Carcinosarcoma (adenocarcinoma, neuroendocrine carcinoma, undifferentiated carcinoma and chondrosarcoma) of the gallbladder.

We present an extremely rare case of carcinosarcoma with 4 different tumor components in an 88-year-old female. After a diagnosis of acute cholecystitis, we performed percutaneous transhepatic gallbladder drainage in the patient without success, followed by a cholecystectomy and choledocholithotomy. The mass was a 60 × 25 mm polypoid lesion of the gallbladder identified histologically as a carcinosarcoma with adenocarcinoma, neuroendocrine carcinoma, undifferentiated carcinoma and chondrosarcoma components. The biliary-type adenocarcinoma portion exhibited acinar growth patterns with columnar cells having large and markedly hyperchromatic nuclei. These tumor cells were immunohistochemically positive for MUC1 and CDX2. The neuroendocrine carcinoma, small cell type, cells were densely packed and small, with scant cytoplasm, finely granular nuclear chromatin and absence of nucleoli. The mitotic index was high. These tumor cells were immunohistochemically positive for synaptophysin, Ki-67 (index 40%), MUC1, CDX2 and c-Kit. The undifferentiated carcinoma consisted exclusively of spindle cells containing large, markedly hyperchromatic nuclei with a high mitotic index. These tumor cells were immunohistochemically positive for AE1/AE3. The chondrosarcoma was composed of blue-gray chondroid matrix and atypical chondrocytes containing large, hyperchromatic nuclei. These tumor cells were immunohistochemically positive for S100. Its attributes might be suggestive of a greater malignant potential and pathogenesis of carcinosarcoma.

Adult-Onset Mixed Germ Cell Tumor Composed Mainly of Yolk Sac Tumor Around the Pineal Gland: A Case Report and Review of the Literature.

BACKGROUND: Mixed germ cell tumors (MGCTs) usually occur in children. In the present report, we discuss an extremely rare case of adult-onset MGCT composed mainly of yolk sac tumor (YST) around the pineal gland. CASE DESCRIPTION: A 54-year-old Japanese man presented with disturbance of consciousness, Parinaud's syndrome, and gait disturbance. Magnetic resonance imaging revealed a pineal mass lesion, and subtotal resection of the tumor was achieved. The histologic diagnosis was MGCT, consisting mainly of YST. Although he underwent 5 courses of chemotherapy and craniospinal irradiation after surgery, tumor dissemination could not be controlled, and he died 10 months postoperatively. CONCLUSION: The present case highlights the need for clinicians to include YST in the differential diagnosis of acute progressive lesions around the pineal region, even in adult patients.

Subependymomas - Characteristics of a "Leave me Alone" Lesion. / Subependymome ­ Charakteristika einer "leave me alone"-Läsion.

PURPOSE: Intracranial subependymomas are rare, mostly asymptomatic tumours, which are often found incidentally and therefore did not receive much attention in previous literature. By being classified as benign grade I in the WHO classification of tumours of the central nervous system, they are given a special status compared to the other ependymal tumours. Tumor recurrences are a rarity, spinal "drop metastases" do not occur. While etiological, pathological and therapeutic characteristics have been subject of several publications over the last few decades and have meanwhile been well studied, the imaging characteristics are much less well received. MATERIAL AND METHOD: Retrospective analysis of our relatively large group of 33 patients with subependymoma, including 4 patients with a mixture of subependymomas with ependymal cell fractions in terms of imaging and clinical aspects and with reference to a current literature review. RESULTS: Subependymomas have typical image morphologic characteristics that differentiate them from tumors of other entities, however, the rare subgroup of histopathological mixtures of subependymomas with ependymal cell fractions has no distinctly different imaging properties. CONCLUSIONS: Knowing the imaging characteristics of subpendymoma and their differential diagnoses is of particular importance in order to be able to decide between the necessity of follow-up controls, an early invasive diagnosis or, depending on the entity, tumor resection. KEY POINTS: · Subependymomas have typical imaging characteristics that are clearly distinguishable from other entities.. · Increased incidence in middle/ older aged men, most frequent localization: 4th ventricle.. · Symptomatic subependymomas, often located in lateral ventricles, are usually characterized by hydrocephalus.. · Radiological identification of mixed subependymoma with ependymal cell fractions is not possible.. · Image based differentiation from other entities is important for the procedure.. CITATION FORMAT: · Kammerer S, Mueller-Eschner M, Lauer A et al. Subependymomas - Characteristics of a "Leave me Alone" Lesion. Fortschr Röntgenstr 2018; 190: 955 - 966.

