 15.0 cm in dimensions. It was pushing the hepatic flexure inferiorly and right kidney posteriorly and abutting the head of her pancreas and the anterior abdominal wall anteriorly. It was abutting the right lateral abdominal wall on the right side and lesser curvature of her stomach on the left side. It demonstrated few enhancing septa. No solid areas or calcification were seen. It was difficult to determine the exact site of origin. However, it seemed to be arising from her liver with exophytic growth in a downward direction. Her intrahepatic ducts were not dilated. The cystic mass was pushing her portal vein upward. Her portal vein was however normal in diameter. The mass was pushing her gallbladder and her right kidney downward , and subepithelial ovarian-type spindly stroma (positive for ASMA and inhibin). Dysplasia was not seen. A diagnosis of intrahepatic biliary cystadenoma was made. She developed abdominal pain and swelling a few months after the initial excision. Repeat radiology re-demonstrated an exophytic, hypodense, lobulated fluid-attenuated lesion with thin internal septation in the region of the porta hepatis involving segment VI of the liver and measuring 11.0 × 8.0 × 7.0 cm. It was displacing her gallbladder anteriorly. In view of her history, a radiological diagnosis of recurrent intrahepatic biliary cystadenoma was made. She underwent a second excision in December 2013 and we received a cystic tissue piece, flattened and already opened, measuring 11.5 × 6.5 cm. On sectioning, it was multiloculated and the inner surface was coated with dark brown, hemorrhagic material. Representative sections showed cystic spaces lined by cuboidal to columnar epithelium with underlying compact, spindly stroma resembling ovarian stroma. There was extensive hemorrhage, acute and chronic nonspecific inflammation, and fibrosis. Liver tissue was identified at the periphery. A diagnosis of recurrent intrahepatic biliary cystadenoma was made.A 28-year-old Balochi woman from Pakistan presented with abdominal pain and swelling. The clinical diagnosis was hydatid cyst of the liver. A radiological examination demonstrated a large, multiseptate, cystic lesion with thin internal septa measuring 14.0 × 12.5 cm arising from the right lobe of her liver. It was splaying the right and left branches of her portal vein. It was causing significant mass effect resulting in compression of her inferior vena cava (IVC), displacement of her aorta as well as stomach and small bowel loops to the left side, and displacement of transverse colon inferiorly and gallbladder posteriorly. Mild intrahepatic biliary dilatation was noted in both lobes of her liver, most likely due to compression effect of the cyst over her common bile duct  has recently recommended the term “mucinous cystic tumors” for intrahepatic biliary cystadenomas. However, a recent study argued that since the large majority of these tumors have a clear rather than mucinous fluid in their lumina and since the cysts are lined by a single layer of cuboidal to columnar epithelium, which more closely resembles the lining of the gallbladder and bile ducts rather than the lining of the intestine, the term “mucinous cystic tumor” is a misnomer. Thus, the terminology and epithelial phenotype of these rare tumors may remain controversial even in the near future . As ovarPathologists need to: ensure adequate sampling of the specimens, especially from any solid or nodular areas; be careful to document the presence of moderate to high grade dysplasia in the histologic sections; and to keep the main differential diagnoses including hydatid cyst of the liver, simple cyst, and ciliated foregut cyst in mind.


Adenocarcinomas of the oral cavity are rare neoplasms, and only four cases of primary colonic adenocarcinoma of the tongue have ever been described in literature. Very few information about chemotherapy sensitiveness of this type of neoplasia is available, with only one regimen that showed some activity in a metastatic patient.We describe the case of a patient bearing a metastatic colonic adenocarcinoma of the tongue submitted to a first-line chemotherapy with oxaliplatin, 5-fluorouracil and folinic acid (FOLFOX regimen). After chemotherapy the patient obtained the complete disappearance of the primitive neoplasia located in the body of the tongue, and a tumor size reduction > 50% of liver and lung metastases.This case demonstrated the activity of the combination of oxaliplatin and 5-fluorouracil in this very rare neoplasia. The FOLFOX regimen might be considered either in advanced and especially in the neoadjuvant setting, when the reduction of the primary tumor is highly needed. In Western countries tumors of the oral cavity are rare and account for four new cases every 1,000,000 persons/year in Italy . SimilarThe case of a patient with an intestinal adenocarcinoma of the body of the tongue with synchronous distant metastases responsive to FOLFOX regimen is here reported.In June 2013, a 75-year-old woman was diagnosed with a solid mass located in the body of the tongue (Fig. This is the first reported case of a primary colonic adenocarcinoma of the tongue with synchronous metastases, the first appeared in a woman and the fifth ever described.The histogenesis of this cancer is uncertain, but the neoplastic transformation of a minor salivary duct epithelium is quite likely. The hypothesis is supported by the immunohistochemical profile of the tumor and by studies of primary intestinal-type adenocarcinoma of the sino-nasal tract where evidence of respiratory epithelium transformation to an intestinal phenotype have been noted in the adjacent non-neoplastic epithelium . In addiThe mainstay of treatment of adenocarcinoma of the oral cavity is surgery. Thus, neoadjuvant treatments may result in better outcomes. However, the lack of knowledge due to the rarity of these neoplasms hampered the definition of specific guidelines.Differently from what we observed in our patient, all the reported cases presented at diagnosis with locoregional lymphnodal metastases and no distant metastases. Two patients  receivedChemotherapy of adenocarcinoma of the oral cavity is very heterogeneous and includes cisplatin, carboplatin, paclitaxel, 5FU, anthracyclines, gemcitabine, and cyclophosphamide, within others . Little Chemotherapy was well tolerated with an immediate clinical benefit; particularly dysphagia that early improved allowing the patient a regular eating. Chemotherapy induced a good local control of the disease, with significant tumor shrinkage at the end of therapy, and a decrease in size of liver and lung metastases. These results encourage clinicians to consider FOLFOX regimen as neoadjuvant treatment of CAT. It should be underlined that oxaliplatin and 5FU might also be associated to radiotherapy, expanding the possibility of treatment in locally advanced tumors.Overall survival of patients bearing salivary gland carcinomas is heterogeneous, with 5-year survival of nearly 50% in high-grade tumors. Palliative chemotherapy did not demonstrated to be highly active, with response rate ranging from 20 to 25% down to 10% according to histological subtype, type of chemotherapy and reported series [


Defined as the tunneling of one bowel segment into an adjacent bowel segment, intussusception is typically observed in pediatric populations. Here, we present the case of a 78-year-old man who, in a series of unlikely events, developed colonic intussusception due to a benign lead point pathology. Intussusception of the colon is an uncommon occurrence in adults. However, adult colonic intussusception, observed in the absence of a malignant lead point pathology, represents a true clinical anomaly. Intussusception of the bowel is one of the most common abdominal emergencies affecting those under two years of age. It is an appreciably more rare finding in adult populations compared to pediatric ones. When it does arise in adults, it tends to involve the small bowel more often than the colon. Furthermore, when colonic intussusception occurs in an adult, it is usually due to a malignant lead point pathology, like primary adenocarcinoma in particular . Thus, iA 78-year-old man, with a medical history of hypertension, diabetes mellitus II, hyperlipidemia, an implanted loop recorder, legal blindness, and residual left-sided weakness resulting from a cerebrovascular accident, was bought into the emergency department complaining of abdominal pain for one week. The patient complained of chronic constipation and left lower quadrant pain for one year, with exacerbating symptoms the past week. The pain was described as five on a scale of 10 on pain scale, intermittent, sharp, and worse with eating or straining. He had also noted increasing abdominal distension, generalized weakness, and two episodes of non-bilious, non-bloody vomiting for four days prior to coming to the hospital. After taking a fleet enema the day before, the patient had produced a large volume of watery, non-bloody stool. He admitted to a twenty-pound unintentional weight loss but was unable to specify the period of time over which that weight loss had occurred. In an interview, the patient denied any prior abdominal surgeries. The patient also claimed that he had three screening colonoscopies done in his life time, the last one at the age of 72. All of the colonoscopies were normal.On presentation, his vitals were stable. On physical exam, the abdomen was distended and mildly tense, with tenderness in both lower quadrants. On auscultation, the bowel sounds were diminished. No abdominal wall hernia was noted. A computed tomography (CT) scan of the abdomen showed dilated small bowel loops with air-fluid levels and a distended large bowel. Also found was evidence of right transverse and left colon diffuse wall thickening and mucosal irregularity, a constricting mass-like lesion in the proximal sigmoid colon, with significant narrowing, and diverticulosis. There were no signs of bowel ischemia noted on the CT scan. The patient was subsequently put nil per os (NPO) and given nasogastric suction, intravenous fluids, and intravenous ciprofloxacin and metronidazole.A colonoscopy was performed, and at 30 cm, an intussusception of the colonic mucosa was identified Figure .The scope was unable to pass