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1.
BMJ Case Rep ; 15(5)2022 May 13.
Article En | MEDLINE | ID: mdl-35568413

A young pregnant woman presented to the emergency department with acute lower abdominal pain. The ultrasound examination showed not only a viable fetus with a gestational age of 24 weeks but also a heteroechoic mass of about 7×7 cm involving parts of the face. The patient was found to be in active labour with rapid progression, and she gave birth to a dead female neonate. The autopsy showed a large malignant mixed tumour of the left parotid gland and revealed infection with cytomegalovirus. To our knowledge, this is the first reported case of a malignant mixed tumour of salivary glands in fetuses.


Carcinoma , Cytomegalovirus Infections , Mixed Tumor, Malignant , Myoepithelioma , Parotid Neoplasms , Carcinoma/pathology , Cytomegalovirus Infections/complications , Cytomegalovirus Infections/pathology , Female , Humans , Infant , Infant, Newborn , Mixed Tumor, Malignant/pathology , Myoepithelioma/pathology , Parotid Gland/pathology , Parotid Neoplasms/pathology , Pregnancy , Salivary Glands/pathology
2.
Medicine (Baltimore) ; 100(40): e27348, 2021 Oct 08.
Article En | MEDLINE | ID: mdl-34622834

RATIONALE: Colorectal mixed neuroendocrine-nonneuroendocrine neoplasms constitute a rare group of gastrointestinal tumors composed by both neuroendocrine and nonneuroendocrine components. Nondiagnostic macroscopic features, specific histological features, and poor awareness of the disease are responsible for the underestimated incidence and conflicting data available. Due to lack of randomized clinical trials and validated clinical guidelines, diagnostic and therapeutic approach are based on the standard of care for pure colorectal neuroendocrine carcinomas or adenocarcinomas. PATIENT CONCERNS: A 76-year-old caucasian male, without relevant medical or familial history, presented a positive faecal occult blood test during colorectal cancer screening. DIAGNOSIS: Total colonoscopy identified a rectal lesion with biopsy showing a moderate rectal adenocarcinoma staged as cT2N0M0. INTERVENTIONS: Anterior resection of the rectum with right ileostomy followed by local radiotherapy with radio-sensitising chemotherapy and adjuvant chemotherapy with capecitabine 1000 mg bid plus oxaliplatin 130 mg/m2. Due to chronic nodular pulmonary aspergillosis and chemotherapy induced immunosuppression patient was on 400 mg/daily of oral voriconazole. OUTCOMES: Overall survival of 15 months after progression under first line treatment and under palliative chemotherapy with platinum plus etoposide regimen. LESSONS: The reported case illustrates the challenge associated to the management of mixed neuroendocrine-nonneuroendocrine carcinomas due to lack of validated guidelines and scientific evidence. From diagnosis and staging to treatment, all steps must be tailored to individual clinical and histological features.


Adenocarcinoma/pathology , Mixed Tumor, Malignant/pathology , Neuroendocrine Tumors/pathology , Rectal Neoplasms/pathology , Adenocarcinoma/therapy , Aged , Antimetabolites, Antineoplastic/administration & dosage , Antineoplastic Combined Chemotherapy Protocols , Capecitabine/administration & dosage , Fatal Outcome , Humans , Male , Mixed Tumor, Malignant/therapy , Neuroendocrine Tumors/therapy , Oxaliplatin/administration & dosage , Radiotherapy , Rectal Neoplasms/therapy , Rectum/surgery
3.
Hum Pathol ; 116: 63-72, 2021 10.
Article En | MEDLINE | ID: mdl-34310982

Primary hepatic neoplasms with neuroendocrine differentiation are extremely rare. Their clinicopathological features and molecular genetic basis are largely unknown. We identified four cases of primary hepatic neoplasms with neuroendocrine differentiation. Electronic medical records were reviewed for clinical history, imaging findings, laboratory results, and follow-up. Pathology slides, immunohistochemistry, and ancillary studies were reviewed. There were two females and two males with age ranging from 52 to 74 years. There was one amphicrine carcinoma with tumor cells simultaneously demonstrating both hepatocellular and neuroendocrine differentiation, one mixed hepatocellular-neuroendocrine carcinoma (NEC) with hepatocellular component intermingled with neuroendocrine component, one small cell NEC, and one well-differentiated neuroendocrine tumor. Next- generation sequencing of the mixed hepatocellular-NEC and small cell NEC showed molecular/genetic alterations commonly seen in hepatocellular carcinoma (HCC). All four cases arose in a background of cirrhosis. Primary hepatic neoplasms arising in cirrhotic livers can have a spectrum of neuroendocrine differentiation. Presence of a NEC component may be an indicator of aggressiveness. In addition, primary hepatic carcinomas with neuroendocrine differentiation likely share the same molecular pathways as HCC.


