Mature cystic teratomas account for 10–20% of all ovarian neoplasms and are the most common neoplasm in younger patients. Spontaneous rupture of the teratoma is rare and has been occasionally reported. Here we present an unusual case of left ovarian teratoma rupturing into sigmoid colon. Mature cystic teratomas account for 10–20% of all ovarian neoplasms and are the most common neoplasm in patients younger than 20 years of age. Mature teratomas are usually benign, but in 0.1-0.2% of cases, it may undergo malignant transformation [An eighteen years old female presented in the emergency OPD with the complaints of pain abdomen, fever, non-passage of flatus and stool, and nonprojectile, nonbilious vomiting of two days duration. There was history of typhoid fever four months back for which she received treatment and recovered. Menarche was at 14 years and menstrual cycles were normal. On examination the abdomen was distended, tense, and tender with marked guarding and rigidity. No other obvious lump or organomegaly was detected. There was masking of liver dullness with evidence of free fluid in peritoneal cavity. Digital rectal examination revealed free fluid in the pouch of Douglas.Plain X-ray abdomen revealed free gas under the diaphragm. There was mild leucocytosis. In serum biochemistry, blood urea was 60.3 mg/dL and serum creatinine was 1.3 mg/dL.The patient underwent exploratory laparotomy with the diagnosis of perforation peritonitis. On exploration, a large 8 × 8 cm teratoma arising from the left ovary was found infiltrating the sigmoid colon near the rectosigmoid junction leading to perforation of the sigmoid colon . The terThe word “teratoma” is derived from Greek work “teraton” meaning monster. It was initially used by Virchow in 1863. The term “dermoid cyst” was coined by Leblanc in 1831 . Both arArising from totipotential cells, these tumours are midline or paraxial. After sacrococcygeal teratomas (57%), gonads (29%) are the second most common site. Mature cystic teratomas are 10–20% of all ovarian neoplasms, mostly occur in patients less than 20 years of age. Complications of ovarian teratoma include torsion, rupture, infection, and malignant change.Spontaneous rupture of the cyst is extremely rare because of its thick wall and is reported in 0.3% to 0.7% of cases . The cysDeodhar et al.  have repIn our case, as there was history of typhoid fever four months back, it could have caused inflammation and adhesion of the dermoid cyst to the bowel wall eventually leading to perforation of sigmoid colon. Ovarian teratoma commonly has an indolent course and presents with abdominal pain due to complications like torsion, hemorrhage, or infection. Spontaneous rupture of teratoma is rare due to its thick wall, and it usually occurs in the peritoneal cavity. Rupture into a hollow viscus due to adhesions is extremely rare complication which may present as perforation peritonitis.


Xanthogranuloma of the stomach is an extremely rare disease, and this lesion has only been found to coexist with early gastric cancer in 2 cases in the literature.We report a case of xanthogranuloma of the stomach combined with early gastric cancer that mimicked an advanced stage tumor. A 65-year-old female was referred to our hospital because of epigastralgia. During a physical examination, a defined abdominal mass was palpable in the region of the left hypochondrium. Imaging studies revealed an advanced gastric cancer, which was suspected of having infiltrated the abdominal wall. Total gastrectomy and resection of the regional lymph node and abdominal wall were performed. Histopathologic examination of the resected specimen demonstrated xanthogranuloma combined with early gastric cancer.Xanthogranuloma presenting as a form of SMT  of the stomach is an extremely rare disease, and diagnosing it preoperatively is difficult. Further accumulation and investigation of this entity is necessary. Xanthogranuloma was first described by Oberling in 1935 [3/μl. Her tumor marker serum levels were within the normal limits (carcinoembryonic antigen (CEA): 1.3 ng/ml, carbohydrate antigen (CA) 19-9: 10.1 U/ml). A gastrointestinal endoscopic examination was performed and disclosed an ulcerated lesion in the lesser curvature of the gastric corpus at about 7 cm from esophagogastric junction, which squashed and isolated the gastric folds from the rest of the stomach  gastric cancer, and the adjacent tumor had extended into the abdominal wall beyond the gastric serosa Figure . HistopaXanthogranuloma is a tumor that is macroscopically characterized by the formation of multiple golden yellow or bright yellow nodules, and histologically, the lesion is predominantly composed of foamy histiocytes mixed with acute and chronic inflammatory cells. The pathogenesis of xanthogranuloma has not been fully established, although it is thought to be a chronic lesion associated with infection, immunological disorders, lipid transport, and lymphatic obstruction .To the best of our knowledge, only seven cases of xanthogranuloma of the stomach have been reported -8, and tPathologically, stromal tumors such as GIST, myogenetic tumors, and neurogenic tumors account for 54 percent of all SMT, followed by heterotopic pancreas, cyst, lipoma, carcinoid, lymphangioma, and hemangioma . There hIn our case, the gastric xanthogranuloma was preoperatively misdiagnosed as an advanced gastric cancer. This occurred for the following reasons: First, a gastrointestinal endoscopic examination demonstrated an elevated lesion close to the anal side of an ulcerated lesion and a moderately or poorly differentiated adenocarcinoma was detected by the endoscopic biopsy. Second, CT indicated that the elevated lesion had invaded the abdominal wall, and a defined abdominal mass was palpable on physical examination. Therefore, the tumor was recognized as an advanced gastric cancer. Biopsy of the elevated lesion should have been carried out preoperatively to obtain a correct diagnosis in consideration of the coexistence of the two lesions.We report an extremely rare case of gastric xanthogranuloma combined with early gastric cancer. When we find SMT of the stomach, we should bear in mind not only neoplastic tumors but also inflammatory tumors. Further accumulation and investigation of gastric xanthogranuloma cases is necessary.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.HK did the literature search and writing of the manuscript. SY, YS, KA and KM collected the clinical data. RK was responsible for the histology consulting and pathology examination. All authors read and approved the final manuscript.


