Laparoscopic resection of a large mixed epithelial-stromal tumour of the seminal vesicle: a rare entity and review of the current literature.

Mixed epithelial-stromal tumours (MESTs) of the seminal vesicle (SV) are a rare neoplasm, with biological behaviour ranging from benign to malignant. Due to their rarity, there are no established guidelines for their treatment. We report a 37-year-old man with a large MEST of the SV which was successfully resected by laparoscopic transperitoneal approach. Amidst the controversy regarding the nomenclature and grading of MESTs in literature, we reclassified the previous reports of MESTs incorporating both the WHO and Reikie et al grading.

Calcifying nested stromal epithelial tumor of the liver in a patient with Klinefelter syndrome: a case report and review of the literature.

BACKGROUND: Calcifying nested stromal epithelial tumor (CNSET) is a primary neoplasm of the liver, characterized by well-demarcated nests consisting of spindle and epithelioid cells with calcification and bone formation. An association of Cushing syndrome with CNSET has drawn attention, but the origin of CNSET has not been clarified. CASE PRESENTATION: We report here the case of a 20-year-old male with Klinefelter syndrome who underwent liver resection for an increasing liver tumor that was pathologically diagnosed with CNSET. He was postoperatively followed up and received several examinations, and recurrences and extrahepatic lymph node metastases were detected on the 64th day after surgery. Chemoembolization and chemotherapy were not effective, leading to tumor progression with development of progressive liver failure, and the patient finally died 164 days after hepatectomy. CONCLUSIONS: This case suggests that an imbalance of hormones affects the genesis and progression of CNSET, and indicates the importance of closely following patients with CNSET by imaging with attention to hepatic recurrence and extrahepatic metastases.

Recurrent/Residual Intracranial Phosphaturic Mesenchymal Tumor Revealed on 68Ga-DOTATATE PET/CT.

Phosphaturic mesenchymal tumors are benign tumors causing majority of tumor-induced osteomalacia. These tumors are most commonly located in the lower extremities followed by craniofacial regions. However, intracranial lesion is very rare. Here we describe Ga-DOTATATE PET/CT findings of a recurrent intracranial phosphaturic mesenchymal tumor in a 68 year old woman.

Ganglioglioma Progression to Combined Anaplastic Ganglioglioma and Anaplastic Pleomorphic Xanthoastrocytoma. Case Report and Literature Review.

BACKGROUND: Composite ganglioma and pleomorphic xanthoastrocytoma with anaplastic features in both components is an extremely rare glioneuronal tumor. Five cases of anaplastic progression in the glioma component have been reported. These tumors generally affect young patients who have brain tumor-related epilepsy, which are usually located in the temporal lobe or in the cerebellum and may have associated leptomeningeal spreading. Its current optimal treatment consists of maximal safe surgical resection and adjuvant chemoradiotherapy. Overall survival at 5 years is 33% in anaplastic pleomorphic xanthoastrocytoma and 53% in anaplastic ganglioglioma. CASE DESCRIPTION: We describe a progression from ganglioglioma to this composite anaplastic entity after 32 months of follow-up, with apparently nontumoral parenchyma separating the 2 components. Polymerase chain reaction showed a wild-type BRAF gene. Seven months after concomitant chemoradiotherapy, radiologic progression led to a second line of chemotherapy, and a third line of chemotherapy was initiated after a subsequent progression at 11 months. CONCLUSIONS: This case may add some evidence in favor of the glioneuronal maldevelopment hypothesis to explain the oncogenesis of these neuroepithelial tumors.

Mixed Pituitary Gangliocytoma and Prolactinoma Resistant to the Cabergoline Treatment.

BACKGROUND: A gangliocytoma rarely coexists with a pituitary adenoma in a sellar lesion. Herein, we describe our experience in treating a mixed gangliocytoma and prolactinoma of the pituitary gland. CASE DESCRIPTION: A 16-year-old male presented with severe headache and vomiting. Magnetic resonance imaging showed a large pituitary tumor with hydrocephalus. Because of the increased levels of serum prolactin (PRL), we treated the patient with cabergoline, which decreased the tumor size and improved the hydrocephalus. Six months after the treatment, the tumor began to increase in size, despite the normalization of the PRL level with cabergoline treatment. An endoscopic transsphenoidal resection was performed and the tumor was mostly removed. Microscopic examination of the resected tumor showed a mixture of prototypical pituitary adenoma cells and the proliferation of mature ganglion cells. Immunohistochemistry showed that the ganglion cells were positively stained for synaptophysin, NeuN, and PRL as shown in the adenomatous component. A few cells were immunostained with both PRL and NeuN, and a few cells were immunopositive for nestin, but not PRL or synaptophysin. CONCLUSIONS: Our findings showed the existence of cells that are phenotypically intermediate between ganglion cells and adenoma cells, and the existence of stem cell-like cells, which support the hypothesis that adenoma cells can transform into ganglion cells or that both ganglion and adenoma cells derive from common stem cells. Furthermore, the ganglion cells seemed to grow rapidly and independently of dopamine, which is in contrast to prototypical prolactinoma cells.