beyond this point, and the patient developed bradycardia, with a heart rate around 30 per minute, so air was removed and the procedure was aborted. Colorectal surgery was consulted and an exploratory laparotomy with mobilization of the splenic flexure was performed. Resection of the sigmoid colon mass along with the adherent ileum and peritoneum was performed. The patient was also given a small bowel side-to-side anastomosis and an end-to-side large bowel anastomosis. A total of 40 cm colon was resected during the surgery. The pathology report of the excised mass showed chronic serosal inflammation and mild fibrosis and determined that the mass was ultimately negative for malignancy. The patient's postoperative care was uneventful and he was discharged home one week after the procedure. The patient was followed up one month after discharge at which point he denied any complaints. The patient is also scheduled to follow up with colorectal surgery.Adult bowel intussusception is a rare clinical finding, accounting for only 5% of all instances of intussusception. Although it commonly first manifests as an obstructive process, only 1-5% of all bowel obstructions are due to adult intussusception. Adult bowel intussusceptions are diagnostically challenging because they can present only with vague abdominal discomfort without any typical obstructive symptoms .The patiIt is estimated that half of all adult bowel intussusceptions result from malignancy, and for this reason, treatment with surgical resection is often unavoidable . One stuBarium enema examinations, endoscopy, and abdominal CT imaging are useful diagnostic tools in identifying patients with colonic intussusception. However, CT is widely regarded as the key to diagnosis, for it can quickly identify intussusception, ensuring appropriate intervention in a timely manner . In partIn a case similar to the one we describe here, a 47-year-old woman developed colonic intussusception in the descending colon as a result of a colonic lipoma. She also cited experiencing constipation over the past four years and abdominal pain or distension in the absence of other symptoms. Although a lipoma is a benign adipose tumor, after it was identified on CT, it was removed through a laparoscopic resection . In anotIn adults, intussusception is managed less conservatively than it is managed in children. Most children can be treated non-surgically first, with either air, water-soluble, or saline enemas. This is especially true at institutions with pediatric surgery departments to rely on for backup support in the case of initial treatment failure. In adults, because malignancy is such a concern, this treatment option is often limited to instances of transient non-obstructive intussusception. It is estimated that 70-90% of adults with intussusception, undergo surgical resection, much like the patient we presented here .Adult colonic intussuseption is a very rare entity and it is even more unusual for it to be a result of a benign etiology. A clinician must be acutely aware of this condition, and when the condition is suspected, diagnosis should be made promptly, failing which serious complications such as bowel obstruction, necrosis, and perforation can occur. Definitive treatment in adults is often surgical in contrast to pediatric cases where conservative management resolves the condtion in a majority of patients.


Ganglioneuroblastoma is a rare tumor with moderate malignancy, which is composed of mature ganglion cells and seen in sympathetic ganglia and adrenal medulla. The diagnosis is possible after cytological and immunohistochemical studies following a needle biopsy or surgical excision. There is no consensus regarding the need for chemo- or radiotherapy after surgery. In this case report, clinical behavior and diagnosis and treatment of the rare tumor cervical ganglioneuroblastoma were discussed. Neuroblastomas are malignant solid tumors. They emerge from out-of-control, immature cells of the sympathetic nervous system. Neuroblastomas can emerge in any place where sympathetic nervous tissues are present. They are most commonly seen in suprarenal gland and areas called truncus sympathicus on neural networks in each side of the spine. In cases where truncus sympathicus is involved, neuroblastomas can emerge in either side of the spine and in any area of it . DiffereIn this study, a 5-year-old patient with no complaints other than neck swelling and a positive pathology of ganglioneuroblastoma is presented.∗3 cm was detected on the front of musculus sternocleidomastoideus in patient's neck. A neck ultrasonography, which had been conducted in the external center, had the result of a lymphadenopathy with the size of 28∗21 mm and with no fat hilus on musculus sternocleidomastoideus anterior.Five-year-old male patient was admitted to our clinic with complaint of swelling in the left side of the neck, which was not accompanied by any pain for four months. He had no other complaints like loss of weight, fever, night sweating, respiratory distress, or difficulty swallowing. In the physical examination, a hard, mobile mass with an approximate size of 2∗2,3 cm size on the front of musculus sternocleidomastoideus and internal carotid artery. The lesion which pushes parotid gland towards the front was starting from the inferior part of the posterior digastric muscle and progressing up to the C4 level  in the postoperative period. However, no sign of this was found in the 3rd-month control examination. Tests like abdominal and thorax imaging, complete blood count, bone marrow aspiration test, 24-hour urinary VMA, and N-myc protooncogene protein scan were conducted. No pathological findings were observed. After the I-131 Metaiodobenzylguanidine (I-131 MIBG) scan in the 6th-month control examination, an involvement was observed in the right cervical region, the opposite area of the operated region, and patient was directed to adjuvant chemotherapy. No evidence of any recurrence was found in the 12th-month control examination.Cervical masses are common pathologies in the pediatric population. While most of them are benign such as inflammatory or congenital ones, over 10% of biopsies extracted from suspicious masses are being reported as malign . GanglioNeuroblastic tumors are divided into four types in International Neuroblastoma Pathology Classification: neuroblastoma, intermixed ganglioneuroblastoma, modular ganglioneuroblastoma, and ganglioneuroma . The typThe International Neuroblastoma Risk Group (INRG) classification system was developed to establish a consensus approach for pretreatment risk stratification. The International Neuroblastoma Staging System (INSS) is a postsurgical staging system. In the INRG classification system, a combination of clinical, pathologic, and genetic markers is used to predict whether the tumor will grow and how it will respond to treatment. These markers are used to define risk. Using the following factors, neuroblastoma is classified into 1 of 4 categories: very low-risk, low-risk, intermediate-risk, or high-risk. INRG includes the following: the stage of the disease according to the INRG Staging System, age at the time of diagnosis, histologic category, such as maturing ganglioneuroma versus ganglioneuroblastoma, intermixed versus ganglioneuroblastoma, or nodular versus neuroblastoma, grade or how cells of the tumor are differentiated, MYCN gene status, chromosome 11q status, and tumor cell ploidy, which is the DNA content of tumor cells.The INRG Staging System (INRGSS) was designed specifically for the INRG pretreatment classification system. It does not include surgical results or spread to lymph nodes to determine the stage. Knowledge regarding the presence or absence of image defined risk factors (IDRFs) is required for this staging system. Stage L1: the tumor is located only in the area where it started; no IDRFs are found on imaging scans, such as CT or MRI. Stage L2: the tumor has not spread beyond the area where it started and the nearby tissue; IDRFs are found on imaging scans, such as CT or MRI. Stage M: the tumor has spread to other parts of the body. Stage MS: the tumor has spread to only the skin, liver, and/or bone marrow (less than 10% bone marrow involvement) in patients younger than 18 months .Al-Jassim had stated that in his case study of 2-year-old patient with ganglioneuroblastoma the patient had developed postoperative Horner syndrome, but this had been resolved spontaneously over time . MoukheiThere is no doubt that imaging techniques are not enough for a definitive diagnosis. Magnetic resonance imaging is recommended. Ganglioneuroblastoma is typically heterogenous in imaging. Generally it shows high signal intensity in T1 images and low signal intensity in T2 images , 6. HoweIt is known that most neurogenic tumors secrete catecholamine. High levels of catecholamine may be an indication of recurrence or the presence of primary tumor. Genetic characteristics are also associated with tumor behavior. N-myc is a protooncogen, where its chromosome arm resides in far end of 2p. Detection of multiple N-myc copies indicates a fast tumor growth and negative prognosis in patients who show these histological characteristics and are over 1 year of age. Also a scintigraphic scan with MIBG should be conducted and invasion should be determined. In our patient, vanilmandelic acid (VMA) and N-myc protooncogene were found to be negative during the postoperative period –8.The need for chemotherapy in postoperative period can be decided after a systematic scan was conducted. In one literature study, a myelogram of a patient, who was under 1 year of age, had showed metastatic cells and the patient had underwent postoperative chemotherapy. Some authors advocate giving chemotherapy even when there is no metastasis, due to ganglioneuroblastoma being a malignant tumor in nature –8. In ouIn this case study, our aim was to discuss clinical behavior and diagnosis and treatment of primary cervical ganglioneuroblastoma together with the literature review. Ganglioneuroblastomas in cervical ganglions are rather rare tumors. They are usually asymptomatic, thus making preoperative diagnosis difficult. Clinicians should bear this in mind as this is found in the differential diagnosis of masses in parapharyngeal space.