Primary retroperitoneal masses are a rare but important group of neoplasms. Cross-sectional imaging has revolutionised the investigation of patients with retroperitoneal neoplasms. Both computed tomography (CT) and magnetic resonance imaging (MRI) can contribute to tumour diagnosis, though histological confirmation is often required because of the considerable overlap of imaging features. Cross-sectional imaging is key to the pre-operative staging and planning of retroperitoneal masses, though ultrasound may also help in certain instances. Imaging also helps to select and guide the site to biopsy from these usually large and heterogeneous neoplasms. This article aims to review many of the primary retroperitoneal neoplasms that may be encountered by the radiologist. The retroperitoneum in the abdomen is the space between the posterior parietal peritoneum anteriorly and the transversalis fascia posteriorly. It extends from the diaphragm superiorly to continue into the extraperitoneal space in the pelvis inferiorly. The retroperitoneum is loosely divided into the anterior and posterior pararenal, perirenal and great vessel spaces. The anterior pararenal space is bordered between the posterior parietal peritoneum anteriorly, the anterior renal or Gerota fascia posteriorly and laterally by the lateroconal fascia. This space includes the pancreaticoduodenal space and the pericolonic space. The posterior pararenal space lies between the posterior renal fascia and the transversalis fascia, whereas the perirenal space is located between the anterior and the posterior renal fascia. The great vessel space surrounds the aorta and the inferior vena cava (IVC) and is anterior to the vertebral bodies and psoas muscles. The anterior and posterior pararenal spaces merge inferior to the level of the kidneys, which communicates inferiorly with the prevesical space and extraperitoneal compartments of the pelvis .The pelvic extraperitoneal space includes three intercommunicating potential extraperitoneal spaces: the prevesical, perivesical and perirectal spaces. The prevesical space is bordered by the infraumbilical abdominal wall anteriorly and the umbilicovesical fascia posteriorly, this space is also referred to as the space of Retzius. The perivesical space is below the pelvic peritoneal reflection, and contains the urinary bladder and the uterus . The perirectal space surrounds the rectum and includes the peripheral presacral space .Primary retroperitoneal neoplasms are a rare but important group of neoplasms. They account for only 0.1–0.2 % of all malignancies and arise outside the retroperitoneal organs . Most prOwing to the loose connective tissue of the retroperitoneum, these masses tend to be large at the time of presentation . They caBoth computed tomography (CT) and magnetic resonance imaging (MRI) can contribute to tumour characterisation, in determining the size of the lesion, its tissue content and relationship to adjacent organs and vessels. CT provides superior spatial resolution and detection of calcification, while MRI has superior soft tissue contrast and capabilities in the detection of fat within a lesion. Whilst there does remain significant overlap in the imaging characteristics of retroperitoneal neoplasms, some lesions have distinctive characteristics and can be diagnosed with some accuracy on imaging, e.g. aggressive angiomyxoma and liposarcoma Table . ImagingThe aim of this article is to illustrate many of the primary retroperitoneal masses and to review the associated imaging features.Liposarcoma is the most common sarcoma to occur in the retroperitoneum . RetropeWell-differentiated liposarcomas are typically round or lobulated and may displace or surround local structures. They contain predominantly fat. The fat content may be in excess of 75 % and these lesions must be distinguished from other fat-containing neoplasms which may occur in the retroperitoneum, such as lipoma, adrenal myelolipoma, renal angiomyolipoma and teratoma . ImagingHistological dedifferentiation may occur within a portion of the well-differentiated liposarcoma, resulting in a more aggressive lesion, which is more prone to metastasise and carries a worse prognosis . These lMyxoid liposarcomas tend to occur in younger patients. They are of intermediate-grade malignancy and are typically lobular lesions with attenuation less than that of muscle on CT. The homogeneous dispersal of fat and soft tissue throughout the lesion gives a ‘pseudocystic’ appearance, near fluid density, on non-contrast enhanced CT. They characteristically show gradual, heterogeneous enhancement. Calcification can occasionally be seen. Pleomorphic/round cell liposarcomas are high-grade sarcomas. They are the least common subtype overall. They appear as heterogeneous soft-tissue masses with little or no fat within and may contain areas of necrosis Fig .Leiomyosarcoma is the second most common primary retroperitoneal tumour in adults . LeiomyoAt CT, these masses typically appear as large masses, often in excess of 10 cm. They have attenuation equal to or less to that of muscle and often have internal regions of low attenuation corresponding to areas of necrosis  Fig. 2a. Areas oUndifferentiated pleomorphic sarcoma (UPS), previously called malignant fibrous histiocytoma (MFH), is the third most common retroperitoneal sarcoma . It is tCT and MR imaging appearances are largely non-specific. Both modalities typically show a large, heterogeneously enhancing soft-tissue mass . Areas oSolitary fibrous tumours (SFTs) are a rare group of neoplasms, which arise from the mesenchyme and account for less than 2 % of all soft-tissue tumours . OriginaSFTs of the retroperitoneum are even more rare and only about 50 cases have previously been described in the literature . ClinicaAt imaging, SFTs usually appear as well-defined masses with intense heterogeneous enhancement in the arterial phase, indicating its hypervascular nature, which persists in the delayed phase because of the fibrous component in the mass. Areas of necrosis, haemorrhage or cystic changes may be seen, though calcification is rare Fig. . On MRI,Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumour, which tends to occur at a young age, generally in the 1st–5th decades. This tumour occurs slightly more commonly in male patients. It arises from the dermal layer of the skin and is classified as a sarcoma. These tumours typically arise on the trunk, head and neck region and on the extremities . It can CT appearances that have been described include a well-defined lesion with tissue attenuation equal to or slightly higher than that of skeletal muscle. Moderate enhancement may be seen post intravenous contrast administration .Synovial sarcoma is extremely rare in the retroperitoneum. In the retroperitoneum, it is associated with an extremely poor prognosis. They typically occur in younger patients, aged 15–40. Imaging findings are non-specific and the diagnosis should be considered in a young patient presenting with a mass in the retroperitoneum  Fig. 6)6).Fig. 6At CT, these lesions tend to be hypoattenuating, with peripheral irregular enhancement and central necrosis. Calcification may also be seen. On MR imaging, the heterogeneous mass is typically isointense on T1-weighted imaging and hyperintense on T2-weighted imaging .Myelolipoma is a benign tumour that consists of mature adipose tissue and normal trilineage hematopoietic components. About 15 % arise outside the adrenal gland and the pre-sacral location accounts for 50 % of the extra-adrenal myelolipomas . The preTeratoma is a germ cell tumour with elements from more than one germ cell layer. They are commonly seen in infants and children, and tumours in adults are more likely to be malignant. In adults, they are about 4 times more commonly seen in women , 19.Clinically, they may be asymptomatic or present with mass or pressure symptoms . On imagAggressive angiomyxoma is an uncommon benign mesenchymal tumour. It predominantly involves the pelvis and perineum of women of childbearing age, typically between the 2nd and 4th decades . Most tuThese are one of the few primary extraperitoneal masses which have a distinctive and characteristic appearance on MR imaging. On CT, these tumours are typically of low attenuation relative to muscle. On MRI, these lesions are isointense to muscle on T1-weighted imaging, and hyperintense on T2-weighted imaging (from the high myxomatous component in these tumours) with a distinctive whorled appearance , 21. FolCastleman disease, a benign giant lymph node hyperplasia, is an uncommon benign lymphoproliferative disorder. Castleman disease may occur anywhere along the lymphatic chain, and about 70 % occur in the mediastinum. Extrathoracic sites include neck, axilla, mesentery, pelvis, pancreas, adrenal and retroperitoneum. A slight female predominance is reported in the literature. In the retroperitoneum, they are more common on the left side.There are two major histological types: the hyaline-vascular type, and the plasma cell type. Clinically, the hyaline-vascular type is usually benign, localised and asymptomatic, whereas the less common plasma cell variant may be disseminated, malignant with systemic symptoms such as fever, anaemia, weight loss and night sweats .The characteristic imaging features of localised and hyaline vascular type of Castleman disease include a well-defined solitary mass or a dominant mass with small satellite nodules, showing intense enhancement Fig. . CentralThe plasma cell or disseminated form of Castleman disease may show hepatosplenomegaly, ascites and lymphadenopathy, which generally shows less enhancement than in the localised form .Lymphangiomas are congenital benign vascular lesions that result as a result of sequestration of embryonic lymphatic vessels that fail to communicate with the rest of the lymphatic or venous channels. They occur more commonly in children, though adult clinical presentation is not rare. Less than 1 % of lymphangiomas occur in the retroperitoneum .Lymphangiomatosis is a rare disease with multifocal sites of lymphatic proliferation that are locally infiltrative, with growth along tissue planes , 26. It Neurogenic tumours constitute up to 20 % of the primary retroperitoneal masses . MajoritNeurofibromas are seen more commonly in the 2nd–4th decades, predominantly as isolated sporadic tumours, and are more common in men. In approximately 10 %, neurofibromas may be associated with neurofibromatosis (NF) 1 and 2. NF1 is commonly an autosomal dominant genetic disorder due to mutation of chromosome 17. In up to 50 % of patients, it can be a sporadic mutation. It comprises of hamartomas, hyperplasia and neoplastic lesions of neuroectodermal or mesenchymal origin. There are many clinical manifestations such as neurofibromas, café au lait spots, Lisch nodules and axillary/inguinal freckling. Multiple plexiform neurofibromas, cutaneous neurofibromas and Lisch nodules are specific to NF1 .On CT, retroperitoneal neurofibromas are well-defined, round, hypodense, inhomogeneous solid masses, which may be seen along the expected course of the nerve. Widening of the neural foramina may be present without bony destruction Fig. . These aPlexiform neurofibromas are seen almost exclusively in NF 1. They are hypodense on CT from presence of adipose tissue, myxoid tissue and schwann cells Fig. . They teThere is up to 5 % lifetime risk of malignant transformation of neurofibroma to malignant peripheral nerve sheath tumour (MPNST)—previously known as neurofibrosarcoma Fig.  30]. Th. Th30]. Schwannomas are the most common retroperitoneal neurogenic tumours, comprising around 5 % of all retroperitoneal tumours. They are benign tumours and arise from the nerve sheaths of peripheral nerves and are most commonly located in the paravertebral region. Schwannomas are seen in young adults, but unlike neurofibromas, they are twice as common in women .Histologically, they consist of two different components: Antoni type A areas are highly cellular Fig. , while AGanglioneuromas occur in adolescents and young children. They are rare, benign neurogenic tumours from the sympathetic ganglia and may arise anywhere along the paravertebral sympathetic plexus or occasionally from the adrenal medulla. They are often detected incidentally even if large, or may present with pain or mass on deep palpation. Occasionally they may secrete catecholamines, vasoactive intestinal polypeptide, or androgenic hormones .On imaging, they appear as well-defined lobulated masses. Discrete or punctate calcification may be seen in up to 20 %. Gradual enhancement is usually seen post contrast Fig. . On MRI,Paragangliomas constitute 10 % of all pheochromocytomas arising from the neural crest cells along the sympathetic chain. The organ of Zuckerkandl located along the aorta just superior to the bifurcation is the most common location. The trigone of the urinary bladder usually contains cells of neural crest origin and, therefore, a rare possible site. A significant number produce high catecholamine levels, causing symptoms such as palpitations, tachycardia, flushing, sweating, etc.Paragangliomas may be associated with von Hippel Lindau syndrome, MEN Type II and NF1, and are generally more malignant with higher metastatic rate than their adrenal counterparts (up to 40 % of parangliomas compared with up to 10 % of pheochromocytomas) .On CT, these are large, lobulated and heterogeneous masses due to presence of necrosis, blood and calcification and are mostly hypervascular after contrast administration. On MRI, they are heterogeneously bright on T2-weighted imaging Fig. , though Primary retroperitoneal neoplasms are rare. Cross-sectional imaging is key to the evaluation of retroperitoneal masses and in the pre-operative staging and surgical planning of these lesions. This article has reviewed the imaging findings of many of these masses that may be encountered by the radiologist in their practice.


Synovial sarcoma (SS) is a rare malignant neoplasm that arises most commonly in joint capsules and articular tendons, but its relationship to the synovium is not always obvious. Synovial sarcoma is a malignant soft tissue tumor representing 5.6% to 10% of all soft tissue sarcomas. They are termed SS because of their histologic resemblance to the synovium, but they rarely involve a synovial structure and are thought to arise from pluripotential mesenchymal cells. The tumor usually occurs in close association with tendon sheaths, bursae, and joint capsules, primarily in the para-articular regions of the extremities, with approximately 9% occurring in the head and neck region. Synovial sarcoma has been reported rarely in the oral cavity. We report a very rare case of Synovial sarcoma of the buccal mucosa in a 24-year-old male patient. Synovial sarcoma (SS) is a rare malignant neoplasm that arises most commonly in joint capsules and articular tendons, but its relationship to the synovium is not always obvious. Synovial sarcoma is a malignant soft tissue tumor representing 5.6% to 10% of all soft tissue sarcomas , 3.The tumor usually occurs in close association with tendon sheaths, bursae, and joint capsules, primarily in the para-articular regions of the extremities, with approximately 9% occurring in the head and neck region. Most studies have found that the median age of SS is in the third decade and approximately 66% of the patients are male. The most common sites involved in the head and neck include the hypopharynx, postpharyngeal region, and the parapharyngeal space .The origin of synovial sarcoma remains unknown, but the neoplasm is thought to arise from primitive undifferentiated pluripotential mesenchymal cells unrelated to synovial tissue . SynoviaA 24-year-old male patient reported to the Department of Oral Medicine and Radiology, with a chief complaint of a swelling on the left side of the face since 2 years. Initially swelling was smaller in size which gradually increased to the present size, associated with difficulty in mouth opening and swallowing. There was a history of weight loss over the last few months. On general physical examination, the patient was moderately built and poorly nourished. All the vital signs were within the normal limits.Extra oral examination revealed facial asymmetry with a diffuse swelling on left side measuring 22 × 17 cms in dimension. Swelling was extending from lower eyelid superiorly to 2 cm below the lower border of the mandible inferiorly. Medially it extends from the philtrum and lateral wall of the nose to 2 cm anterior to tragus of the ear. Skin over the swelling was stretched, shiny, and erythematous. The nasolabial fold was obliterated and the patient was not able to close his mouth. Swelling was firm to hard in consistency and nontender on palpation  tumors [Patient with a head and neck sarcoma might present with dysphagia, hoarseness, or headache, depending on the plane of spread and site of origin of the tumor. In a large study of 672 cases, males and females were affected equally . The gro) tumors , 15.Variants of synovial sarcomas (SS) are subclassified into three groups: (i) monophasic epithelial type; (ii) monophasic spindle cell type; and (iii) biphasic type with distinct epithelial and spindle cell components. Regardless of the subtype, the conventional clues and immunohistochemical evidence of the epithelial element are characteristic of synovial sarcoma, and have led to the consensus that this tumor should be regarded as a carcinosarcoma of soft tissue origin . In addiIn the present case, histopathological picture showed sheets of neoplastic cells having round hyperchromatic nuclei with scanty discernible cytoplasm. Some cells had clear cytoplasm. Immunohistochemistry revealed that tumor cells express Vimentin, Bcl-2, and Mic-2 with crisp cytoplasmic positivity suggesting the diagnosis as poorly differentiated small cell variant synovial sarcoma. Bcl-2 protein is regularly expressed in synovial sarcomas, and CD99, the product of the MIC2 gene, is seen in 67% of all cases .Wide surgical resection is the primary treatment option for SS. Because complete excision of intraoral tumors is not always possible, a multimodal therapeutic approach consisting of extensive radical local excision, postoperative radiation therapy, and chemotherapy is often recommended. The metastatic potential (29.2%) and recurrence rate (20.8%) of the oral tumors appeared to be lower than those of synovial sarcoma originating at other sites . MultiplTumor recurrence typically manifested in the first 2 years after initial therapy. Prognosis is generally poor  . PrognosIn the present case, the prognosis was poor; patient expired within a year after diagnosis. Synovial sarcoma is rare in the oral cavity, a feature that may increase the potential for misdiagnosis. These tumors should be considered in histopathologic differential diagnosis of both malignant primary and metastatic spindle cell tumors of the oral cavity.The rarity of the present case is very rare malignant soft tissue tumor representing 5.6% to 10% of all soft tissue sarcomas, with approximately 9% occurring in the head and neck region, its aggressive nature, and its tendency to metastasize to other sites. This emphasizes on the early diagnosis and management of synovial sarcoma.