Endoscopic Histologic Mapping of a Mixed Germ Pineal Tumor.

BACKGROUND: The accurate histologic diagnosis of germ cell tumors in the pineal region is a keystone for determining the best treatment strategy and prognosis. This situation poses a challenge for the neuropathologist, considering the lack of a standarized procedure to obtain biopsy samples, which results in few and small specimens, which are not suitable for diagnosis. CASE DESCRIPTION: We report a case in which a pineal region mixed germ cell tumor was accurately diagnosed by performing histologic mapping through a dual burr-hole endoscopic approach. The technical pitfalls and other considerations necessary for obtaining an accurate diagnosis in this tumor subgroup are specified. In addition, the histologic analysis regarding the sampling technique used is described. CONCLUSIONS: The supraorbital frontal endoscopic approach enables the surgeon to perform histologic mapping of pineal region tumors, allowing standarization of the procedure used to obtain the specimens. This approach could result in a more accurate diagnosis, especially in mixed germ cell neoplasms.

Calcifying nested stromal-epithelial tumor (CNSET) of the liver: a newly recognized entity to be considered in the radiologist's differential diagnosis.

Calcifying nested stromal-epithelial tumor (CNSET), an extremely rare tumor found in the liver, was first described in 2001 by Ishak et al. The characteristic imaging features include large size, well-circumscribed, enhancing mass with calcification. To our knowledge, since 2001, there have been 29 reported. Typically arising from the right hepatic lobe, it is primarily found in children and shows clear predilection for females. Emphasizing imaging, we report a 14-year-old female with Beckwith-Wiedemann syndrome who presented with CNSET.

A rare case of primary choriocarcinoma in the sigmoid colon.

Primary colorectal choriocarcinoma is an extremely rare neoplasm and is usually associated with a poor prognosis. Only 13 cases of colorectal choriocarcinoma have previously been reported. There is no standard chemotherapeutic regimen for this tumor type. A 68-year-old man presented with melena and was diagnosed with sigmoid colonic adenocarcinoma with multiple liver metastases. He underwent a laparoscopic sigmoidectomy. Pathology revealed choriocarcinoma with a focal component of moderately differentiated adenocarcinoma of colon origin. Based on the collagen gel droplet-embedded culture drug sensitivity test (CD-DST) results, mFOLFOX6 and bevacizumab were administered, which suppressed aggressive tumor growth for 4 mo. The patient died 9 mo after the initial diagnosis. Our study results suggest that the standard chemotherapy regimen for colorectal cancer might have suppressive effects against primary colorectal choriocarcinoma. Moreover, CD-DST may provide, at least in part, therapeutic insight for the selection of appropriate antitumor agents for such patients.

Mixed epithelial and stromal tumor of the kidney: report of eight cases and literature review.

Mixed epithelial and stromal tumor of the kidney (MESTK) is the term given to a class of uncommon biphasic tumors of the kidney, with few reported cases. We describe eight cases of MESTK with detailed clinicopathological data and follow-up information. With this report, we hope to increase clinical awareness that MESTK should be considered as one of the possible diagnoses for cystic renal mass, especially in peri-menopausal women or those who receive hormone therapy. In addition, regular follow-up is necessary for the any cases with malignant potential.

Pilomyxoid astrocytoma of the cerebellum with Williams syndrome: a case report.

CASE REPORT: A 3-year-old boy with Williams syndrome associated with supravalvular aortic stenosis was admitted to our hospital with disturbance of consciousness and a 2-month history of truncal ataxia. T1-weighted magnetic resonance imaging with contrast medium showed a heterogeneously enhanced tumor in the right cerebellum with severe hydrocephalus. The patient underwent tumor resection via suboccipital craniotomy. At the end of resection of the tumor, sudden cardiac arrest occurred after ST segment elevation. Despite immediate cardiopulmonary resuscitation, the patient died. Histological examination of the cerebellar tumor revealed that the tumor consisted of monomorphous bipolar spindle cells on a background of myxoid matrix, and angiocentric arrangement without Rosenthal fibers or eosinophilic granular body. The final diagnosis was pilomyxoid astrocytoma. CONCLUSION: This case of Williams syndrome with cerebellar pilomyxoid astrocytoma suggests the importance of investigation of the development of brain tumors and occurrence of intraoperative cardiac arrest associated with Williams syndrome.