Adamantinoma is a low-grade, extremely rare primary malignant bone tumour suggested to be of epithelial lineage. These tumours commonly present at between 10 and 50 years of age and have a propensity for presentation in the tibia (approximately 90% cases), although they have been reported in various other bones  based on symptom improvement, ability to mobilise independently without any walking aids and his age. To our knowledge, this is the oldest case in English literature of a patient with adamantinoma of the femur. He was followed up in our clinic for 36 months with serial radiographs and was free of disease at the last follow-up. Written informed consent was obtained from the patient.Some case reports of femoral adamantinoma exist, one of which describes a 60-year-old lady  and in aThis case report describes the difficulty in diagnosing this rare lesion, which is particularly rare in elderly patients. It reminds us of the fact that one should never drift from the basic oncological principles in the management of a suspicious lesion. All these patients should be treated at a specialist unit with multidisciplinary expertise to avoid surprises. All previous investigations should be reviewed prior to planning and offering any further treatment.


We present a case of metastatic recurrence of carcinoid tumor accompanied by carcinoid syndrome in a 68-year-old Japanese man, 12 years after resection of typical pulmonary carcinoid. Histopathologic examination from percutaneous liver biopsy revealed metastatic typical carcinoid. Clinical symptoms gradually improved after administration of octreotide LAR. Two years after starting treatment, the disease remains well controlled. This case report illustrates the possibility of antiproliferative effects of octreotide LAR on typical pulmonary carcinoid. Typical pulmonary carcinoid is known as a low-grade subtype of lung neuroendocrine tumor (NET) and is characterized by an indolent nature. Recurrence of typical pulmonary carcinoid after complete resection is very rare 3–5%) . The fre–5% . The2 and absence of necrosis were consistent with typical carcinoid. The hilar and mediastinal lymph nodes were negative for any tumor, and the surgical margins as well were free of any tumor.A 68-year-old Japanese man was referred to our hospital because of multiple hyperechoic lesions in the liver without any symptoms. The patient was a former smoker with a 45-pack-year history. Twelve years prior to this presentation, he had undergone thoracoscopic left upper lobectomy for typical pulmonary carcinoid . Histopathologic examination of the surgical specimen revealed finely granular nuclear chromatin, eosinophilic cytoplasm, and rosette formation . The imm2 and no necrosis were also demonstrated, which was compatible for metastatic typical carcinoid. During 6 months of close observation, the patient developed chronic diarrhea resulting in 12 kg of weight loss. His general condition worsened, and he was hospitalized.The patient had a history of atrial fibrillation. Physical examination revealed no abnormalities. Computed tomography (CT) with intravenous contrast of the abdomen revealed multiple low-density areas. Percutaneous liver biopsy of one of the lesions demonstrated well-differentiated tumor with positive expressions of chromogranin A, synaptophysin, and CD56 . The preα-fetoprotein were both elevated, at 24.7 ng/ml  < 16.3 ng/ml) and 56 ng/ml (NR < 10 ng/ml), respectively. Blood 5-hydroxyindole acetate (5-HIAA), urine 5-HIAA, and serotonin degradation metabolite levels were elevated at 1121 ng/ml (NR 57–230 ng/ml), 472 ng/ml (NR 1.8–6.1 ng/ml), and 86.9 mg/13 h (NR 1–6 mg/24 h), respectively, confirming the diagnosis of carcinoid syndrome. Chest CT showed no abnormalities. Abdominal contrast-enhanced CT revealed multiple tumors in the liver and spleen, as well as hepatomegaly , subcutaneous octreotide therapy was started at a dose of 100 μg/day . Blood s2 and/or the presence of necrosis [2 (10 high-power field (HPF)), nuclear pleomorphism, and irregularity with hyperchromatism and prominent nucleoli, areas of increased cellularity with disorganization, and necrosis. Travis et al. reported increased mitotic activity as the only independent predictor of prognosis in the criteria described by Arrigoni [2 (10 HPF) in viable tumor or the presence of necrosis should be the histologic criterion for atypical carcinoid, because atypical carcinoid would be underestimated if the mitotic range is set between 5 and 10 mitoses (10 high-powered fields), and the presence of necrosis is indicative of poor prognosis.Pulmonary carcinoid tumor is a rare NET that accounts for less than 2% of all lung tumors. Good prognosis can be expected following complete surgical resection of pulmonary carcinoid tumor. The recurrence rate for typical carcinoid is lower 3.6%) than that of atypical carcinoid (33.3%) . Histolo% than thnecrosis . As for necrosis  first deOnly a small number of patients with typical carcinoid experience recurrences, with a median time to recurrence of 4 years , longer than that for atypical carcinoid . As a reThe primary lesion was diagnosed as typical carcinoid. However, part of the primary lesion showed conspicuous nucleoli and high cellularity, consistent with atypical carcinoid according to the former criteria . These fWhether liver metastases originated from the lung in our case represents an important issue, because there is a possibility of the presence of another primary site. When liver metastases are demonstrated, CT of the chest and abdomen, abdominal ultrasonography, upper gastrointestinal endoscopy, and colonoscopy are performed to exclude primary tumors other than those of the lung, but no other gastroenteropancreatic neuroendocrine tumors were found in our case. Brain metastasis without pulmonary lesions is not common for primary sites other than the lung.Patients with carcinoid syndrome display a shorter overall survival  than patients without carcinoid syndrome (5.6 years) . PatientSomatostatin receptor scintigraphy (SRS) is useful for the diagnosis of neuroendocrine tumors . AccumulThe LAR formulation of octreotide inhibits tumor growth in patients with metastatic midgut neuroendocrine tumors . OctreotIn summary, this case report illustrates the possibility of antiproliferative effects of octreotide LAR on typical pulmonary carcinoid.