Cementoblastoma is an uncommon disease, representing only 1–8% of all odontogenic tumours. Furthermore, this tumour is especially uncommon in children, as only five cases have been reported in this age group. Here, we describe a case of cementoblastoma arising in the maxilla of an 8-year-old boy, that was treated with a partial maxillectomy. The patient's facial appearance has remained satisfactory, and the tumour has not recurred in the 9 years after the operation. Cementoblastoma, characterized by the formation of hard cemental tissue contiguous with a dental root, is an uncommon mesenchymal neoplasm, representing 1% to 8% of all odontogenic tumours . ReporteThe patient was an 8-year-old boy. He was referred to our department on August 24, 2001 for evaluation of a hard mass in the right maxilla. He had noticed the asymptomatic mass 3 months before. The patient had no significant past medical history. Oral examination revealed a firm, painless mass located on the posterior hard palate, contiguous with the maxillary first molar . The les For the operation, an incision was performed through the mucosa of the gingivobuccal sulcus and the paramedian surface of the hard palate. The periosteum of the lateral maxilla was preserved. The approach for the bone cut was through the mucosal incision. The mucosa of the residual maxillary antrum that showed inflammatory changes was curetted out, and the tetracycline ointment gauze was introduced. A previously fabricated dental obturator was set. The postoperative course was uneventful, and the dental prosthesis was frequently adjusted by our maxillofacial prosthodontist. Nine years after the operation, the right corner of the patient's mouth was slightly higher than the left corner, because of scarring, but there have been no developmental disorders of the right maxillary region , and theMacroscopically, the surgical specimen consisted of 3.5 × 3.3 × 3.0 cm, a rounded hard tissue mass confluent with the first molar tooth roots . Microsc Cementoblastoma has a propensity to develop in the mandible, most commonly in the molar region (80~95%). The tumour size ranges from 0.5 to 5.5 cm, and the average is 2.1 cm . Some pa Brannon et al.  reported The treatment in most cases of odontogenic tumours in children should be the same as for those in adults. However, lesions developing in childhood grow relatively more rapidly and have unlimited growth potential , 8, 11;  In this paper, we describe a case of cementoblastoma in an 8-year-old boy. This patient has retained a satisfactory appearance without tumour recurrence for the 9 years after his operation. The authors declare that there are no conflict of interests.


The case presented here illustrates a 75 year old female patient who underwent surgical resection of a right adrenal mass of uncertain nature. The final histological diagnosis was consistent with leiomyosarcoma arising from the adrenal anatomic site.Primary leiomyosarcoma of the adrenal gland is a very rare malignant mesenchymal neoplasm: to our knowledge, this is only the twelfth case reported in literature.We describe the clinical course and a brief review of clinical and histological features, biologic behaviour, diagnostic approaches and therapeutic strategies. A 75-year-old white female was admitted to the Department of Urology of our hospital following the ultrasound finding of a right adrenal mass of uncertain nature. Her medical history was unremarkable, except for long-term essential arterial hypertension. For a month prior to hospitalization the patient had complained of persistent and progressive epigastric and right loin pain. Physical examination showed only mild abdominal discomfort at palpation. No enlarged lymph nodes were present. Laboratory tests detected no abnormalities. Urinary levels of vanilmandelic acid, catecolamines and plasmatic level of aldosterone were normal.Chest radiograph and electrocardiogram revealed no pathological findings. Upper gastrointestinal endoscopy detected oesophageal hiatal hernia with reflux oesophagitis, and total-body CT confirmed the presence of a right adrenal mass of about 5 × 4 cm, suspected to be an adrenal carcinoma, so radical surgery was planned and performed with the resection of a roundish formation from the adrenal anatomic site. Normal adrenal gland was not visible .Three days after surgery the patient developed a pulmonary embolism: an inferior vena cava thrombosis was identified by angio-CT scan. Pulmonary embolism was identified by angio-CT scan, too. Moreover, an unexplained progressive drop of hemoglobin concentration down to 7.7 g/dl, requiring infusion of two units of packed red cells, was observed. The patient was transferred to our Intensive Care Unit, where she received routine treatment for pulmonary embolism including anticoagulant therapy first with intravenous heparin and then with warfarin.The clinical course was satisfactory, and subsequent Doppler-ultrasonography confirmed the return to normal of venous flow through inferior vena cava. The patient, clinically stable with a good cardio-respiratory compensation, was discharged on postoperative day 18; continuation of anticoagulant therapy and physical rehabilitation were recommended. No oncological treatment was provided after surgery, and no evidence of recurrent disease was observed one year later.Gross pathological examination of the resected tissue showed a roundish, fibrous neoformation of 8 × 4 × 5 cm. A thrombus was detected inside the adrenal vein; this thrombus was not visible on CT performed before surgery. On sectioning of the mass, no adrenal structure was recognizable. Resection margins were free of disease. Histologically, tissue sections showed a neoplasm consisting of elongated cells with eosinophilic cytoplasma and elongated nuclei with rounded ends, small nucleolus with moderate polymorphism; the cells are arranged in fascicles; on average, 16/50 HPF mitoses were observed. No necrotic areas were detected .Neoplastic cells were strongly and diffusely positive immunohistochemical staining for desmin, SMA1A4)(-Smooth Muscle Actin (1A4) Rimary Antibody) and actin (HHF3 Muscle-Specific Actin -HHF35- Primary Antibody 5-) ; immunohA4(-Smoot2Intra-abdominal soft-tissue tumours represent only 0,1%–0,2% of all adult malignancies; among them leiomyosarcoma is the most common histological subtype, accounting for approximately 25%–30% of cases. However, primary leiomyosarcoma of the adrenal gland is a very rare malignant mesenchymal tumour: to our knowledge, this is only the twelwfth case reported in literature.12The aetiology of these neoplasms is unclear. A growing incidence of smooth muscle tumours in HIV and Epstein-Barr virus positive patients has been reported, but nothing certain is known about the pathogenetic involvement of these viruses.At present, the prognosis for leiomyosarcoma patients is not predictable. Survival depends on tumour size, location, feasibility of complete resectionBecause of the prolonged time interval separating the diagnosis of the primary tumour and the involvement of distant sites, an accurate and early diagnosis of these neoplasms must be made. Indeed, improved diagnostic approaches are enabling the early diagnosis and surgical treatment of an increasing number of cases. Radical surgical resection remains the only proven therapeutic modality that prolongs the survival in patients with leiomyosarcoma.Postoperative adjuvant radiation therapy is recommended for the treatment of locally advanced malignancy.23Conclusively, complete surgical resection followed by a combination of radiotherapy and chemotherapy may constitute the optimal treatment for advanced leiomyosarcoma to ensure long-term survival, even if recurrences are frequent.Treatment was therefore limited to surgical resection with no oncological treatment provided after surgery, and no evidence of recurrent disease was observed one year later.


The case we report shows rapid progression and a very poor prognosis only for a month that differs from the clinical course reported in the literature. An 83-year-old man was referred to our hospital for macroscopic hematuria. Computed tomography (CT) revealed a large bladder tumor measuring 4 cm × 3 cm and magnetic resonance imaging revealed extravesical invasion and pelvic wall invasion of the tumors. Chest CT and bone scintigraphy revealed no evidence of distant visceral metastases, and a clinical diagnosis of T4N0M0 was made. Transurethral resection of the bladder tumor (TUR-BT) was performed for histopathological diagnosis 18 days after admission, and no further adjuvant treatment was given. At 15 days after TUR-BT, the patient's clinical status worsened with symptoms of exertional dyspnea. CT showed multiple metastatic lesions in the lung, liver, and retroperitoneal lymphadenopathy. The patient died 2 days later and underwent autopsy. A final histopathological diagnosis of leiomyosarcoma was made based on immunohistochemical staining. Nonepithelial urinary bladder tumors account for less than 5% of bladder malignancies overall, with leiomyosarcoma comprising 0.1% of all bladder cancers [An 83-year-old man was referred to our hospital for macroscopic hematuria and dysuria, and his general condition was poor. Cystoscopy revealed a large broad-based non-papillary bladder tumor on the right bladder wall. Computed tomography (CT) revealed a large bladder tumor measuring 4 cm × 3 cm and right hydronephrosis , and magα-smooth muscle actin and vimentin and negative staining for cytokeratin  was performed for histopathological diagnosis 18 days after admission, and Leiomyosarcoma was suspected histopathologically. Because of his advanced age and poor performance status, radical cystectomy was not available and no further adjuvant treatment was given. At 15 days after TUR-BT, the patient's clinical status worsened with symptoms of exertional dyspnea. CT showed multiple metastatic lesions in the lung, liver, and retroperitoneal lymphadenopathy. The patient died 2 days later and underwent autopsy. The autopsy revealed macroscopic multiple metastatic lesions in the bilateral lungs, pleura, diaphragm and liver, and retroperitoneal lymphadenopathy. A final histopathological diagnosis of leiomyosarcoma was made based on immunohistochemical staining, which revealed positive staining for  Figures .Urinary bladder leiomyosarcoma is relatively rare, with few large series reported in the literature. Rosser et al.  reportedUrinary bladder leiomyosarcomas have always been considered highly aggressive tumors that require aggressive surgical extirpation, and radical cystectomy with wide margins should be performed whenever possible , 5. NeoaSome studies suggest that patients with bladder leiomyosarcoma with low mitotic activity (<5 per 10 HPF) and mild-to-moderate nuclear atypia have a good prognosis, whereas those with higher mitotic activity (≧5 per 10 HPF) have a worse prognosis . In our