Carcinoma, Hepatocellular/pathology , Carcinoma, Neuroendocrine/pathology , Liver Neoplasms/pathology , Mixed Tumor, Malignant/pathology , Aged , Carcinoma, Hepatocellular/etiology , Carcinoma, Neuroendocrine/etiology , Female , Humans , Liver Cirrhosis/complications , Liver Neoplasms/etiology , Male , Middle Aged , Mixed Tumor, Malignant/etiology
4.
Am J Otolaryngol ; 42(6): 103135, 2021.
Article En | MEDLINE | ID: mdl-34171696

OBJECTIVE: Malignant mixed tumors of the salivary gland are a group of neoplasms comprised of carcinoma-ex-pleomorphic adenoma, carcinosarcoma, and metastasizing pleomorphic adenoma. An alternative classification, malignant mixed tumor-not otherwise (MMT-NOS), is a diagnosis of exclusion for neoplasms that do not fit the previous histologically profiled subtypes. The objective was to provide a comprehensive assessment of MMT-NOS and determine prognostic factors. METHODS: This retrospective cohort study queried the Surveillance, Epidemiology, and End Results database for patient and tumor characteristics of US patients with MMT-NOS of the major salivary glands from 1973 to 2016. Kaplan-Meier and Cox regression analysis were performed to determine 5-year survival and prognostic factors. RESULTS: 434 patients were identified with a mean age at diagnosis of 61.5 years. The majority of neoplasms were high grade and stage (70.8% grade III/IV; 63.8% stage III/IV). Extraparenchymal extension (40.6%) and lymph node involvement (28.5%) were common; distant metastases (2.4%) were rare. Treatment included surgery (93.0%), radiation (51.6%), and chemotherapy (10.4%). Facial nerve sacrifice was common (50.8%). Median survival was 66.5 months. 5-year overall and disease-specific survival were 65.7% and 83.0%, respectively. In multivariate analysis, nodal involvement (HR 7.0; P < 0.001), surgery-radiation-chemotherapy (HR 6.1; P = 0.02), extraparenchymal extension (HR 2.50; P = 0.04), and tumor size >4 cm (HR 1.3; P = 0.03) were prognostic factors. CONCLUSION: Despite high stage and grade at diagnosis, MMT-NOS portends a good 5-year prognosis and low rate of distant metastasis. Prognostic factors were nodal involvement, tumor size, and extraparenchymal extension.


Adenoma, Pleomorphic/epidemiology , Adenoma, Pleomorphic/mortality , Mixed Tumor, Malignant/mortality , Salivary Gland Neoplasms/mortality , Adenoma, Pleomorphic/pathology , Adenoma, Pleomorphic/therapy , Female , Humans , Lymph Nodes/pathology , Male , Middle Aged , Mixed Tumor, Malignant/epidemiology , Mixed Tumor, Malignant/pathology , Mixed Tumor, Malignant/therapy , Neoplasm Staging , Prognosis , Retrospective Studies , SEER Program , Salivary Gland Neoplasms/epidemiology , Salivary Gland Neoplasms/pathology , Salivary Gland Neoplasms/therapy , Salivary Glands/pathology , Survival Rate
5.
J Cutan Pathol ; 48(6): 775-780, 2021 Jun.
Article En | MEDLINE | ID: mdl-33470448

Chondroid syringoma (CS) represents the cutaneous counterpart of mixed tumor (pleomorphic adenoma) of salivary glands. Definitive diagnosis is made on histopathology and is based on the presence of characteristic epithelial and stromal components. We report a case of an atypical CS arising on the extremity of an elderly male patient. Histomorphologic features of necrosis and cellular atypia raised suspicion for malignant degeneration, an exceptionally rare circumstance in this context. To further support the diagnosis of malignancy, array comparative genomic hybridization was performed from both low and higher grade areas of the tumor. Both regions demonstrated multiple copy number gains and losses, with additional loss of q7p (TP53), loss of 19p, and loss of heterozygosity on16q demonstrated in the more atypical foci. To our knowledge, this is the first case description of malignant degeneration of a CS with correlative microarray analysis. The findings in this case may prove useful in confirming the diagnosis in future ambiguous cases.