Leiomyosarcoma (LMS) is a malignancy which very rarely occurs in maxillofacial location, and the course of the disease is not very characteristic.In this case report, we present a 58-year-old female patient with a painless tumor of the left angle of the mandible causing slight asymmetry of the face. She also reported that she observed deterioration in fitting of the lower denture in the oral cavity for several months, which she had used successfully for 5 years.On the basis of clinical tests, histopatological examination, and imaging , the patient was diagnosed with primary malignant leiomyosarcoma (LMS) of the mandibular corpus and ramus on the left side. The patient received combined surgical and oncological treatment. The first stage was a surgery, and then adjuvant radiotherapy was applied on the site of the resected tumor—a total dose of 60 Gy in 35 fractions. The patient's postoperative course was uneventful. She also underwent adjuvant therapy well. In the period of 3-year follow-up, no signs of recurrence were observed.The findings may extend our knowledge and experiences in the treatment of leiomvosarcoma in the craniofacial area. Sarcomas of smooth muscle origin, LMS, are extremely rare in the area of the mouth and jaws. This is due to the rarity of smooth muscle in that area, that is, in the vessel walls, arrector pilli muscles, circumvallate papillae, and myoepithelial cells of salivary glands.,3 Only 65 cases of leiomyosarcoma (LMS) located in the mandible were described in the English literature in 1950 to 2011.Sarcomas are a rare group of malignant mesenchymal tumors. They account for about 1% of all malignancies in adults. Histologically, there are 2 types of leiomyosarcomas: more frequent rhabdomyosarcoma (RMS) formed from striated muscle cells and rarely encountered leiomyosarcoma (LMS) developing of smooth muscle mainly in the gastrointestinal tract, bladder, and retroperitoneal space. Cases in a wide range of ages (1–88 years) were also described in the specialist literature. This type of malignancy is slightly more common in men than women. The group of the etiological factors distinguished in the specialized literature included: predisposing disease , environmental factors , and genetic disorders .Leiomyosarcoma is a malignant tumor occurring most frequently in mature age, especially in the sixties.LMSs grow slowly in the initial period and do not have characteristic symptoms. Leiomyosarcoma usually manifests in the mouth in the form of significantly malignant tumor. Previously described locations were related to the tongue, mandible, palate, cheeks, maxillary sinuses, upper and lower gingiva. Initially, LMS affects gingival soft tissues as a nonulcerated swelling that can occur on the buccal and lingual tissue.This kind of tumor is characterized by rapid growth progression and requires combined treatment: radical surgery and then adjuvant radiotherapy. Apart from histological examination, imaging examinations such as CT and MRI are used in the diagnosis of LMS in order to accurately determine the size, location of the tumor, and its relation to surrounding anatomical structures. The size and the location of the tumor are primary factors which allow us to determine prognosis. Patients with small tumors located in the area allowing for a radical resection with wide margin of healthy tissue have a greater chance of recovery. Radical tumor resection is crucial. Unfortunately, anatomical conditions in the head and neck cause an increased risk of too narrow margin of healthy tissue. The patient remains under strict oncological supervision for at least 5 years.Reported size of tumors ranged from 1 to 10 cm. Immunohistochemistry plays an important role in diagnosis and differentiation from other tumors with similar histopathological image. Leiomyosarcomas are composed of spindle cells with visible smooth muscle differentiation and distinct features of atypia. Immunohistochemical tests indicate the presence of actin and desmin, which are detected in 100% and 50% of cases, respectively. Sarcomas tend to spread mainly through the circulatory system , and rarely through the lymphatic system (to the lymph nodes).The aim of the study was to present a case of a patient treated at Clinical Department of Maxillo-Facial Surgery in Rzeszów due to a leiomyosarcoma in the left angle of the mandible.2The patient aged 58 years was referred to the Department of Maxillo-Facial Surgery in Rzeszów due to a painless tumor of the left angle of the mandible causing slight asymmetry of the face. On admission, the patient reported that she observed deterioration in fitting of the lower denture in the oral cavity for several months, which she had used successfully for 5 years. At the same time, the patient also noticed discomfort in the left angle of the mandible that she associated with problems in lower denture fitting, and therefore the patient visited a dentist to ask for a new prosthetic restoration. The doctor referred her to the local department of Maxillo-Facial Surgery.On admission, soft tissue swelling causing a slight asymmetry in the left mandibular angle was found in the patient. Submandibular lymph nodes on the left were enlarged, movable, and slightly tender. Pain was observed at wide opening of the jaws. A tumor located in the retro-molar space in the mandible, mainly from the lingual side, was found in the intraoral examination with the diameter of 35 to 40 mm covered with unchanged mucosa. It was painless on palpation with clearly demarcated upper border of the tumor. A cyst was suspected in the patient on the basis of a preliminary examination. Pantomography showed spherical thinning of the bone structure with osteosclerotic border in the left mandibular angle Fig. .The patient was referred to microscopic tumor verification—excisional biopsy. The procedure was performed under general endotracheal anesthesia with intraoral access. Intraoperative examination revealed a damaged cortex of the mandible and osteolytic bone loss of 3- to 3.5-cm diameter, which was filled with a tissue mass of lard consistency easily separated from the bone foundation. The surgical wound was sutured with simple interrupted sutures. The removed tumor in 10% formalin solution was sent for histopathological examination. Microscopic preparations were consulted in the Department of Pathology, Collegium Medium, Jagiellonian University in Kraków. The test No. 1776506: leiomyosarcoma (low grade). Immunohistochemical profile (IHC): SMA + Vimentyna + Desmina-, S100-, Ki67 + 10%.The diagnosis was extended in connection with the results obtained in the histopathological examination. CT and MRI of the head and facial skull, ultrasound of the neck and abdomen, chest X-ray were performed. CT of the head and neck showed irregular loss in the bone structure sized 30 × 37 × 20 mm; the surrounding bone structure of uniformly increased sclerosis was filled with hypodense palpable mass without contrast enhancement , the patient was diagnosed with primary malignant leiomyosarcoma (LMS) of the mandibular corpus and ramus on the left side. The patient was referred to combined surgical and oncological treatment. The first stage was a surgery, and then adjuvant radiotherapy. The patient was qualified for extended surgery, that is, partial resection of the corpus and ramus on the left and excision of groups I, II, and III lymph nodes with simultaneous restoration of bone continuity by means of a titanium plate. The resection of mandibular corpus and ramus, and the titanium plate reconstruction were performed under general anesthesia. Immediately after surgery, the patient was monitored in the postoperative room. The patient's postoperative course was uneventful, and in the following days, her general condition was good. Granulation and prolonged wound healing were observed locally in the distal part of the wound. Redon drain was removed 5 days after the surgery. The patient reported, after the surgery, hypoesthesia of the lower lip, chin, and the mucous membrane of the mouth bottom on the left. After 10 days of surgery, the patient was discharged from the clinic and transferred to the Department of Radiotherapy, Subcarpathian Oncology Centre, where adjuvant radiotherapy was applied on the site of the resected tumor—a total dose of 60Gy in 35 fractions. From the 10th day of radiotherapy radiation, lesions were observed on the face, neck, and oral mucosa. She underwent adjuvant therapy well. The patient is under supervision of Oncology Clinic, Subcarpathian Oncology Centre, Rzeszow, until now. CT examination of facial part of cranium and neck did not reveal any local recurrence. Mandibular condyle head was movable on palpation, opening of the jaws was somewhat limited to ∼3 cm. The scar in the chin and submandibular area on the left was linear and smooth, with no