Histological examination of specimens after subtotal removal revealed a primary mixed germ cell tumor with extensive yolk sac tumor elements, often referred to as an intracranial “yolk sac tumor.” The preoperative diagnosis of NGGCTs in unusual age and locations is extremely difficult. Clinicians should consider the possibility of NGGCTs, including yolk sac tumors, when intracranial tumors with unusual MRI findings are encountered.Intracranial nongerminomatous germ cell tumors (NGGCTs) in unusual locations are extremely rare. Here, we report a case of a yolk sac tumor in the frontal lobe in a middle-aged patient. A 42-year-old man was admitted to our hospital for headache and nausea. Magnetic resonance imaging (MRI) showed an enhanced mass lesion with a marked cyst component. The serum alpha-fetoprotein ( Nongerminomatous germ cell tumors (NGGCTs) of the central nervous system (CNS) are rare, accounting for fewer than 1% of primary brain tumors, and they occur predominantly in children or young adults . Twelve A 42-year-old Japanese man was admitted to our hospital for headache and nausea. These symptoms had progressed for 4 days before admission, and he was gradually unable to walk steadily. Magnetic resonance imaging (MRI) revealed a mass lesion with marked cyst components. The lesion had a maximum diameter of 58 mm . T1-weigαFP); partially positive for CD117 (C-kit); negative for glial fibrillary acidic protein (GFAP), S-100 protein, CD30, and the beta subunit of human chorionic gonadotropin (βHCG) (αFP (55500 ng/mL) observed before surgery, which was confirmed after surgery. A testicular ultrasound and a computed tomography (CT) scan of the chest and abdomen showed no lesions outside of the brain, and the final diagnosis was a primary mixed germ cell tumor of the CNS with extensive yolk sac tumor elements, often referred to as a “yolk sac tumor.” The patient underwent 5 courses of chemotherapy with 900 mg/m2 of ifosfamide, 20 mg/m2 of cisplatin, and 60 mg/m2 of etoposide along with 30.6 Gy of radiotherapy to the whole brain and a local boost of 30.6 Gy. MRI after treatment showed a reduction in the size of the lesion  . These re lesion . The serreatment . The patThe authors retrospectively speculate that the tumor in the present case originated from the basal ganglia (most likely from the caudate nucleus) and extended into the frontal lobe. Detailed descriptions of intracranial yolk sac tumors in the frontal lobe or the basal ganglia have been published for only 7 cases, including our case –10. In aαFP level will be the most useful marker for preoperative diagnosis. However, this value was measured before surgery in only 5 cases (23%) out of the previous cases of CNS yolk sac tumors located in unusual areas [αFP level, which was evaluated for the differential diagnosis of metastatic brain tumors along with other tumor markers, was extremely high, and the αFP level decreased after surgery. In the present case, the preoperative diagnosis by neuroradiologists was GBM or metastatic brain tumor. However, retrospectively, various MRI findings including the existence of an enhanced nodule with cystic components lacking severe peritumoral edema and the very low peak for NAA on 1H-MRS were unusual for GBM. Similarly, in the previous cases of primary intracranial yolk sac tumor in unusual locations, the preoperative diagnosis was difficult . The autal areas , 10–12. In conclusion, the preoperative diagnosis of NGGCTs in unusual age and locations is extremely difficult. Clinicians should consider the possibility of NGGCTs, including yolk sac tumors, when patients present with intracranial tumors with unusual MRI findings.


Histopathological examination showed an oral mucosa fragment with proliferation of striated muscle bundles admixed with small blood vessels, collagen, and nerve fibres. A supplementary analysis with immunohistochemistry demonstrated positivity for desmin, HHF35, smooth muscle actin, S-100, and CD34. Based on these findings, the lesion was diagnosed as rhabdomyomatous hamartoma. The aesthetic result has been very satisfactory after a 14-month followup.Rhabdomyomatous hamartoma is a rare disease that occurs predominantly in the skin. This paper describes a congenital lesion in a 17-year-old male, who came to our clinic presenting a circumscribed swelling involving the oral mucosa and vermillion border of the upper lip, purplish in color, and blanching under pressure. The patient reported that he had had lesion since his birth. A clinical diagnosis was of congenital haemangioma, and the patient was treated by photocoagulation using diode laser. When the lesion became smaller, by having its blood content reduced, the upper portion of the lesion was sliced off with CO Rhabdomyomatous hamartoma (RH) is a rare, congenital lesion with only a few reports (approximately 25 cases), most of them affecting the skin, particularly of the face and neck , 2. Lesi2 lasers.This paper describes a case of RH on the upper lip, diagnosed with the aid of immunohistochemical analysis, followed by largely successful treatment with diode and COA 17-year-old male came to our clinic for treatment of a circumscribed swelling on the mucosa and vermillion border of the upper lip Figures , 4 cm in2 laser beam parallel with the mucosa surface and sliced off the top of lesion with one single cut. Subsequently, the tissue was sent for histopathological and immunohistochemical examinations.A clinical diagnosis of congenital haemangioma was made and the patient was treated under local anesthesia by photocoagulation using diode laser at 830 nm with power set at 3.5 W. Irradiation was delivered by means of a flexible quartz fiber that was kept 2-3 mm away from the lesion, in continuous wave mode for 10–20 seconds. This surgical procedure was repeated 3 times at 30-day intervals, thus allowing for recovery of the patient and shrinking of the lesion between sessions. When the lesion became smaller, by having its blood content reduced, the upper portion of the lesion was removed by orienting the COThe aesthetic result has been very satisfactory after a 14-month followup, with only a discrete swelling appearing on the lip Figures .The histological examination (HE) revealed oral mucosa covered by squamous epithelium with proliferation of striated muscle bundles admixed with small blood vessels, collagen, and nerve fibres. No cytological atypia, necrosis, or mitosis was observed Figures . ImmunohHamartoma, a term coined by the Greek , is used in the medical literature to describe a disordered arrangement of normal elements forming a tumor-like lesion , 3. HistThe clinical form most often reported in the literature of RH is a solitary papule or nodule, occurring particularly on the skin in the region of the head and neck . In the The treatment yielded a great improvement of the lesion, and the patient felt very pleased with the aesthetic result, reporting little concern about the remaining slightly bluish-swollen aspect of his left upper lip.In the present case, the histopathological analysis coupled with immunohistochemical assay was highly instrumental for disclosing the congenital origin of the lesion while the high-power lasers treatment provided a safe and effective resolution, largely successful and without scarring.


Osseous involvement of Hodgkin's lymphoma is uncommon. When osteolytic lesions are seen on imaging it is important to evaluate potential other causes.We report the case of a 10-year-old Caucasian boy who presented to our facility with a bony lesion of the right clavicle and enlarged cervical lymph nodes. A simultaneous biopsy of the lymph node and of the osteolytic process of his right proximal clavicle was performed and revealed two different kinds of lesions: a mixed cellularity Hodgkin's lymphoma and an osteochondroma.Since the latter is a common benign bone tumor, which should not interfere with the staging of the lymphoma, we emphasize the importance of ensuring that all efforts are made to acquire a diagnostic biopsy of all atypical lesions. Lymphoma is the third most common childhood malignancy following leukemia and brain tumors, accounting for approximately 12% of childhood cancers. Two-thirds of lymphomas diagnosed in children are non-Hodgkin's lymphomas (NHL), with the remainder being Hodgkin's lymphomas (HL). Anatomic extent of disease and tumor burden at presentation are significant factors determining choice of therapy and prognosis. HL typically involves the lymphatic system, and is usually supra-diaphragmatic. HL often follows a pattern of contiguous spread from one nodal group to the next anatomical region. Extra-nodal involvement is more common in NHL. Extra-nodal invasion of adjacent tissues is seen in up to 15% of cases and hematogenous spread in up to 10% of newly diagnosed cases. Osseous localizations have been described in 10% to 20% of cases of relapsed or refractory HL, but less than 2% at the time of initial presentation. Here, we describe a case of an osteochondroma (OC) in a child with Hodgkin's disease not affecting therapy or prognosis.Due to enlarged lymph nodes in his right neck region, a 10-year-old Caucasian boy underwent ultrasonic investigation and was treated with a short course of antibiotics 18 months prior to his presentation at our facility. Two months before his current admission, our patient reported local pain and enlargement of the same area in the neck. No B symptoms were evident. A second antibiotic treatment was prescribed, presuming a diagnosis of lymphadenitis at that time, and all laboratory tests were within the normal range except a slight microcytic hypochromic anemia .Plain X-rays of the chest showed no abnormal findings. An ultrasonographic study of the neck showed enlarged lymph nodes (measuring up to 3.2 cm in diameter) on the right side, mostly in the posterior triangle. A computed tomography (CT) scan of the neck, thorax and abdomen confirmed a heterogeneous mass of enlarged lymph nodes on the right side of the neck and an osteolytic process accompanied by a periosteal (and soft-tissue) reaction in the right proximal clavicle, conspicuous for a tumor or chronic osteomyelitis Figure . RadionuBiopsies of a lymph node in the posterior triangle on the right side of the neck and from the right proximal clavicle were taken. The biopsy of the lymph node confirmed the diagnosis of Hodgkin's lymphoma with mixed cellularity and a focal inter-follicular pattern. Immunohistochemistry stains were positive for CD15 and CD30. The clavicle biopsy showed bone tissue with exophytic cartilaginous tissue Figure , consistOsteocartilaginous exostoses (osteochondromas (OC)) are the commonest of all bone tumors, compromising about one-third of benign bone tumors. They may be sporadic, genetic or secondary . The majority are solitary, mostly located around the knee and in the proximal humerus, while about 20% arise in the axial skeleton. Typically, they are metaphyseal or metadiaphyseal in origin, oriented away from the adjacent joint. OC may occur at multiple sites, particularly in cases of hereditary multiple exostoses syndrome (HMES) with an autosomal dominant inheritance. Most children are asymptomatic. OC usually presents as a solitary mass, as a fracture or as a mechanical osteoarticular complication (deformity or joint dysfunction). Plain radiography is the mainstay of imaging for OC, while a CT scan can be helpful when planning resection. An MRI scan is needed when there are concerns of a malignancy. Bone scans are highly sensitive, but with low specificity, and are in general not useful. Histology is necessary to confirm a diagnosis of OC, showing a cartilage-capped exophytic, sessile or pedunculated projection that has a continuous peri-osteum, cortex and marrow connecting with the underlying bone. The cartilage cap is the site of active growth and the degree of maturity is parallel with the host bone. It is not common for OC to grow beyond skeletal maturity. The risk of malignant transformation to chondrosarcoma of a solitary OC is 1% to 5%, but for HMES the reported incidence is up to 9% [Although presenting symptoms due to osseous involvement rarely occur in HL, osseous involvement is seen at radiography in approximately 20% of affected patients throughout the course of the disease . The roeWe describe a case of coinciding osseous lesion and HL. We conclude that all efforts should be taken to make an exact diagnosis by biopsy of all suspicious locations, including bony structures, in order to make an accurate diagnosis and subsequently start the appropriate treatment.Written informed consent was obtained from the patient's legal guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.MA was responsible for the data collection, obtaining consent, and was the author of the manuscript. VD was responsible for the imaging and part of the Discussion section. AM was responsible for carefully reviewing the article. JK was responsible for the medical care of our patient and for part of the Discussion section. All authors read and approved the final manuscript.