Ampullary Mixed Adenoneuroendocrine Carcinoma: Surprise Histology, Familiar Management.

INTRODUCTION: Mixed adenoneuroendocrine carcinoma (MANEC) has recently been defined by the World Health Organization in 2010. These are rare tumors and MANECs of ampullary region are even rarer. Only 19 cases have been reported in literature. We present 3 cases; the largest series, second case of amphicrine tumor and first case associated with chronic pancreatitis. METHODS: Retrospective review of 3 patients who were diagnosed to have ampullary MANEC. RESULTS: All 3 patients were diagnosed preoperatively as neuroendocrine carcinoma and underwent margin negative pancreaticoduodenectomy. The histopathology revealed MANECs of small cell, mixed type in 2 patients and large cell, amphicrine type in 1 patient. The neuroendocrine component was grade 3 in all, the tumor was T3 in 2 and T2 in 1 and all had nodal metastases. Two patients received adjuvant chemotherapy and 2 of them had recurrence at 13 and 16 months. The median survival was 15 months. CONCLUSION: Ampullary MANECs are rare tumors. They are diagnosed on histopathologic examination of the resected specimen. Clinical presentation, management, and prognosis is similar to ampullary adenocarcinoma in literature.

Malignant mixed germ cell tumour of ovary--an unusual combination and review of literature.

Mixed germ cell tumours of the ovary are malignant neoplasms of the ovary comprising of two or more types of germ cell components. Most of the malignant mixed germ cell tumours consists of dysgerminoma accompanied by endodermal sinus tumours, immature teratoma or choriocarcinoma. There are only few case reports of mixed germ cell tumours with different combinations of malignant components. We report a very rare case of mixed germ cell tumours consisted of malignant components of endodermal sinus tumour, emryonal carcinoma, and benign component of teratomatuos and trophoblastic differentiation. This is the first case report in the literature with both benign and malignant component of type described to best of our knowledge. Patient was an 18 year old girl, who presented with pain abdomen, abdominal mass and irregular bleeding. Ultrasound and CT scan showed a huge mass with solid and cystic component. Tumour markers i.e alpha feto- protein (AFP), human chorionic gonadotropin (hCG), lactate dehydrogenate (LDH) and Ca-125 were raised. We performed fertility sparing surgery by preserving one ovary, tube and uterus. Conclusion: Malingnant mixed germ cell tumours of ovary are highly aggressive neoplasm and early intervention and fertility sparing surgery is required for any adolescent girl presenting with rapidly enlarging pelvic mass.

Uterine endometrial carcinoma with trophoblastic differentiation: a case report with literature review.

Choriocarcinoma is categorized as either gestational or nongestational depending on its origin. Nongestational choriocarcinoma originated in the trophoblastic differentiation is a rare but an aggressive tumor. This article reports a nongestational case of a uterine endometrial carcinoma with trophoblastic differentiation. A 54-year-old woman with a history of atypical genital bleeding that underwent semi-radical hysterectomy, bilateral salpingo-oophrectomy, and pelvic lymph nodes dissection. Pathological investigation showed that the tumor had endometrioid adenocarcinoma and choriocarcinomatous components. Although a series of multimodality treatments including craniotomy were performed, she died of aggressive lung and brain metastases one year after the primary surgery.

18F-FDG PET/CT and MR findings of ovarian carcinoid within a dermoid cyst.

Ovarian carcinoid is a rare neoplasm of low-grade malignancy occurring within a dermoid cyst or mucinous tumor, predominantly in perimenopausal women. Reports with radiologic features are scarce. We present a case of a 57-year-old woman with an ovarian carcinoid within a dermoid cyst manifested as a multilocular cystic mass with a solid component showing 18F-FDG PET uptake (SUVmax=6.02).

A case of resected synchronous multiple primary lung cancer comprising adenocarcinoma and carcinoid (cancer-in-cancer).

We performed surgical resection of synchronous multiple primary lung cancer, comprising adenocarcinoma and carcinoid components. The latter tumor was located within the central scar tissue formed by the adenocarcinoma (cancer-in-cancer). The patient was a 67-year-old Japanese woman who had a nodular lesion in the right S1 on chest computed tomography. Lung cancer was diagnosed intraoperatively by rapid needle aspiration cytology, and right upper lobectomy with mediastinal lymph node dissection was performed. Histological examination of the resected specimen revealed cancer-in-cancer, consisting of adenocarcinoma and a carcinoid tumor. The carcinoid component was localized within the central fibrous scar of the dominant adenocarcinoma component.