signs of recurrence; the left cheek was slightly hollowed. Intraoral examination showed the presence of scar tissue on the bottom of the oral cavity on the left without characteristics of local recurrence. The surrounding soft tissues had increased density Fig. . In the 3A very rare occurrence and lack of characteristic symptoms of leiomyosarcoma developing in maxillofacial area poses a significant diagnostic and therapeutic challenge in clinical practice.Difficulties in diagnosis of the described case were associated with less distinctive microscopic image of collected slice and surgical material. Consultation of preparations in Reference Centre contributed to proper diagnosis and initiation of treatment. In addition to the standard histopathological examination, immunohistochemical profile: SMA + Vimentyna + Desmina-, S100-, Ki67 + 10% plays an important role in LMS diagnostics; it allows us to differentiate tumors with similar histopathological image, that is, rhabdomyosarcoma, fibrosacoma, angiosarcoma, and the others. conducted metanalysis of 20 LMS cases treated during 37 years in West China Stomatology Hospital. The authors reported that LMS located in the maxillo-facial area is very rare, and its incidence did not exceed 0.06% of all malignant tumors developing in the area of the oral cavity.Only isolated cases of LMS located in the mouth were described in the Polish specialist literature. Yan et alThe presented case of 58-year-old woman did not correlate with etiologic factors. the 5-year survival rate for primary LMS in the oral cavity is ∼55%. Local recurrence occurs in ∼34% of cases. The regional lymph nodes are affected in >15%. Distant metastases, mostly to the lung, occur in ∼35% of patients with LMS tumor in the oral cavity. The issue of the LMS treatment is a major challenge for a surgeon and an oncologist. Surgical treatment was applied in the patient treated in our clinic; it consisted of tumor excision with partial resection of the mandible and reconstruction of a tissue defect with titanium plate and adjuvant radiotherapy. The patient had uneventful perioperative course.According to modern views on LMS therapy, combined therapy is effective . The most important predictor is microscopic image of free surgical margins. According Ethunandan et al,


A superior orbitectomy can be a challenging but accomplishable surgical option in dogs with a tumor which involves the dorsal bony part of the orbit. The procedure described in this report can be vision‐sparing and life prolonging even in a case of an aggressive growing frontal sinus squamous cell carcinoma. Squamous cell carcinoma (SCC), which primarily originates from the mucosal lining of the frontal sinus, is a rare disease in humans as well as in animals Treatment of frontal sinus tumors in humans is inconsistent Complete resection and adjuvant therapy (chemo‐ or radiotherapy) are the recommendations of another study for the treatment of this sort of tumor. However, it is unclear whether the use of chemotherapy and/or radiotherapy can improve the prognosis of pFS‐SCC in humans.In dogs and cats cranial and orbital tumors pose diagnostic and therapeutic challenges. Most tumors located in the periorbital region are malignant Precise recommendations for the treatment of pFS‐SCC in animals exist.Without therapy, eight dogs with SCC of the nasal cavity and frontal sinus had a median survival time of 3 months A 9.5‐year‐old spayed female Small Munsterlander (body weight 14 kg) was presented with a facial deformity limited to the left frontal sinus region. A mass (about 3 × 3 × 3 cm) had developed over a three‐month period. The owners described sneezing and sniffing before and after visibility of the mass. Apart from that, abnormal clinical signs were not noted.The dog was alert and in fairly good body‐condition. A solid mass with a diameter of 3 cm could be observed dorsomedially to the left eye Fig. . The masA complete ophthalmic examination was performed including biomicroscopy , indirect ophthalmoscopy with 30‐ and 20‐diopter condensing lenses , and tonometry . The left eye showed minimal proptosis with a slight ventro‐lateral deviation.The eyelids, conjunctiva, cornea, and anterior chamber were normal bilaterally. Both lenses showed minor fiberglass/crystal‐like nuclear cataracts. Examination of the fundus revealed bilateral local and symmetrical chorioretinal hypoplasia. The intraocular pressure was 8 and 6 mm Hg in the right and left eye, respectively.For determination of appropriate therapeutic options, blood chemistry (BC), complete blood cell count (CBC), abdominal ultrasound, and computed tomography (CT) examinations of the head and thorax and biopsies of the mass were obtained. BC and CBC were found normal. Abdominal ultrasonography did not reveal discernible abnormalities.®, Temmler Pharma, Marburg, Germany) and 0.5 mg/kg levomethadone in a fixed combination with 0.025 mg/kg fenipramide . Anesthesia was maintained via endotracheal intubation with 1% isoflurane  dissolved in oxygen at a flow rate of 10 mL/kg/min. CT findings  of the head included an isoattenuating (to surrounding musculature) soft tissue mass at the zygomatic process of the frontal bone, which caused lysis and erosion of the bone and extended into the frontal sinus and the orbit  was clipped and prepared aseptically for surgery, and the dog was positioned in sternal recumbency Fig. .®, Saw blade 70/49 × 20 × 0.6/0.4 mm; Saw Attachment with variable deflection; Synthes inc., West Chester, PA). In order to leave the left eye, optic nerve and retrobulbar muscles intact, extreme care was required.Next, skin and subcutaneous muscle were cut sharply as shown in Figure ®; Ethicon Johnson‐Johnson, Norderstedt, Germany) were sutured into the defect  and 4/0 polyamide  respectively using a simple interrupted suture pattern. Finally, to avoid seroma development an active drainage system  was inserted into the left temporal region and placed beneath the flap  administered orally for 10 days, 4 mg/kg SID carprofen  administered for 3 days and topical eye drops  administered five times a day for 10 days. Eye drops  were administered four times a day for 2 months to ensure the moisturization of the conjunctiva and cornea. An Elizabethan collar prevented self‐inflicted trauma. The day after the surgery the habitus of the dog was found normal. He was discharged three days postoperatively after the drain removal. Postoperatively, the dog showed a slight ventro‐lateral deviation of the left globe, and he was able to close his eye despite an iatrogenic ectropium of the upper eyelid.The postoperative treatment involved 20 mg/kg BID amoxicillin/clavulanic acid  every 3 weeks with six repetitions. Meloxicam 0, 1 mg/kg SID  was added to carboplatin. The chemotherapy was well tolerated by the dog.Consequently chemotherapy was initiated with carboplatin 300 mg/mFor the purpose of evaluation of the response to treatment CT examination of the head was performed 5 months after initiating chemotherapy but further progression of the mass has to be stated Fig. .Only mild bloody nasal discharge was observed by the owners at this stage. No evidence of visible and palpable tumor regrowth could be found clinically Fig .Chemotherapy was stopped on the grounds of not having any therapeutic effect. Changing into any another treatment protocol (Cisplatin e.g.) was declined by the owners.The patient was discharged from the hospital due to the relative absence of symptoms. The owners were advised to euthanize the dog should clinical signs. This came to pass 21 months after orbitectomy and 5 months after finishing chemotherapy because of progressive epilepsy.Human studies have shown a poor prognosis when the pFS‐SCC is advanced Recommendations for removal of pFS‐SCC in dogs do not exist. For oral SCC surgical margins of at least 2 cm are recommended If the bony orbit is involved, then an orbitectomy is necessary for curative resection Orbitectomy is infrequently reported in the veterinary literature and can be subdivided into total or partial orbitectomy. A partial orbitectomy includes an excision of either the superior  or inferior  orbit. In case of total orbitectomy enucleation was always included In the dog in this case report the tumor extended into the orbit, causing mild proptosis. Thus in the presented case a superior orbitectomy was supposed to maximize the probability of removing all cancer cells. En bloc resection of the tumor for radical resection was performed, with preserving of the left globe. The histopathological examination revealed clean wound margins.In one case report the pFS‐SCC could be removed, but the removal was incomplete Eye sparing superior orbitectomy, possibly in combination with concurrent chemotherapy seems to be an appropriate treatment option for of pFS‐SCC in dogs.AS: sole authorNone declared.