About 2% of all paragangliomas are located in the chest, and a few have been described to be found in the heart. Primary cardiac paragangliomas are extremely uncommon tumors and surgical experience with this neoplasm is limited. Treatment strategies described in the literature have included simple excision, excision with reconstruction, autotransplantation after excision of the tumor and even orthotopic cardiac transplantation, depending on the extent of disease. A primary retrocardiac paraganglioma catecholamine-productive was identified in an asymptomatic 49–year old female associated to familial pheochromocytoma-paraganglioma syndrome caused by germline mutation of the gen which codifies for the subunit B of succinate dehydrogenase enzyme (SDHB). The neoplasm was surgically excised from the posterior surface of the left atrium via median sternotomy using cardiopulmonary bypass. Direct ligation of feeding vessels of the tumor along with left atrial reinforcement using a pericardial patch was performed. The post-operative course was uneventful, with normalization of catecholamine secretion and no recurrence at three-month follow-up. We review the current literature about this exceptional cardiac tumor, pathophysiological conditions and options for surgical management. Pheochromocytomas are catecholamine-producing neuroendocrine tumors arise from the chromaffin cells of the embryonic neural crest. It is estimated that the annual incidence is approximately 0.8 per 100.000 persons and they occur most often in the fourth to fifth decade [About 85–90% of them develop in the abdomen  and only 10% originates from extra-adrenal sites. Extra-adrenal pheochromocytomas  originate in the ganglia of the sympathetic nervous system and most are located within the abdomen , head and neck , urinary bladder (1%) and mediastinal compartments (less than 1%) .Thoracic paragangliomas are the most uncommon location and they can be divided into two groups: those located in the anterior mediastinum (arise from parasympathetic paraganglia) and located in the posterior mediastinum (sympathetic chain). Primary cardiac pheochromocytomas (paragangliomas) are extremely rare, occurring in only 0.001%–0.003%, with female predominance [In general terms, the clinical manifestations of these tumors are varied, not particularly specific and it depends on the secretion of excessive amounts of catecholamines, such as noradrenaline (norepinephrine) and adrenaline (epinephrine). Although these tumors may be a cause of hypertension, they are the underlying cause of only about 0.01% of cases of high blood pressure.About 75% of pheochromocytomas are sporadic and the remaining 25% are hereditary. Mutations of the genes which codify for the subunits D, A, C y B of succinate dehydrogenase enzyme  involved in the Krebs cycle can lead to tumorogenesis in chromaffin cells and they have been identified as causing familial adrenal pheochromocytoma and extra-adrenal paraganglioma; moreover, these tumors may also occur in the multiple endocrine neoplasia (MEN) syndrome type 2A and 2B, von Hippel-Lindau disease or von Recklinghausen’s neurofibromatosis .The diagnosis of these tumors can be established by measuring catecholamines and metanephrines in plasma or 24-hour urine along with imaging techniques such as computed tomography (CT), magnetic resonance imaging, iodine-123-marked metaiodobenzylguanidine (MIBG) scan and positron emission tomography (PET) scan .Treatment of pheochromocytomas involves blocking the effect of catecholamines with an alpha adrenergic blocker and after, it then becomes safe to surgically remove the tumor. Although surgical resection is the treatment of first choice in most part of cases, extra-adrenal localizations (such as those paragangliomas originated in cardiac structures) involve an uncommon and complex surgical approach for a complete removal.We describe a case of surgical excision of a primary functioning retrocardiac paraganglioma associated with hereditary syndrome and we review the relevant literature about the peculiar features and surgical strategies for removal of this cardiac neoplasm.We report a case of an asymptomatic 49-year old Caucasian female, with hereditary type 4 pheochromocytoma-paraganglioma syndrome caused by germline mutation  of the gen which codifies for the subunit B of succinate dehydrogenase enzyme (SDHB) of the mitochondrial complex II. She had a family history of extra-adrenal paraganglioma: genetic testing had revealed the gen SDHB mutation for eight first- and second-degree relatives and carotid body paraganglioma had been resected in three of them. Urinary and plasma levels of catecholamines  and their metabolites (normetanephrine) were elevated and computed tomography of the abdomen ruled out adrenal gland involvement.18F-dihydroxy-phenyl-alanine (18F-DOPA) PET scan showed a primary retrocardiac mass with metabolic activity. Metastasic disease was not found and no other intrathoracic masses were identified. Transesophageal echocardiography (TOE) and TC imaging of the heart showed the large mass (40 × 20 mm), extrinsic to the heart, on the posterior wall of the left atrium (LA)  was instituted before operation for the catecholamine-secreting tumor. She underwent surgical removal of the retrocardiac tumor via median sternotomy. As part of our initial surgical planning, cardiopulmonary bypass (CPB) was started through right femoral artery and venous cannulation  Figure A-B becauDirect ligation of feeding vessels was performed for control of bleeding: the collaterals vessels from the right coronary artery (RCA) and circumflex artery (CxA) were ligated Figure C-D. TOE Following excision of the superior pericardial reflection Figure A, the leCPB time was 143 minutes and aortic cross-clamping time was 105 minutes. Intraoperative TOE revealed a complete excision of the tumor and no perioperative hypertensive crisis on palpation of the tumor were demonstrated. The patient was then weaned from bypass successfully.Histological examination confirmed the paraganglioma and malignant changes were not found Figure B-C. Chroth post-operative day and short-term anticoagulation was started for prevention of LA thrombus formation due to transient endothelial dysfunction. At 3–month follow-up, she remains asymptomatic and biochemical evaluations have confirmed the normalization of levels of catecholamines.The post-operative course was uneventful, no bleeding was detected, sinus rhythm was maintained and antihypertensive therapy was not necessary. She was discharged at 8Cardiac paraganglionic tumors are rare neoplasms derived from the neural crest in the mediastinum, associated with the autonomic nervous system, which account for 0.3% of all mediastinal neoplasms and less than 1% of all primary cardiac tumors . They arEdwin Besterman  was the first author to report a successful resection on CPB of a cardiac pheochromocytoma in the LA in 1974 ,9 and thClinical presentation depends on the functional status of the paraganglioma and the location at the heart: paroxysmal hypertension due to secretion of catecholamines, chest pain , symptomIn a short number of patients, cardiac paragangliomas can be associated to hereditary syndromes such as the Carney triad   or familMost cardiac paragangliomas are benign, but local invasion can occur and metastases have been reported in isolated cases ,21. LocaThe preoperative goals for this type of tumors are to normalize hemodynamic variables such as blood pressure and avoid surges in catecholamine release with tumor manipulation during surgery. Nevertheless, preoperative alpha-1-adrenoceptor antagonist seems to have no benefit in maintaining intraoperative hemodynamic stability in patients with normotensive adrenal pheochromocytoma . AlthougLess than 40 cases about surgical excision of a cardiac paraganglioma have been described in adults  and the Because they are highly vascular, some groups advocate the embolization before surgery to reduce perioperative bleeding  when surNevertheless, in our patient, for prevention of a catastrophic haemorrhage during the time of the operation, a direct ligation of feeding vessels of the tumor was performed. To date, isolated cases of direct ligation at surgery have been described  and no cAs these tumors are of locally invasive nature and they have a less well-defined capsule than adrenal pheochromocytomas, a complete resection can be technically difficult by the position of the tumor and should be performed on CPB and cardioplegic arrest in the most part of cases in order to achieve disease-free excision margins. When the interatrial septum is involved, the anterior aspect of the septum must be preserved to avoid heart block; in other situations, the tumor invades surrounding vascular structures and resection may be extremely difficult. For selected patients, extensive cardiac reconstructive surgery may therefore be required, with its associated risks. Our patient underwent a successful complete resection of the “caged” tumor between the pulmonary veins along with reconstruction of the resected site and no complications were detected after surgery.Reconstruction of the LA using either autologous or bovine pericardial patch has been previously described and, when a portion of the pulmonary veins is also infiltrated and excised, it must be repaired and reconstructed with the patch , althougAlthough surgical resection and reconstruction is the gold standard treatment for these tumors, this approach can be impossible due to extensive involvement of the great vessels or other structures of the heart. When total surgical resection is extremely difficult, cardiac autotransplantation has been described and, in selected cases, when a complete removal is not possible, orthotopic cardiac transplantation is a feasible technique in those paragangliomas that have direct coronary artery involvement, extend into the left ventricle or invade the atrioventricular groove . In geneIn the only reported case series including 14 patients over the last 30 years, at Mayo Clinic [When a complete removal of the tumor is achieved, long-term prognosis seems to be excellent and survival of up to 14 years has been reported in a patient with excised mediastinal paraganglioma . To dateOur case is relevant in that there were no classic signs of catecholamine excess despite to be a functioning tumor and it illustrates successful management of a benign left atrial primary paraganglioma associated with SDHB mutation, including direct ligation of feeding vessels of the tumor, complete excision and subsequent reinforcement of the LA, without evidence of recurrence at short-term follow-up.– Primary cardiac paragangliomas are exceptional tumors and surgical resection is the gold standard treatment.– Control of feeding vessels, complete resection of the tumor and reconstruction of the resected sites are the goals for surgical management.– Although treatment strategies can be varied according to the extent of the disease, in general terms, cardiac paragangliomas can be safely resected using well-established surgical techniques with appropriate surgical planning.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.18F-dihydroxy-phenyl-alanine positron emission tomography scan;TOE: Transesophageal echocardiography;LA: Left atrium;CPB: Cardiopulmonary bypass;RCA: Right coronary artery;CxA: Circumflex artery;SVC: Superior vein cavae;RA: Right atrium;Ao: Aorta;CS: Coronary sinus;LPVs: Left pulmonary veins;IVC: Inferior vein cava;LIPV: Left inferior vein cava;LAA: Left atrial appendage;OS: Oblique sinus;H & E: Haematoxylin and eosin;C & S: Chromogranin and synaptophysinSDHB: Subunit B of succinate dehydrogenase enzyme;SDHD: Subunit D of succinate dehydrogenase enzyme;SDHA: Subunit A of succinate dehydrogenase enzyme;SDHC: Subunit C of succinate dehydrogenase enzyme;MEN: Multiple endocrine neoplasia syndrome;CT: Computed tomography;MIBG – scan: Iodine-123-marked metaiodobenzylguanidine – scan;PET – scan: Positron emission tomography – scan;(18F-DOPA) PET scan: The authors declare that they have no competing interests.MTGL: Design, data collection, photographs and writing article. SGG; ESG; SGR; and JGL: Critical revision and helped to draft the manuscript. All authors read and approved the final manuscript.