Management of in situ lobular neoplasia detected on needle core biopsy of breast.

AIMS: To evaluate the risk of having occult ductal carcinoma in situ or invasive carcinoma in the region of a focus of lobular (in situ) neoplasia (LN) diagnosed on needle core biopsy (NCB) of breast. METHODS: All cases of LN diagnosed on NCB of breast over 10 years (2000-2009 inclusive) were reviewed. The clinical presentation, radiological appearances and final pathological diagnosis on open diagnostic biopsy (ODB) were correlated. RESULTS: 125 cases of LN on NCB were identified from diagnostic codes. Of these, 72 (58%) had a coexistent, higher-grade lesion that mandated surgery. Fifty of the remaining 53 (94%) underwent ODB. The majority of patients were asymptomatic, with 68% presenting through the breast screening programme, and in 89% of patients, the target abnormality was microcalcification. Of the 50 patients, 13 (26%) had a final diagnosis of in situ or invasive carcinoma requiring therapeutic surgery. When the cases of pleomorphic LN were excluded, 21% (10/47) were upgraded. Two of these 10 cases had discordant radiology which could have been diagnosed on repeat NCB leaving an upgrade rate of 18% (8/45). In four of the eight cases of invasive malignancy, the disease was multifocal. CONCLUSIONS: LN is frequently asymptomatic, being identified by mammographic microcalcification alone. In 21% of classical LN cases, it is associated with an undiagnosed, higher-grade lesion requiring oncological management. In our view, patients with LN discovered on NCB should undergo open diagnostic biopsy.

Composite renal cell carcinoma with clear cell renal cell carcinomatous and carcinoid tumoral elements: a first case report.

Renal endocrine tumours are extremely rare, and carcinoid tumoral elements in renal cell carcinoma have never been reported. This is the first report of a composite renal cell carcinoma containing a clear cell renal cell carcinoma associated with carcinoid tumoral elements, in a patient with synchronous metastatic disease. In the absence of specific radiological and clinical manifestations, typical morphological features as well as an immunostaining profile of neuroendocrine differentiation were identified by microscopy. Secondary nodal and liver localisations were characterised by carcinoid elements only. Despite antiangiogenic therapy, liver metastasis progressed, suggesting that adjuvant therapy cannot be based on the presence of the clear cell renal cell carcinoma component. In this context, extensive tissue sampling is recommended to reveal the endocrine component that is the most aggressive element of such a composite carcinoma.

Nonhepatocytic malignant mixed tumor of the liver in adults: report of a long surviving case.

A primary nonhepatocytic malignant mixed tumor in the liver contains both epithelial and mesenchymal components, and the incidence in adults is extremely rare. A 45-year-old female was admitted because of abdominal fullness. Abdominal imaging studies revealed a huge cystic tumor with a mural nodule in the right lobe. A right trisegmentectomy and an invaded partial diaphragm resection were performed. Diagnosis was established after surgery. The patient is still alive 11 years after surgery, and to our knowledge is the longest surviving patient with a primary nonhepatocytic malignant mixed primary tumor of the liver.

Positron emission tomography in relation to Noguchi's classification for diagnosis of peripheral non-small-cell lung cancer 2 cm or less in size.

BACKGROUND: F-18 fluorodeoxyglucose positron emission tomography ((18)F-FDG-PET) scanning has become a critically important tool in diagnosis and management of non-small-cell lung cancer. However, the effectiveness of (18)F-FDG-PET as a diagnostic tool for small-sized lung cancer is controversial. The purpose of this study was to examine the accuracy of( 18)F-FDG-PET in relation to Noguchi's classification in the diagnosis of small peripheral non-small-cell lung cancer. METHODS: Between January 2003 and April 2006, 150 patients with peripheral lung lesions who were undergoing chest computed tomography (CT),( 18)F-FDG-PET, and operation were analyzed. RESULTS: Eighty-three patients had malignant lesions, and 67 patients had benign lesions. PET had a sensitivity, specificity, positive predictive value, and negative predictive value of 75.9%, 64.1%, 72.4%, and 68.3%, respectively. In 37 patients with peripheral lung cancer measuring 2.0 cm or less in the greatest diameter, the sensitivity was 51.4% and the specificity was 51.9%. Among them, all 4 cases of Noguchi type A adenocarcinoma [localized bronchioloalveolar carcinoma (LBAC)], 4 of 5 type B and 8 of 17 type C were false negative, while 9 of 11 (81.8%) types D, E, and F (invasive carcinomas without a BAC component) were true positive. CONCLUSION: The accuracy of( 18)F-FDG-PET is generally low in distinguishing malignancy from benign lesions in small lesions (<2.0-cm diameter). The significance of PET as a diagnostic tool is small, especially when the tumor has a ground-glass component at a high rate. The sensitivity of PET is high in small invasive carcinomas without a BAC component, but it is difficult to distinguish carcinoma from benign tumor from its image.