We present the first ever report on a colonic mucocele observed at the distal stump of a transverse loop colostomy caused by neoplasia. A 37-year-old female consulted us because of abdominal pain and vomiting caused by cystic lesions in the upper left abdominal quadrant. A preoperative checkup revealed no sign of neoplastic lesions; however, tumor resection was performed because of the symptoms. The tumor was a mucocele of the distal stump of the transverse colon with obstruction interposed between the mucocele and stoma. Pathological diagnosis was low-grade adenoma; however, it appeared like low-grade mucinous neoplasia of the appendix rather than a normal colonic adenoma. The neoplasia existed in the transitional segment between obstruction and dilatation. As this is the first case of colonic mucocele caused by mucinous neoplasia, no definite consensus for diagnosis and treatment exists. With reference to low-grade mucinous neoplasia, we propose that complete surgical resection be performed for diagnosis and a favorable outcome. Mucocele of the colon is a rare clinical manifestation, with only few reports being available. The etiology of colonic mucocele is yet to be unraveled, and no pathological features have been described so far. Here, we describe the first case of colonic mucocele caused by a low-grade colonic neoplasia, providing an insight on the clinical and pathological etiology of colonic mucocele.A 37-year-old female with a history of frequent surgery consulted us because of recurrent vomiting and abdominal pain in the upper left quadrant. The first surgery, which was performed when the patient was 1 year old, was transverse loop colostomy for colonic obstruction due to a yolk sac tumor anterior of the sacrum; the tumor was subsequently resected when she was 2 years old. The following year, the tumor presented a local recurrence and was resected along with the sacrum below S4. Posterior pelvic exenteration for a complicated fistula between the vagina, rectum, and urinary bladder was performed when the patient was 8 years old. Radical cystectomy for hemorrhagic cystitis was performed the following year, and the patient was then subject to left nephrectomy for renal hypertension at the age of 12. Furthermore, several minor surgeries were performed; however, all operative records, including the ones referred to above, were not available. For the purpose of understanding the present anatomy and finding the cause of the symptoms, we performed diagnostic imaging. Contrast-enhanced computed tomography (CT) showed a large cystic tumor in the upper left abdominal quadrant, exhibiting expansive growth with no sign of invasion into the stomach, pancreas, or spleen—the organs surrounding the tumor. No sign of rupture was found. A thin string-like structure interposed between the tumor and the stoma was observed in the CT image (Fig. Following upper midline abdominal incision, we found the transverse colon, which was distal to the site of loop colostomy, to be expanded indicating a cystic tumor. The segment between the loop colostomy and cyst appeared strictured; therefore, we speculated that mucin was trapped at the stump Fig. . We detaMacroscopically, the cyst was filled with yellowish mucin, and no tumor change was found at the mucosal surface of the cyst and stricture segment. However, during microscopy Fig. , dysplasThe patient was discharged on the 12th postoperative day; the serum CEA level decreased to 3.3 ng/mL 1 month after the surgery.Appendiceal mucocele, which is caused by the accumulation of mucin due to appendiceal obstruction, is a well-known type of appendiceal tumor, accounting for 0.1–0.3 % of all appendectomies , 3. The The existence of neoplastic lesions similar to low-grade appendiceal mucinous neoplasia was a striking feature in our case. Low-grade mucinous neoplasia is often found in appendiceal mucocele; however, no such neoplasia has been reported so far in a patient with colonic mucocele. We hypothesized on how neoplasia could be related to the etiology of the mucocele of the present case. First, atrophic changes developed in the colon distal to the site of loop colostomy, resulting in narrowing of the luminal diameter. Even at this point, the intraluminal space would be preserved for continuous discharge produced by the distal stump. Subsequently, however, the neoplastic lesion produced more amounts of viscous mucin which then pass the stricture where the mucocele developed. This hypothesis presents a possible explanation for why the patient developed mucocele a long time after colostomy. Hence, the development of neoplasia was the primary cause of the mucocele.We did not obtain definitive diagnostic results prior to the surgery. Although percutaneous aspiration biopsy is potentially accompanied with complications like hemorrhage or tumor dissemination, we did not obtain informative histological findings even after this procedure. No abnormal accumulation was found by positron emission tomography, and signs of neoplasia were not identified by colonoscopy. This diagnostic uncertainty was reminiscent of the challenges encountered when diagnosing appendiceal mucocele .Because this is the first case of colonic mucocele accompanied by low-grade mucinous neoplasia, any consensus regarding appropriate operative procedures does not currently exist. According to the WHO classification, low-grade appendiceal mucinous neoplasia is a malignant tumor, and therefore, complete surgical resection is suggested. Hence, complete surgical resection appeared appropriate in the present case. Moreover, given the condition that it is difficult to obtain a definitive pathological diagnosis preoperatively, we should consider surgery for colonic mucocele even in the absence of proof of dysplasia. The postoperative course of appendiceal mucocele is typically characterized by recurrence presenting as intraperitoneal myxoma, and medical follow-up by CT scan every 6–12 months is recommended. With reference to this recommendation, we intend to perform a periodic follow-up of the colonic mucocele.In conclusion, we demonstrated colonic mucocele caused by low-grade mucinous neoplasia. Although standardized diagnostic criteria and surgical procedures do not exist as yet, complete resection for colonic mucocele to avoid rupture and unfavorable clinical outcomes should be performed.We obtained written informed consent for publication from the patient.