Adenoma, Pleomorphic/genetics , Cell Transformation, Neoplastic/genetics , Comparative Genomic Hybridization/methods , Sweat Gland Neoplasms/pathology , Adenoma, Pleomorphic/diagnosis , Adenoma, Pleomorphic/pathology , Adenoma, Pleomorphic/surgery , Aged, 80 and over , Biomarkers, Tumor , Cell Transformation, Neoplastic/pathology , Humans , Male , Mixed Tumor, Malignant/pathology , Skin Neoplasms/pathology , Treatment Outcome
6.
Am J Surg Pathol ; 45(6): 727-741, 2021 06 01.
Article En | MEDLINE | ID: mdl-33284192

One hundred malignant mixed germ cell tumors of the ovary that occurred in patients 3 to 55 years (mean: 20 y) of age are described. The clinical presentation was usually that of any highly malignant tumor of the ovary (abdominal pain and distension), but rarely (3 cases) endocrine manifestations were present. The tumors were usually unilateral (96%), ranged from 4 to 38 cm (mean: 16 cm), and were uniformly solid or, more often, solid and cystic; occasionally the typical appearance of dysgerminoma could be appreciated. The most common tumor type was yolk sac tumor (91%), followed by dysgerminoma (61%), immature teratoma (58%), embryonal carcinoma (38%), and choriocarcinoma (11%). A variety of admixtures were encountered; dysgerminoma and yolk sac tumor was the most common combination (25% of the tumors) with the 2 components often being sharply demarcated. Immature teratoma and yolk sac tumor was the next most common pairing (20%) followed by yolk sac tumor and embryonal carcinoma, with or without immature teratoma (16%). Tumors with a choriocarcinoma component had the most varied combinations of tumor types. Embryoid bodies were seen in 21% of the tumors, most often as fragmented forms arranged in a nodular manner with yolk sac tumor and/or embryonal carcinoma; uncommonly they occurred singly or in clusters. Numerous confluent well-formed embryoid bodies (polyembryoma) were prominent in 2 tumors. Three tumors had a focal diffuse embryoma pattern. The specific tumor types showed the known diverse spectrum of microscopic appearances, but the frequent haphazard arrangement of 2 or more subtypes often resulted in complex morphology. Overgrowth of another neoplastic component, most often primitive neuroectodermal tumor, occurred in 10% of the tumors further complicating the histologic picture. This is the largest series of ovarian malignant mixed germ cell tumors reported and details their characteristics including associations of their subtypes and the frequent apparent role of embryoid bodies in giving rise to yolk sac tumor and embryonal carcinoma components.


Mixed Tumor, Malignant/pathology , Neoplasms, Germ Cell and Embryonal/pathology , Ovarian Neoplasms/pathology , Adolescent , Adult , Carcinoma, Embryonal/pathology , Child , Child, Preschool , Choriocarcinoma/pathology , Dysgerminoma/pathology , Endodermal Sinus Tumor/pathology , Female , Humans , Middle Aged , Neoplasm Staging , Teratoma/pathology , Tumor Burden , Young Adult
7.
Medicine (Baltimore) ; 99(50): e23271, 2020 Dec 11.
Article En | MEDLINE | ID: mdl-33327249