[Carcinosarcoma arising from a preexisting pleomorphic adenoma--true malignant mixed tumor of the parotid gland]. / Miesakorak rozwijajacy sie w obrebie gruczolaka wielopostaciowego--"prawdziwy" zlosliwy guz mieszany slinianki przyusznej.

UNLABELLED: Carcinosarcoma (true malignant mixed tumor) is rare in salivary gland neoplasms. Even rarer is a carcinosarcoma arising from a preexisting pleomorphic adenoma. CASE: a 49-year-old woman was admitted with left neck pain. Computed tomography and USG-examination revealed a mass originating from left parotid gland. The patient undergone total parotidectomy with facial nerve preservation and then irradiation and chemioteraphy. Clinical and histologic date revealed it had developed from a preexisting pleomorphic adenoma. CONCLUSION: microscopic examination showed and neoplasm comprised of sarcomatous and carcinomatous cells. This tumor seemed to consist of two histogenetically different populations of cells. We also review the literature and dicuss the histogenetic origin of the carcinosarcoma of salivary gland.

Extra-adrenal composite pheochromocytoma-ganglioneuroma.

Composite tumors containing pheochromocytoma and ganglioneuroma are rare tumors typically arising in the adrenal glands. Very few were reported outside of the adrenals. We present the case of a middle-aged woman with a retroperitoneal mass that was discovered when she complained of local signs of compression and systemic signs of catecholamine hypersecretion. On pathology the mass was composed of large polygonal and pleomorphic cells arranged in nests characteristic of pheochromocytoma that were mixed with clusters of mature ganglion cells and bundles of spindle cells characteristic of ganglioneuroma. The histological features were benign, but due to its invasion of the inferior vena cava and of the right renal artery, we had concerns about the malignant potential of the pheochromocytoma component, which is extremely rare in these composite tumors.

Potential sampling error in fine needle aspiration biopsy of dedifferentiated chondrosarcoma: a report of 4 cases.

BACKGROUND: Dedifferentiated chondrosarcoma is a rare, poorly understood and often fatal sarcoma that usually manifests as a high grade, non-cartilage-producing sarcoma juxtaposed against a low grade chondrosarcoma. A correct diagnosis requires recognition of both components. In the absence of complete resection, rendering a specific diagnosis on small biopsy specimens, such as fine needle aspiration biopsy (FNAB), may be extraordinarily difficult. CASES: We retrospectively reviewed 4 cytology samples (3 primary, 1 metastatic) from 3 patients with dedifferentiated chondrosarcoma, initially analyzed by FNAB, emphasizing the potential for sampling error. Two women, aged 78 and 57 years, both of whom had prior histories of carcinoma, presented with lesions involving the right and left femur, respectively. One 27-year-old man with multiple osteochondromatosis developed a dedifferentiated chondrosarcoma of the left pelvis. Two primary cytologic specimens consisted of moderately cellular smears containing a spindled to polygonal, nonspecific, pleomorphic sarcoma unaccompanied by definite matrix material; 1 of these had a concomitant core needle biopsy (CNB), also demonstrating pleomorphic sarcoma. The third primary cytologic specimen revealed low grade chondrosarcoma, but a concomitant CNB showed only a high grade, non-matrix-producing sarcoma. The last patient developed a metastasis to the opposite femur; FNAB revealed a high grade spindle cell sarcoma. In none of the FNAB or CNB specimens were both low and high grade components of dedifferentiated chondrosarcoma recognized. However, the diagnosis was strongly suspected based on the clinical and radiographic findings. CONCLUSION: Due to sampling error, the diagnosis of dedifferentiated chondrosarcoma may be difficult to establish by cytologic examination alone. Clinical and radiographic correlation is essential for an accurate diagnosis.