The dorsal root ganglion is a rare manifestation of metastatic spread. We report what we believe to be the first case of metastasis of a pulmonary adenocarcinoma to the lumbar dorsal root ganglion. Only four descriptions for different primary tumors spreading to the dorsal root ganglion have been described in the literature so far.A 70-year-old Caucasian woman with a four-month history of left-sided lumbar radiculopathy was admitted to our department under the assumption of a herniated lumbar disc. Her past medical history included a pulmonary adenocarcinoma and invasive ductal breast cancer.Lumbar magnetic resonance imaging revealed a space-occupying mass in her left neuroforamen L3-L4 with compression of her L3 nerve root. Neurinoma was taken into account as a differential diagnosis, although not considered typical. Surgery revealed a metastasis of pulmonary adenocarcinoma to her dorsal root ganglion.Dorsal root ganglion metastases seem to be extremely rare and can mimic primary local nerve sheath tumors. Therefore, they usually present as incidental findings. Resection should be performed strictly under intraoperative monitoring as tumor spread between the nerve fibers is commonly observed. Metastases should be taken into account in spinal nerve tumors involving the dorsal root ganglion, especially in patients harboring known malignant diseases. The low incidence means that no clear treatment advice can be given. Resection is possible under intraoperative monitoring and relieves neurological symptoms. Dorsal root ganglion (DRG) metastasis has been rarely described in the literature. Up to now, only four cases in three publications are listed, comprising two cases from autopsy series and two case reports. The underlying pathologies include ductal breast cancer, pulmonary oat-cell cancer, renal cell carcinoma and uterine carcinoma -3. AlthoA 70-year-old Caucasian woman with a four-month history of left-sided lumbar radiculopathy was admitted to our department under the assumption of a herniated lumbar disc. Her current pain level was 7 out of 10 on the visual analogue scale. Walking was still possible using crutches. In a clinical examination, a left-sided hip flexor paresis, paresthesia and numbness in the distribution of her left L3, and a loss of the patellar reflex were seen. Her past medical history included a pulmonary adenocarcinoma (T2 N1 G2 R0 M0) treated by partial pulmonary resection on her left side 13 years before, and invasive ductal breast cancer , treated by right-side mastectomy and sentinel lymph node dissection five years before. In clinical follow-up, no local recurrence or metastases were seen.Lumbar magnetic resonance imaging revealed a space-occupying mass in her left neuroforamen L3-L4 with compression of the L3 nerve root Figure . Gadolinin situ stimulation signaled disseminated active fibers in the tissue, impeding further dissection and complete tumor removal. An extended decompression of the ganglion and nerve root was performed following subtotal tumor removal.Due to the intraforaminal location of the lesion, a dorsolateral minimal invasive approach was used. Following conservative decompression and opening of the neuroforamen, only residual dural sheath was found. An unclear structure filling her neuroforamen was identified as a swollen ganglion by following its course. Nerve fibers were splayed with interjacent dense tissue Figure . No saliOur patient recovered in time and had no complaints of radicular pain post-surgery. Her flexion paresis was not completely normalized but significantly improved.A histological examination revealed a metastatic lesion consisting of epithelial tumor cells, which grow in small nests between large ganglion cells Figure . ImmunohStaging included cerebral and whole spine magnetic resonance imaging. An additional C7 osseous metastasis and a muscular metastasis in her right gluteal were seen. No additional metastases in her central or peripheral nervous system were found. Following interdisciplinary discussion, our patient was referred for radiochemotherapy, including fractionated radiation of the L2-L5 segment with a cumulative dose of 40Gy. Despite intensive treatment, our patient developed rapid clinical deterioration in the following weeks and was diagnosed with meningeal carcinomatosis 12 weeks after surgery. She died two weeks later.DRG metastases are rare and may be incidental findings in surgery for herniated discs, dorsal root neurinoma or spinal meningioma. This has to be taken into account in unanticipated intraoperative findings, including normal disc anatomy and a thinned dural sheath of the nerve root. A common finding in this pathology is enlargement of the ganglion with splaying of the nerve fibers. Metastasis of pulmonary adenocarcinoma to the DRG has not previously been described but cases of trigeminal ganglion metastasis have . Our casNo metastases to the nerve root itself have been described so far. The possible pathophysiological mechanisms of DRG metastasis were discussed in a description of a breast cancer DRG metastasis. Based on histopathological examinations, it was assumed that the fenestrated endothelium, in contrast to the non-fenestrated endothelium in the nerve root, facilitates DRG but not nerve root metastasis. However, the number of known cases is too small to detect similarities shared by the different entities described. It is noticeable that none of the patients described so far harbored or developed metastases in the central nervous system. DRG metastasis might require distinct genetic changes in the primary tumor to evolve.Metastatic spread of different primary tumors into the DRG, including different species of carcinomas, has been described; however, it is likely that a significant number of cases remain undetected. Besides the low incidence of this entity, this may be explained by the reduction in neurologic deficits and pain due to nerve root decompression. In unclear cases, an exposition of the DRG with inspection and biopsy has to be performed. Although bone metastases of the spine with compression of neuronal structures are more common than DRG metastases, the latter should be considered as a rare differential diagnosis of a lumbar disc herniation or schwannoma for patients with a known malignancy and a radiological mass at the neuroforamen.Surgery for these rare instances is complicated by their usually unanticipated occurrence. Lesions unclear on radiology, especially in patients with known malignancies, should result in an increase in presurgical diagnostic work-up, ideally including positron-emission tomography imaging. Intraoperative neurophysiological monitoring should be used in all unclear lesions. Previous reports indicate that clear delineation of tumor and nerve tissue or tumor capsules is usually not found, and subtotal resections were generally performed . CompletAdvanced treatment options include resection of the process including the DRG with acceptance of neurologic deficts followed by local radiation therapy. We believe these options should be subject to the patients’ discretion. Radical surgery may then be considered following complete tumor staging, including cerebrospinal fluid cytology.DRG metastases are extremely rare. Different tumor entities seem to possess the potential for DRG metastasis. When there are inconclusive imaging and unusual intraoperative findings, rare causes have to be kept in mind, and not only in those patients with pre-existing malignancies. No clear treatment recommendation exists. Based on the few cases reported, nerve root decompression, subtotal resection and adjuvant treatments including local radiation seem to represent the best management option.Written informed consent was obtained from the patient’s relatives for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.DRG: Dorsal root ganglion.The authors declare that they have no competing interests.PJS, JFC and KMA analyzed and interpreted the patient data including follow-up and reviewed the literature. PJS and RB were major contributors in writing the manuscript. TMS performed the histological examinations. All authors read and approved the final manuscript.


Schwannomas are rare mesenchymal tumors of the gastrointestinal tract. Occurrence of these tumors is more common in the stomach than in the large intestine. These tumors usually present as polypoidal intraluminal lesions and based on their location can present with rectal bleeding, colonic obstruction, and abdominal pain or defecation disorders. We present a case of a thirty-five-year-old woman who presented with abdominal pain and melena. Patient was diagnosed with a nonobstructing superficially ulcerated mass in the cecum on colonoscopy and required right hemicolectomy. A very rare pathological diagnosis of cecal schwannoma was made postoperatively. Schwannomas originate from the Schwann cells, which form the neural sheath and encompass the nerves of the myenteric plexus. Schwannomas were first described by Verocay in 1910. The exact incidence of schwannomas is not known. Review of the literature suggests that soft tissue tumors contribute about 1% of all gastrointestinal tumors . This isA 35-year-old woman presented to our emergency department with epigastric pain and melena for two weeks. Abdominal pain was described as sharp in character, severe in intensity, and worse after eating food. Patient also reported nausea, vomiting, and some red blood in the stool for two days. Medical history included gastroesophageal reflux disease, herniated disk, migraine headache and depression. Prior medications included nonsteroidal anti-inflammatory drugs. Physical examination was unremarkable except for epigastric tenderness. Laboratory data showed hemoglobin of 9.2 g/d with iron deficiency. Nasogastric lavage did not reveal any active bleeding. With an initial impression of possible upper gastrointestinal lesion an upper gastrointestinal endoscopy was performed. It did not reveal any pathology to explain etiology of melena. A colonoscopy performed revealed a nonobstructing superficially ulcerated mass in the cecum . BiopsieSchwannomas of the gastrointestinal tract are being increasingly diagnosed with the advent of newer immunohistochemical staining. Schwannomas when present in the gastrointestinal tract are common in the stomach and are rarely found in the large intestine . In a reSchwannomas tend to share the gross morphological features with the other submucosal tumors and may be hard, solid, ulcerated, or calcified in rare cases , 7. It iHistopathological and immunohistochemical study of the tumor cells remains the most important diagnostic tool. In an analysis done by Miettinen et al. , the comSchwannomas of colon are rare tumors of the gastrointestinal tract and classified under mesenchymal tumors. They manifest themselves with varied clinical features depending on their location in the gastrointestinal tract. Endoscopic biopsy at times may fail to achieve an appropriate tissue diagnosis as in our case, though at accessible sites endoscopic ultrasound guided fine needle aspiration may increase the yield. Management differs for various soft tissue tumors and hence it is vital to distinguish them based on their immunehistochemistry. Schwannoma of colon needs excision with tumor-free margins without chemo-or radiation therapy. Our case illustrates an example of a very rare tumor presenting with melena in a young patient requiring surgery.