BACKGROUND: Mixed neuroendocrine nonneuroendocrine neoplasms (MiNENs) originating from the biliary system (gallbladder, biliary tract, or ampulla of Vater) are extremely rare and have not been discussed in detail or systematically. We aimed to present the demographics, clinicopathological characteristics, management, and prognostic factors of biliary MiNENs. METHODS: A systematic search of electronic biomedical databases (Web of Science, PUBMED, and Embase) was performed to identify eligible studies. Survival was analyzed with the Kaplan-Meier method. Log-rank tests were used to evaluate the differences between groups, and the effects of various clinical and histopathological features on prognosis were analyzed by univariate and multivariate Cox regression. RESULTS: Fifty-three publications (patients, n = 67) were included. The median overall survival time was 21.0 months. Fifty-one patients (76.1%) underwent radical surgery and median survival for 41 months (P < .001). Twenty-two patients who received adjuvant radiochemotherapy treatment after radical surgery had a median survival for 43 months (P = .076). Radical resection (P < .001), Ki-67 index (P = .011), tumor stage (P < .001), neuroendocrine (NEC) grade (P = .011), and non-NEC grade (P = .017) were independent statistically significant prognostic factors according to univariate analysis; radical resection (P = .010) and small morphological subtype (P = .036) were independent statistically significant prognostic factors associated with higher overall survival according to multivariate analysis, and radical resection (P = .005) and age < 65 years (P = .026) were associated with higher recurrence free survival time. CONCLUSION: Radical resection is essential for long-term survival. Aggressive multimodality therapy with adjuvant radiochemotherapy and biotherapy may improve survival of biliary MiNENs. Further randomized controlled trials are needed to determine the standard treatment.


Biliary Tract Neoplasms/pathology , Carcinoma, Neuroendocrine/pathology , Mixed Tumor, Malignant/pathology , Neuroendocrine Tumors/pathology , Biliary Tract/pathology , Biliary Tract Neoplasms/diagnosis , Biliary Tract Neoplasms/therapy , Carcinoma, Neuroendocrine/diagnosis , Carcinoma, Neuroendocrine/therapy , Humans , Mixed Tumor, Malignant/diagnosis , Mixed Tumor, Malignant/therapy , Neuroendocrine Tumors/diagnosis , Neuroendocrine Tumors/therapy , Prognosis , Survival Analysis
8.
Rev. senol. patol. mamar. (Ed. impr.) ; 33(3): 112-115, jul.-sept. 2020. ilus
Article Es | IBECS | ID: ibc-197295

OBJETIVO: El adenoma pleomorfo es un tumor con una gran heterogenicidad arquitectural y citológica, de ahí su denominación como tumor mixto. Compuesto característicamente por células epiteliales y mioepiteliales con estroma mesenquimal, presenta un comportamiento benigno, siendo su localización habitual las glándulas salivares. La mama es una localización muy infrecuente para este tipo de tumores. CASO CLÍNICO: Presentamos el caso clínico de una paciente de 76 años sin antecedentes de patología mamaria, valorada en consultas externas de cirugía por una secreción unilateral de aspecto serosanguinolento desde la mama derecha, asociada a un nódulo de unos 2cm de diámetro mayor. Tras completar su estudio, se decide exéresis de la tumoración, obteniendo como resultado en la anatomía patológica una tumoración heterogénea con predominio de células miopiteliales; descripción compatible con un adenoma pleomorfo. DISCUSIÓN: El adenoma pleomorfo de la mama, es una tumoración epitelial benigna que tiene un patrón histológico similar al del tumor de las glándulas salivares. Los rasgos histológicos más importantes es la metaplasia ósea y mixocondroide, junto con presencia de calcificaciones, tejido epitelial (con o sin diferenciación glandular), y tejido mioepitelial; siendo esta gran variedad estructural lo que le caracteriza. Hay que realizar un diagnóstico diferencial con patología maligna a la cual asemeja. Se presenta de forma poco frecuente a nivel mamario y suele ser una lesión bien delimitada y solitaria, elastica y no adherida. Pese a su comportamiento benigno (no ha presentado en ninguno de los casos documentados metástasis a distancia) el tratamiento es quirúrgico, asegurando buenos margenes de resección para evitar recidivas


OBJECTIVE: Pleomorphic adenoma shows wide architectural and cytological heterogeneity and is consequently classified as a mixed tumour. These benign adenomas are characteristically composed of epithelial and myoepithelial cells with mesenchymal stroma and are usually located in the salivary glands. Localisation in the breast is extremely infrequent for this type of tumour. CASE REPORT: We report the case of a 76-year-old female patient with no prior history of breast disease who was assessed in the surgery outpatient clinic for unilateral serosanguinous secretion from the right breast associated with a nodule of approximately 2cm in diameter. After workup, it was decided to extirpate the tumour. Histological findings indicated a heterogeneous tumour with a predominance of myoepithelial cells, compatible with pleomorphic adenoma. DISCUSSION: Pleomorphic adenoma of the breast is a benign epithelial tumour whose histological pattern is similar to that of pleomorphic adenoma of the salivary glands. The most important histological features are osseous and myxochondroid metaplasia, together with the presence of calcifications, epithelial tissue (with or without glandular differentiation) and myoepithelial tissue. This wide structural variety is what characterises these tumours. The differential diagnosis should be performed with malignant disease, which these adenomas frequently mimic. Pleomorphic adenomas are very infrequent in the breast and are usually well-demarcated, solitary, elastic and non-adherent tumours. Despite their benign behaviour (there have been no reported cases of distant metastases), treatment is surgical, with good resection margins to avoid recurrences