Third ventricle, cerebral aqueduct, and fourth ventricle mixed ependymal neurocytoma: a case report and review of the literature.

PURPOSE: A case report of an 8-year-old female who underwent interhemispheric transcallosal approach to the third ventricle, cerebral aqueduct, and fourth ventricle for resection of a mixed ependymal neurocytoma and review of the literature was evaluated. METHODS: An Ovid MEDLINE review of the literature was conducted starting in 1946 to current using search terms as described in our keywords. RESULTS: A total of 16 neurocytoma have been described in the literature as either posterior third ventricle or posterior fossa in origin. Of these lesions, five have been described as mixed glial-neuronal and all of these were located in the fourth ventricle. To our knowledge, this is the first described mixed glial-neuronal tumor located in the posterior third ventricle and aqueduct.

Primary mixed germ cell tumor arising in the pancreatic head.

Germ cell tumors, comprised of gonadal and extra-gonadal types, are relatively rare tumors arising from primordial germ cells. Extra-gonadal germ cell tumors have been reported to occur at many non-gonadal locations, from the brain to the sacrococcygeal region. However, primary germ cell tumors in the pancreas are extremely rare. Herein, we present the first case of a 12-month-old girl with a primary mixed germ cell tumor, consisting of both endodermal sinus tumor and mature teratoma, in the pancreatic head.

Pulmonary pleomorphic carcinoma with rapid progression.

We describe a case of pleomorphic carcinoma showing a recurrent tumor with massive hemorrhage and myxoid change in the chest wall 2 months after complete resection. Whereas specimens from the initial surgery revealed both adenosquamous carcinoma and sarcomatous elements, the recurrent tumor predominantly consisted of a sarcomatous element. The recurrent tumor had stronger immunoreactivity for mesenchymal markers than the primary tumor, indicating that the sarcomatous element had more malignant potential than the epithelial element.

Dyspnea in a patient with Mounier-Kuhn syndrome.

Poorly differentiated non-small cell lung carcinoma with a component of sarcoma-like (spindle and/or giant cells) or sarcoma (malignant bone, cartilage, or skeletal muscle) cells are called pleomorphic carcinoma. These carcinoma represent one of the 5 subtypes of rare pulmonary malignancies collectively classified as sarcomatoid carcinoma by the World Health Organization histologic classification of lung tumors. The pathogenesis of sarcomatoid carcinoma remains unclear, and treatment of this malignant tumor is less defined. Very few cases of sarcomatoid carcinoma involving the upper respiratory tract have been reported in the literature. We report here an atypical presentation and location of this tumor (in the trachea), causing obstruction with a positional ball-valve effect, in a patient with tracheobronchomegaly (Mounier-Kuhn syndrome). In addition, we discuss the recurrent nature of the disease and the potential therapeutic difficulties.

[Management of a case of unilateral exophthalmos due to an intraorbital lipofibroma at Kara teaching hospital in Togo]. / Prise en charge d'un cas d'exophtalmie unilatérale due à un lipofibrome intra-orbitaire au CHU de Kara au Togo.

In childhood, benign tumors are uncommon causes of exophtalmos. We report a case of intraorbital lipofibroma in an 11-year-old girl who presented with painless, progressive proptosis of the right eye over the preceding 6 months. Ultrasound had shown an extraconal intraorbital mass. Complete excision and pathologic examination were performed, revealing a lipofibroma with no signs of malignancy This case report is notable due to the rarity of the condition.

Verrucous carcinoma of the renal pelvis with a focus of conventional squamous cell carcinoma.

Verrucous carcinoma (VC) is a rare variant of squamous cell carcinoma (SCC) with an extremely well-differentiated microscopic appearance. It is able to show extensive local invasion, but practically never metastasizes. VCs mostly occur in the oral cavity, larynx, nasal cavity, esophagus, vulva, vagina, anorectal region, penis and skin. VCs sometimes coexist with conventional SCCs, and in these instances they are associated with a higher recurrence rate than pure VCs. The occurrence of VC in the renal pelvis is very rare and to date only 4 cases have been reported. We report here a case of VC with a focus of conventional SCC in the renal pelvis. The patient showed fistula formation by residual tumor in the follow-up period.