Intestinal pathologies due to ovarian cyst are observed rarely. Although a limited number of cases in neonatal and adolescent periods have been observed, no adult case has been reported in the literature. Two mechanisms are involved in intestinal complications due to ovarian cysts: torsion due to adhesion or compression of giant ovarian mass with a diameter of 9-10 cm. We report here a terminal ileum necrosis case due to compression by an ovarian cyst with 11 × 10 × 7 cm size in an 81-year-old woman. Although ovarian cysts are commonly observed in women, those with sizes huge enough to fill in the pelvic cavity are rare. These giant cysts may not cause a symptom until reaching a certain size. On the other hand, depending on the localization, size, and presence of compression, they may cause acute abdomen  leading to such symptoms as abdominal pain, nausea, vomiting, and constipation. Gynecologic disorders presenting with acute abdominopelvic pain incidence have been reported to comprise 1.5% of office-based visits and 5% of emergency department admissions . RadioloIntestinal pathologies due to ovarian cyst are observed rarely. Although limited number of cases in neonatal and adolescent periods has been observed, no adult case has been reported in the literature. We report here a case of terminal ileum necrosis due to the compression by a giant ovarian cyst who underwent urgent surgery.3, urea: 123 mg/dL, creatine: 0,97 mg/dL, glucose: 346 mg/dL, and amylase: 256 U/L. Fluid levels were observed in upright abdominal X-ray and dilated intestinal loops, and a cystic mass were detected in abdominal ultrasound. Abdominal computer tomography revealed air-fluid levels in jejunal and ileal segments, minimal fluid between loops and a 10 × 10 cm cystic mass on the right ovary  are observed commonly in emergency surgery practice. Localizations of intestinal obstructions that prevent distal transfer of gastrointestinal content are classified into three as follows: intestinal lumen, intestinal wall, or extraintestinal localization. AMIOs occur generally due to adhesions as a complication of past abdominal operations, as well as invagination, polypoid tumor, gallstone, bezoar formation, foreign body, intestinal parasites, trauma, tumor, abdominal inflammation, external-internal herniation, volvulus, stricture due to inflammatory bowel disease, submucosal hematoma due to long-term anticoagulant use. None of these etiologies was present in our case. We detected oliguria and azotemia due to hypovolemia caused by prolonged AMIO and vomiting, as well as reduced central venous pressure and cardiac output, hypotension, and hypovolemic shock which lead to irreversible dehydration and hemoconcentration and deteriorated the patient's general condition . TherefoIn conclusion, intestinal pathologies due to giant ovarian cysts should be considered in women admitted to emergency service with symptoms of acute abdomen.


Hidradenoma papilliferum is a rare tumor that occurs almost exclusively in females on the anogenital area. Rare cases of ectopic  hidradenoma papilliferum have been described. The lesions usually present as an asymptomatic slow-growing, red, firm, mobile, well-delimitated nodule that grows for a long time before resection. We describe a case of an 26-year-old man that presented with an enlarging nodule on his right eyelid. The histological findings revealed a hidradenoma papilliferum. So far, among the very few reports of ectopic hidradenoma papilliferum, only a very small number were localized to the eyelid. Hidradenoma papilliferum is a slow-growing benign adnexal tumor with apocrine differentiation, and for some authors it can be considered an analog of intraductal papilloma of the breast . The tumA 26-year-old white man presented in our department with a 2-year history of an asymptomatic enlarging lesion of the upper eyelid. No previous lesion, namely nevus sebaceous was documented. His personal and familiar medical history was unremarkable. On physical examination a well-circumscribe, firm, umbilicated, reddish, molluscum contagiosum-like nodule approximately 1 cm in diameter localized to the upper right eyelid was observed . DermoscMicroscopic examination showed a well-circumscribed dermal nodule characterized by a large cystic space containing eosinophilic debris and papillary folds. The lesion seemed to be connected with the epidermis and a central ulceration was observed. Papillae and micropapillae projected from the cyst wall into its cavity . The papHidradenoma papilliferum is originated in the apocrine glands, which are mainly concentrated in the anogenital region, axillae, and periumbilical areas. The tumor occurs mainly in those areas with ectopic localization being rarely reported . The dis


Incisional endometriosis (IE) is a rare entity reported in 0.03–1.08% of women following obstetric or gynecologic surgeries. Most cases reported in literature have appeared after cesarean sections and were often clinically mistaken for hernia, abscess, suture granuloma or lipoma. We hereby report a case of IE following a second trimester hysterotomy, which was diagnosed by fine needle aspiration cytology (FNAC). Our patient was 26 years old, presenting with a mass over anterior abdominal wall, associated with incapacitating pain during each menstrual cycle. FNAC showed epithelial cells, stromal cells and hemosiderin laden macrophages. Based on the typical history, clinical and cytological features, the diagnosis of IE was established. Wide surgical excision was done and the resulting rectus sheath defect was repaired. Patient was followed for 6 months during which time she was symptom free. This article also reviews the spectrum of cytological features and the rare possibility of malignant transformation that can occur in IE. Incisional endometriosis (IE) is a rare entity reported in 0.03–1.08% of women who have undergone obstetric or gynecologic surgeries. Most cases reported in literature have appeared after cesarean sections. Clinically, IE is often mistaken for abscess, hernia, suture granuloma or lipoma. We hereby report a case of IE following a second trimester hysterotomy, which was diagnosed on fine needle aspiration cytology (FNAC). Review of literature shows that FNAC can be useful in the diagnosis of IE. However, the cytopathologist should be aware of the spectrum of changes that can occur in both endometrial glandular cells and stromal cells, while evaluating these cases.A 26-year-old lady presented with a mass over anterior abdominal wall, of 2 years duration. She complained of incapacitating pain in the swelling, which started a week before menstruation and subsided a week after her periods. She also reported a corresponding fluctuation in the size of the swelling during each cycle. The patient had two normal deliveries in the past. Her third pregnancy was terminated in the second trimester by a hysterotomy, 4 years earlier.On examination, the swelling was located on the left side of the anterior abdominal wall, close to the previous pfannenstiel incisional scar. It measured 6×4 cm, was firm, had well-defined borders and was situated in subcutaneous plane. There was no clinical or sonological evidence of pelvic endometriosis.FNAC showed sheets of epithelial cells and fibromyxoid stroma Figure  and b. TSurgical excision was done, leaving a wide margin on all sides. Peroperatively, swelling was found attached to the anterior rectus sheath, part of which was also excised. The rectus sheath defect measuring 2×1 cm was repaired with number one proline. Recovery was uneventful.Grossly, the excised specimen was an irregular fatty mass, which on cut section showed a central fibrous area surrounded by multiple, tiny bluish spots . HistopaEndometriosis refers to functional endometrial glands and stroma lying outside the uterine cavity. Endometriosis occurring in a surgical scar is called IE or scar endometriosis. An endometrioma refers to a circumscribed mass of ectopic endometrial tissue. The pres2IE is reported to occur in 0.03–1.08% of women after obstetric or gynecologic surgeries, particularly after hysterotomy. The inci2IE has also been reported in scars resulting from hysterectomy, tubal ligation, ectopic pregnancy, salpingectomy, uterine suspension, inguinal herniorrhaphy, bartholin cyst excision, episiotomy, laprotomy, abdominoplasty, laproscopic trocar tract and needle tract following diagnostic amniocentesis.–8 Of theet al,[The most common site of IE is near a pfannenstiel incision. This is possibly related to the wider dissection of the tissue planes when compared to vertical midline incision. Teng et al, have repDiagnosis of scar endometriosis is usually made on clinical grounds. In clinically doubtful cases, FNAC has been a valuable diagnostic tool. Cytology smears show sheets of epithelial cells, spindled stromal cells and a variable number of hemosiderin laden macrophages. The stromal cells are plump, spindled and arranged around a vascular meshwork. The presence of any two of the three components is required for the diagnosis of endometriosis. The impoet al,[The epithelial and stromal cells in IE can undergo a wide spectrum of changes which may pose diagnostic challenges. Epithelial cells may also undergo squamous, mucinous or tubal metaplasia. Nuclear atypia and cytoplasmic vacuolation has been reported in the glandular cells during secretory phase. Stromal 11et al, have repet al, If nucleMedical management with oral contraceptive pill, progestogens and gonadotropin releasing hormone analogues provide alleviation of symptoms, but recurrence is common after cessation of therapy. Wide surgical excision with at least 1 cm margin on all sides and patch grafting of the fascial defect, if necessary, is the treatment of choice.8 It is b