Leiomyosarcoma (LMS) is a malignant tumour formed of cells with distinct smooth muscle features. Leiomyosarcomas rarely metastasise to the oral cavity and this literature review details all reported cases of metastasis to the mandible found in the literature. This offers a unique perspective by specifying mandible as the site of metastasis of leiomyosarcoma.A 53-year-old female presented to her General Practitioner (GP) with heavy menstrual bleeding and was diagnosed with multiple fibroids. Folowing a hysterectomy and removal of both tubes and ovaries for these symptomatic uterine fibroids, an incidental diagnosis of low grade leiomyosarcoma was made. A CT scan found no evidence of residual or metastatic disease and no further treatment was deemed necessary. 6 months later she presented to A & E with a numb lower lip but it took another 6 months for the diagnosis of metastatic LMS to the mandible to be made.Leiomyosarcomas are aggressive tumours which are liable to metastasise and therefore have a poor prognosis. An extensive literature review was undertaken to explore the frequency of metastasis in the maxillo-facial region.Although metastasis to the oral region is very rare as suggested from the literature review, when patients present with unusual symptoms after a diagnosis of LMS, physicians should be aware of the possibility of LMS metastases. Leiomyosarcomas (LMS) are smooth muscle tumours arising from the embryonic mesenchyme cell lines. Uterine LMS are aggressive tumours often associated with a poor prognosis however a rare entity accounting for 1% of cases with a uterine malignancy. Treatment is surgical in the early stage when the disease is confined to the uterus. Recurrent disease is diagnosed by the development of new symptoms such as the index case. Metastases to the oral cavity are rare and we report an interesting case of uterine LMS with metastases to the mandible and review the literature to assess frequency of mandibular metastases.A 53 year old female presented to her General Practitioner with heavy menstrual bleeding and was referred to a gynaecologist at her local hospital. Her previous medical history included one normal vaginal delivery aged 28, a Mirena IUS in situ, and, a normal cervical screening history. Drugs prescribed included ranitidine and citalopram. Family history included an identified BRCA mutation carried by her mother and aunt. She had not been genetically tested herself but underwent regular 6 monthly checks from ages 41 to 50.Multiple fibroids were identified on a trans-vaginal ultrasound of the pelvis. In view of her ongoing symptoms, the patient was offered a hysterectomy with a bilateral salpingo-oopherectomy.The histology of the uterine specimen demonstrated one fibroid measuring 70 mm diameter and showing an abnormal focal area and central cystic necrosis consistent with a low grade leiomyosarcoma surrounded by normal tissue with no evidence of vascular infiltration. The leiomyosarcoma border was 10 mm clear of the serosal surface. A CT scan of the chest, abdomen and pelvis found no evidence of residual or metastatic disease and her case was discussed at a Gynaecologic Oncology multi disciplinary meeting (MDT). It was agreed that the likelihood of further metastasis was remote and she was discharged from follow-up.Six months later, the patient presented to the accident and emergency department with a complaint of numbness in her lower left lip. A full neurological examination was normal so the patient was assured it would likely resolve. However, following a consultation with her dentist, she was referred to the maxillofacial department. The patient was known to grind her teeth due to anxiety. Altered sensation over left chin was reported, followed by numbness in her left lower lip. On examination, she was found to have decreased sensitivity to soft touch in the left mental nerve distribution area and an electric shock like sensation through her lip when pressure was applied to her mental foramen. Dental pulp testing with ethyl chloride was used on her lower left second premolar but no response was recorded and on X-ray, the apex was found to be directly next to the mental foramen and an apical radiolucency was associated with this tooth. She was treated with a course of amoxicillin on the diagnosis of an apical infection affecting the mental nerve. Despite the antibiotic treatment, the numbness continued and the patient went on to develop dysesthesia of the left 4th and 5th fingers. She also experienced an episode of severe left-sided facial pain associated with difficulty in opening her jaw; the left side of her chin became swollen and rubbery to touch. Her left lower second molar was treated with a root canal filling, which also failed to improve her symptoms. Further neurological examination confirmed trigeminal motor function was unaffected and Tinel’s sign was negative in the left elbow; peripheral nerve and Electromyography (EMG) studies were also found to be normal.Magnetic resonance imagery of the brain and mandible along the course of the mandibular branch of the trigeminal nerve showed no evidence of metastasis. CT of the skull base, mandible and neck showed no bony abnormality and did not identify any prominent lymphadenopathy. Other investigations were undertaken, including chest X-ray, full blood count, ESR 5, clotting screen, B12 levels, plasma electrolytes, calcium, C reactive protein, angiotensin converting enzyme levels, liver function tests, thyroid function and blood sugar levels which were all normal. Tumour markers were tested and normal as well as Wassermann reaction, anti-nuclear factor, anticardiolipin antibodies, ANCA (antineutrophil cytoplasmic antibodies) screen and borrelia titres were negative, making any infective or autoimmune causes less likely. The patient declined to have a lumbar puncture (LP), instead wishing to try a course of acupuncture first. After completing her sessions of acupuncture over 4 weeks, she felt that the sensory disturbance in her left hand and left chin had improved by about 40%. She therefore decided to carry on with another four sessions of acupuncture before considering a LP. Having finished the acupuncture, the patient found her left hand sensory disturbance to be almost completely resolved and her left chin was continuing to improve. She was prescribed antibiotics by her dentist to treat an abscess of the lower left wisdom tooth however this did not resolve the problem and led to a swelling in her left cheek. The left lower third molar was removed, and while extracting the tooth, a biopsy of abnormal ‘moth eaten’ looking soft tissue was also taken.The histopathology report of the biopsy revealed an undifferentiated sarcomatous malignant tumour. Immunohistochemistry staining was used to compare this specimen to the uterine LMS from the hysterectomy previously and was consistent with recurrent disease. A second CT of the neck, abdomen and pelvis performed after an interval of 6 months revealed an extensive mass lesion affecting the left jaw and soft tissue mass on both sides Fig. . A dentaPrimary treatment was resection of the masseter, medial pterygoid and angle of the mandible. A positron emission tomography (PET) scan was suggestive of small metabolically active area in pelvis, possibly further nodal spread, but it was not macroscopically visible on CT so no further excision was recommended.Radiotherapy to the left mandibular lesion followed, with clinical follow-up. Further metastases subsequently developed with two lesions in the lungs, and one in the left pelvic side-wall. Diagnosis was made after a PET scan and the pelvic mass was treated with resection and radiotherapy. A pathological fracture to the humerus, secondary to the bony LMS metastasis at the site, lead to surgical correction and further radiotherapy. She later developed a metastatic lesion within the duodenum and underwent a pancreatic duodenectomy with resection of metastasis. An osteolytic mass was then identified in the right 4th rib and local palliation radiotherapy was administered. After further disease progression, 6 cycles of chemotherapy and palliative radiotherapy to the right iliac area, she died of progressive disease, 4 years following the original diagnosis of uterine LMS.A review of the literature was undertaken to assess frequency of mandibular metastases from uterine LMS. Databases including MEDLINE, EMBASE and google scholar were searched, the search being limited to the English language but with no time limit. Searching of the grey literature was also undertaken. Articles containing case reports of LMS metastasising to the ‘oral cavity’ were then further analysed to find cases specifically involving the mandible as a site of metastasis. From this thorough search, LMS metastasizing to the mandible was only reported in seven other cases, three from the uterus –3 as in The index case is only the 4th reported case of uterine LMS metastasizing to the mandible found by this extensive search of the literature Table  and highLeiomyosarcoma (LMS) is a malignant tumour formed of cells with distinct smooth muscle features . UterinePatients presenting with uterine LMS have a median age of 55 years and commonly present with vaginal bleeding (56%), a palpable pelvic mass (54%) and pelvic pain (22%) . HoweverPathologically, LMS structure is irregular and large with necrosis, haemorrhage, atypical nuclei and often with extra uterine extension . MagnetiThis case is unusual due to the location of the metastasis, as the oral cavity and oropharynx are rare sites of metastases from primary cancers of any organ. In fact, only 1–2% of oral malignant tumours are metastases from other primary sites . HoweverClinicians need to remain suspicious of metastases in any site following treatment for a primary LMS. LMS are difficult to diagnose initially and there may be an inevitable delay in diagnosis due to investigation into infectious or erosive causes. However if a patient has a previous history of LMS, a metastasis should always be considered if they present with unusual symptoms, including in the jaw as in our patient. Consideration of metastatic LMS in a patient with a previous diagnosis is likely to allow for a swift diagnosis, and a possible improved prognosis. In this index case, it took over 6 months after the patient presented with sensory defects to be diagnosed with a metastasis to the jaw. Delay in the diagnosis and therefore treatment of metastatic LMS has the potential to allow the cancer to develop and increase the chance of further spread. A delay in diagnosis also prevents the onset of palliative care and leaves the patient symptomatic.As can be seen by Table The referral of the index case to the supraregional centre for sarcomas may have improved outcome, due to experienced staff optimising the treatment given to this patient. At the time of referral, their diagnostic resources were superior to those in the local hospital: for example they could use PET to detect further metastases that were not apparent on CT.This case demonstrates the possibility of LMS metastasising to the jaw and affecting nerves in this region, proving the aggressive nature of the tumour. If there is a recent history of leiomyosarcoma and a patient presents with nonspecific or unusual symptoms or with sensory disturbance in any system of the body, a secondary leiomyosarcoma should be considered in the differential diagnosis. Aggressive treatment in a specialist centre combined with the continued identification of new metastases may improve survival.