Adrenocortical carcinomas are rare aggressive tumors. Their annual incidence is approximately one to two per million among the population of the United States of America. Patients with active endocrine tumors often present with Cushing's syndrome accompanied by virilizing features. Conversely, patients with non-functioning tumors may present with symptoms related to a mass-occupying lesion, such as abdominal pain and flank pain. Although varicoceles and acute kidney injuries are common problems in medicine, they are uncommon presentations of these rare tumors and easy to miss. We report a case of a large adrenocortical carcinoma that presented as testicular pain, varicocele, and acute kidney injury secondary to renal vein thrombosis.A 54-year-old Caucasian man with a left-sided varicocele presented to our emergency department with lower abdominal pain and a decrease in urination. Four months previously, he had noticed pain and swelling in his left groin and had been diagnosed with left-sided varicocele. For one week, he began developing left-sided abdominal pain and decreased urination frequency, so he came to our emergency department for evaluation. His physical examination revealed a hard mass occupying the entire left side of his abdomen, crossing the midline, and extending to the pelvic brim. His blood tests showed acute kidney injury and mild anemia. Computed tomography of his abdomen showed a large retroperitoneal mass on the left side, displacing the left kidney inferiorly and the spleen superiorly with thoracic epidural compression. Thrombus was also identified in his left renal vein and inferior vena cava. Computed tomography of his chest showed bilateral pulmonary nodules. A computed tomography-guided abdominal mass biopsy was performed, and the diagnosis of adrenocortical carcinoma was made on the basis of pathology and immunohistochemistry. His hormonal evaluations were normal. His kidney function improved with intravenous hydration and anti-coagulation treatment. Unfortunately, the adrenal mass was unresectable because of the extent of the tumor. Treatment with mitotane, an adrenocorticolytic drug, was started with concomitant with irradiation of a lesion at T5, followed by combination chemotherapy thereafter.Unilateral right-sided varicoceles are rare and should alert the clinician to possible underlying pathology causing inferior vena caval obstruction. Left-sided varicoceles, in contrast, are common secondary to the venous anatomy of the left testis; however, the enlargement of the left testicle can be associated with blockage of the left testicular vein by tumor invasion of the left renal vein. Varicoceles could be an early presentation of a non-functioning adrenocortical carcinoma. Acute kidney injury can occur as a result of mass effect or thrombosis of renal vessels. Large tumors can cause abdominal pain as a late manifestation. Physicians should perform a complete abdominal examination in every patient with varicocele or testicular pain. Adrenocortical carcinomas (ACCs) are rare aggressive tumors; their annual incidence is approximately 1 to 2 per million among the population of the United States of America . ConversA 54-year-old Caucasian man with a history of ischemic stroke and ischemic cardiomyopathy presented to our emergency department with constant left-sided lower abdominal pain and decrease in urination of one week's duration. Four months prior to presentation, he had noticed pain and swelling in his left groin. Because of his concerns of a hernia, he sought clinical evaluation. His family physician sent him for an ultrasound of the scrotum, which revealed a left-sided varicocele. He was then referred to a urologist. For one week, he developed continuous, unrelenting left-sided abdominal pain localized primarily in the left lower quadrant. He had diminished appetite and noted a 12-pound weight loss during the prior one month. Because of decreased urinary frequency, he came to our emergency department for evaluation. His family history was negative for malignancy. His physical examination revealed a hard mass occupying the entire left abdomen, crossing the midline, and extending to the pelvic brim, as well as the presence of a left-sided varicocele. He had no lymphadenopathy or hepatomegaly and no clinical signs of hormone access of 1.7 from a baseline of 1.0, hyponatremia (serum sodium 130 mmol/L), and normocytic anemia  His hormonal evaluations, including fasting blood glucose, serum potassium, adrenocorticotropic hormone (ACTH), morning serum cortisol, and androgen levels, were normal. Renal ultrasound showed that the left kidney was inferiorly displaced by what was thought to be an enlarged spleen. His home medication of lisinopril was discontinued. Intravenous fluid was started. Contrast-enhanced computed tomography (CT) of the abdomen and pelvis performed after the patient's renal function improved showed a large retroperitoneal mass on the left side displacing the left kidney inferiorly and the spleen superiorly with T5 epidural compression , and serum estradiol in men and post-menopausal women ,10 is thA variety of staging systems have been used for ACC. The Union for International Cancer Control (UICC) proposed the first TNM classification of Malignant Tumors for ACC in 2003. However, an analysis based on data from the German ACC Registry revealed several shortcomings of this classification system. Therefore, ENSAT developed a revised staging system. The superiority of the ENSAT staging system over the 2004 UICC/American Joint Committee on Cancer classification system for prognostication was confirmed in a recent North American study. Estimated five-year disease-specific survival rates of patients with stage I and stage IV cancer in the studies were 82% and 13%, respectively ,13.Nowadays, adrenocortical cancer is often diagnosed after a great delay, when the cancer is very advanced, as shown in the present case report. The only potentially curative treatment for ACC is surgical resection , which iTesticular pain and varicocele could be an early presentation of non-functioning ACCs. Acute kidney injury can occur as a result of a mass effect or as a result of thrombosis of renal vessels. Large tumors can cause abdominal pain in the late manifestation and unresectable stage. The diagnosis of varicoceles necessitates evaluation of the abdomen and retroperitoneum for underlying malignancy. To our knowledge, herein we report the first case of a large, left-sided, non-functioning ACC presenting as a left-sided varicocele and acute kidney injury secondary to left renal vein thrombosis.ACC: adrenocortical carcinoma; ACTH: adrenocorticotropic hormone; CT: computed tomography; ENSAT: European Network for the Study of Adrenal Tumours; UICC: Union for International Cancer Control.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.WC, JMH, and CBH were involved in the diagnosis and treatment of the patient. WC drafted the manuscript. JMH and CBH revised the manuscript. All authors read and approved the final manuscript.


Myxomas are locally invasive, benign mesenchymal neoplasms with odontogenic, osteogenic, or soft tissue origin. Facial myxomas probably account for less than 0.5% of all paranasal sinus and nasal tumors. We report a case of a left painless periocular mass in a 11-month-old girl. The lesion was resected with a clinical diagnosis of lacrimal sac tumor. Histopathology and immunohistochemistry proved the tumor to be a myxoma. There has been no recurrence after 4 years of followup. Midfacial myxomas should be differentiated from other benign and malignant tumors such as dermoid, hamartoma, neurofibroma, nasolacrimal duct cyst, and sarcomas in particular embryonal rhabdomyosarcoma. Because of the infiltrative nature of these tumors, a wide surgery is required to achieve clear resection margins and avoid recurrence. Childhood myxomas of the head and neck are rare, but some well-documented cases have been published in the literature.They may be related to dental malformations or missing teeth, but many occur without any such abnormalities.Their local aggressiveness and ability to erode bone should not be underestimated, thus surgical resection with free margins of these tumors is mandatory . A case An otherwise healthy 11-month-old baby girl presented with an 8-day complaint of painless swelling in the left medial canthal region. Her family reported a mild upper respiratory infection treated with antibiotics 5 days earlier. No accompanying history of preceding trauma, nasal, ocular, or dental disease was mentioned. Physical examination of the child revealed a nontender, firm, and fixed infraorbital mass on the left side that appeared to be arising from the maxillary sinus. The margins of the swelling were ill-defined and measured 2 × 2 cm. The skin overlying the lesion was intact. Ophthalmological examination confirmed no direct involvement of her left eye and lacrimal pathway .Echographic examination of the left orbit disclosed an ill-demarcated mass with irregular internal structure and medium internal reflectivity (44%). Peripheral bony destruction was noted . CT scanExcisional biopsy of the lesion was performed and the specimen submitted to pathology. Gross examination revealed a well-circumscribed nonencapsulated solid tumor measuring 26 mm on its largest diameter. The tumor was soft in consistency. The cut surface had a glistening gray-yellowish appearance . HistopaAfter the surgery, the patient returned for follow-up examination 7 days, 6 months, one year, and 4 years later. The wound healed without complication, and there has been no evidence of recurrence .Stout, in 1948, provided the first extensive review and clear diagnostic criteria for myxoma. He defined myxomas as true neoplasms of primitive mesenchyma that do not metastasize .Facial myxomas are benign slow-growing expansible tumors with odontogenic, osteogenic, or soft tissue origin . They arIn conclusion myxoma should be considered as a differential diagnosis in children with a painless periocular mass and differentiated from more malignant tumors, such as embryonal rhabdomyosarcoma. The immunohistochemistry is a useful complementary tool to confirm the diagnosis of myxoma and to rule out other benign and malignant myxoid tumors. Complete resection of the tumor with free margins is the treatment of choice.


Sex cord-stromal tumors of the ovary are uncommon. They behave unpredictably and often have a late recurrence, making counseling, management, and prediction of prognosis challenging.A 52-year-old Moroccan woman with an sex cord-stromal tumors underwent a bilateral oophorectomy. The histology was unusual but was likely to be a luteinized thecoma with suspicious features for invasion. Seven years later, after a gastrointestinal bleed, a metastasis within the small bowel mucosa was detected. This represents probable isolated hematogenous or lymphatic spread, which is highly unusual, especially in the absence of concurrent peritoneal disease.To the best of our knowledge, this is the second reported case of an sex cord-stromal tumors recurring in small bowel mucosa and mimicking a primary colorectal tumor. This highlights the diverse nature and behavior of these tumors. Sex cord-stromal tumors (SCSTs) of the ovary are uncommon neoplasms that account for 5% to 8% of all ovarian malignancies . Their hWe report the case of a 52-year-old Moroccan woman who had been menopausal for 14 years. She presented with a lower abdominal pain. She had not experienced any postmenopausal bleeding and had no bowel or urinary symptoms. She was a nonsmoker. She had no other significant medical history and no sign of virilization. An examination revealed a smooth cystic mass posterior to the uterus. A transvaginal and transabdominal pelvic ultrasound demonstrated a 9 × 3.7 cm heterogeneous mass in her left adnexa. No vascular flow or ascites was seen, the ovaries did not appear to be separate from the mass. A chest X-ray was normal. Serum CA-125 level was 6 IU/mL. Renal, liver, and hematologic parameters were all in normal range. At laparotomy, a large torted left ovarian cyst with small bowel adhesions to its surface was discovered. Her right ovary appeared normal but was adherent to her small bowel; her uterus was normal. Her liver and omentum appeared normal, and there was no pelvic or abdominal lymphadenopathy. There was a small amount of ascites that was sent for cytology. An oophorectomy was performed with no macroscopic residual disease. An omental biopsy was taken. The histology showed a tumor of sex cord-stromal type in the ovary but the tumor was difficult to classify further (Figure SCSTs vary greatly in prognosis and behavior, depending on the subtype. Therefore, deciding on methods of management for recurrence can be difficult, especially when the tumor is at an unusual site, as in this case. Since disseminated spread is the most common mode of metastasis in ovarian cancer, the gastrointestinal tract is frequently involved. In some advanced cases, determining whether the origin of the tumor is bowel or ovarian can be difficult. The use of immunohistochemical markers such as CK7 and CK20 can assist in the differentiation. The question of tumor origin did not present a problem with this case as the histologic morphology clearly demonstrated a recurrent SCST as opposed to an adenocarcinoma, which could be of ovarian or colonic origin. The majority of gastrointestinal recurrences from ovarian tumors occur via direct transcoelomic spread and invasion of the serosal surface in a centripetal direction. Rose and colleagues , in a reThis report further highlights the diverse nature and behavior of this group of tumors. This case demonstrates the difficulty in adopting an evidence-based approach to the treatment and follow-up in these rare cases. The need for careful discussion with the patient about additional treatment, if any, is of paramount importance.BEP: bleomycin, etoposide, and cisplatin; SCST: sex cord-stromal tumor.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.TEM analyzed and interpreted the patient data in regard to its oncological and imaging features and was involved in drafting the manuscript. IE analyzed and interpreted the patient data in regard to its oncological and imaging features. HM was involved in drafting the manuscript. HE gave final approval of the version to be published. All authors read and approved the final manuscript.