Mature cystic teratoma (MCT) is the most common neoplasm of the ovary and includes at least two well- differentiated germ cell layers. Different combinations of mature tissue derivatives with varying arrangements in the tumour cause a wide spectrum of radiological presentation ranging from a purely cystic mass to a complex cystic mass with a considerable solid component. In different imaging modalities, each radiological feature reflects a specific pathologic equivalent that forms because of diverse compositions of histological components. Understanding uncommon findings as well as the classic signs with basic knowledge of pathological equivalents permits a more accurate diagnosis and guides adequate treatment. In this review, radiological features of MCT in different imaging modalities  including specific signs and useful radiological artefacts with brief emphasis on pathological basics are discussed.Teaching pointsOvarian mature cystic teratomas (MCTs) have a wide spectrum of radiological presentation.• Each radiological feature of MCT reflects a specific pathologic equivalent.• Understanding radiological signs with basic knowledge of pathology can permit a more accurate diagnosis.•  Teratoma is the most common germ cell tumour of the ovary and accounts for about 20% of all ovarian neoplasms . PatholoIn imaging, MCTs have a wide spectrum of radiological presentation ranging from a purely cystic mass to a complex cystic mass with a considerable solid component. Ovarian teratomas are usually incidentally detected as a heterogeneous mass via ultrasonography (US). Although the fat component seems hyperechogeneous, it is not always easy to differentiate it from a complex haemorrhagic cyst. However, some radiologic signs, such as the dot-dash and tip of the iceberg signs, enable radiologists to diagnose teratomas sonographically. Cross-sectional imaging methods (computed tomography and magnetic resonance imaging) have excellent sensitivity for the diagnosis of MCT due to identification of fat . In addiThis review covers common and uncommon imaging features, pathognomonic signs and useful radiologic artefacts in the diagnosis of MCT with a brief discussion of pathological equivalents.MCTs are seen in a younger age group (mean age 30 years) than epithelial ovarian neoplasms . They arMost MCTs are benign and asymptomatic unless a complication or a paraneoplastic syndrome develops. They grow slowly at a rate of 1.8 mm/year . They arMCT usually looks like a unilocular cystic cavity at macroscopic examination. Also it may include septa dividing the cyst into several compartments. The tumour cavity is filled with sebaceous material because of the squamous epithelia in the wall. This material is liquid at body temperature and semisolid at room temperature . There iHistologically, mature tissues from different cell lines lie within the wall. Walls of the cyst are frequently lined by squamous epithelium and often hyalinized, compressed ovarian stroma covers the external surface . In an MThe presence of any immature tissue warrants a diagnosis of immature teratoma. If the teratoma is predominantly or exclusively composed of one germ cell line , then it is referred to as a monodermal teratoma .Ultrasonography is the most commonly used imaging modality for assessment of pelvic genital organs. The major role of US is to confirm the presence of a mass and determine its organ of origin . By asseMCTs may show a variety of appearances sonographically because of their variable composition. However, they usually have a non-specific appearance. The sonographic appearance of ovarian cystic teratoma has been described to have “virtually limitless combinations” of different echo patterns . TherefoThe Rokitansky nodule is the most common sonographic typical finding and is seen as a densely echogenic protuberance projecting into the cystic lumen Fig.  5]. Thi. Thi5]. This finding is the second common manifestation of MCT . It resuThis sign refers to the sonographic appearance of a mass with an amorphous echogenic focus in the near field that causes posterior shadowing and thus obscures the posterior portion of the lesion and any structures behind it Fig.  22]. Th. Th22]. In fact, acoustic shadowing is an attenuation error artefact that can be used by the clinician to determine the composition of a structure . When thAs a diagnostic pitfall, the echogenic dermoid plug can resemble bowel gas or faecal material in the colon so that it can be easily overlooked or dismissed, particularly when sound attenuation obscures the posterior wall of the lesion creating the tip of the iceberg sign . TherefoThe classic sonographic appearance of echogenic hair floating within an MCT is described as the “dot and dash” sign, which has the highest positive predictive value (98%) . This soSebum floating above aqueous fluid forms a fat-fluid level separated by a linear interface that changes position with gravity. However, a layered appearance in a cyst on US is not specific for MCT. Fat-fluid or fluid-fluid levels have prognostic significance only when identified in combination with at least one additional sonographic feature associated with MCT .The histological composition of the fatty component, varying from pure liquid sebum to adipose tissue intermixed with hair, desquamated epithelium or fibrous tissue, results in different acoustic properties . When thIntracystic hyperechoic floating balls are an uncommon pathognomonic feature of MCT Fig.  21]. In. In21]. When hair makes a level with fluid, a comet tail appearance forms . Due to CT has an excellent sensitivity (93–98%) because of detecting fat in the diagnosis of MCT . When USThe presence of fat inside an ovarian tumour appears to be specific to ovarian cystic teratoma . Fat mayFat displays lower attenuation than water on CT images; therefore when the proportion of fat within a voxel is large enough, the corresponding image pixel seems dark, with a density number ranging from −144 to −20 HU .On the CT image, a Rokitansky nodule can look like a rounded structure protruding into the cystic lumen, mural thickening, a bridge across the cyst or sometimes only a tooth Fig.  35]. Th. Th35]. It is possible to see curvilinear or globular calcification in the Rokitansky protuberance, in the wall of the MCT or in/near the septa Fig.  6]. In . In 6]. Two different liquid components can be clearly demonstrated by measuring their attenuation values on CT. Supernatant fatty layers of lower attenuation, similar to that of subcutaneous fat, and dependent fluid layers of higher attenuation are classically seen on CT Fig.  29]. So. So29]. A striking uncommon finding of MCT is numerous small spherical structures within the cyst Fig.  31]. Us. Us31]. On MR imaging, intratumoral fat shows high intensity on T1-weighted images and a signal drop on fat-saturated T1-weighted images Fig.  15]. Si. Si15]. Teratomas may show reversed chemical shift artefact, which is a boundary artefact perpendicular to the direction of the frequency encoding gradient . This arOn MRI, fat and fluid may show layering with high signal intensity of the supernatant fatty layer on T1-weighted images and low signal intensity on fat-suppressed T1-weighted images Fig. . On T2-wMorphological features of the Rokitansky nodule  can easily be evaluated with MRI Figs.  and 21. Matted hair, which usually constitutes the major part of the layered debris, is gravity dependent and may show mobility when changing the patient’s position Fig.  4]. Thi. Thi4]. Protrusions, which are usually located at the point of contact with residual ovarian tissue, vary from small nodules to polipoid palm tree-like masses projecting into the cyst cavity Fig.  8]. The. The8]. Floating globules usually have a nidus composed of debris, desquamative material or fine hair shafts that is hyperintense on T2-weighted and hypointense on T1-weighted images . The outThe keratinoid substance is a scleroprotein that originates from the cytoskeletal structure of the epidermis. Radiologically it exhibits low signal on T1- and high signal on T2-weighted images resembling serous fluid . HoweverMCTs have a wide spectrum of appearances in different imaging modalities. Each radiological sign is a description of a specific pathologic appearance and reflect the different combinations of histological components. Understanding uncommon findings as well as classic signs with basic knowledge of pathological equivalents permits a more accurate diagnosis and guide adequate treatment.