Humans , Female , Aged , Adenoma, Pleomorphic/pathology , Breast Neoplasms/pathology , Mixed Tumor, Malignant/pathology , Nipple Discharge/cytology , Immunohistochemistry/methods , Salivary Gland Neoplasms/pathology
9.
Endocr J ; 67(10): 1007-1017, 2020 Oct 28.
Article En | MEDLINE | ID: mdl-32522910

Medullary thyroid carcinoma (MTC) may mimic mixed medullary and follicular thyroid carcinoma (MMFTC). MTC originates from para-follicular cells, while MMFTC is an uncommon tumor characterized by coexistence of follicular and para-follicular cell-derived tumor populations. A 35-year-old woman was diagnosed with MTC but showed a hot nodule in thyroid scintigraphy. The tumor included diffusely-spread follicular lesions within it, which were immunostained with thyroglobulin and calcitonin. Immunofluorescence showed the presence of several tumor cells that were double-stained with thyroglobulin and calcitonin. To clarify whether or not the tumor was MMFTC, we used duplex in situ hybridization (ISH). Thyroglobulin and calcitonin-related polypeptide alpha mRNA were not expressed together in a single cell, so we suspected false-positive staining of tumor cells with thyroglobulin. To make comparisons with other follicular lesions in MTC, we searched our hospital database. Five cases within a ten-year period had been pathologically diagnosed as MTC. All had follicular lesions in the tumor, but unlike the other case, they were peripherally localized. Dual differentiation into follicular or para-follicular tumor cells was not indicated by either immunofluorescence or duplex ISH. Compared with the case suspected to be MMFTC, there was only mild invasion of tumor cells into the follicular epithelium. The extent of follicular lesions and invasiveness of tumor cells may be associated with pseudo-staining of thyroglobulin in MTC. Duplex ISH can distinguish MTC that are stained with thyroglobulin from MMFTC.


Adenocarcinoma, Follicular/metabolism , Carcinoma, Neuroendocrine/metabolism , Mixed Tumor, Malignant/metabolism , Procalcitonin/metabolism , Thyroglobulin/metabolism , Thyroid Neoplasms/metabolism , Adenocarcinoma, Follicular/diagnosis , Adenocarcinoma, Follicular/pathology , Adult , Aged , Calcitonin/metabolism , Carcinoma, Neuroendocrine/diagnosis , Carcinoma, Neuroendocrine/pathology , Diagnosis, Differential , False Positive Reactions , Female , Humans , In Situ Hybridization , Male , Middle Aged , Mixed Tumor, Malignant/diagnosis , Mixed Tumor, Malignant/pathology , Neoplasm Invasiveness , RNA, Messenger/metabolism , Radionuclide Imaging , Thyroid Neoplasms/diagnosis , Thyroid Neoplasms/pathology
11.
Clin J Gastroenterol ; 13(1): 110-115, 2020 Feb.
Article En | MEDLINE | ID: mdl-31264080

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.


Adenocarcinoma/pathology , Carcinoma, Neuroendocrine/pathology , Carcinosarcoma/pathology , Chondrosarcoma/pathology , Gallbladder Neoplasms/pathology , Mixed Tumor, Malignant/pathology , Adenocarcinoma/complications , Adenocarcinoma/diagnostic imaging , Adenocarcinoma/metabolism , Aged , CDX2 Transcription Factor/metabolism , Carcinoma/complications , Carcinoma/diagnostic imaging , Carcinoma/metabolism , Carcinoma/pathology , Carcinoma, Neuroendocrine/complications , Carcinoma, Neuroendocrine/diagnostic imaging , Carcinoma, Neuroendocrine/metabolism , Carcinosarcoma/complications , Carcinosarcoma/diagnostic imaging , Carcinosarcoma/metabolism , Cholecystectomy , Cholecystitis, Acute/complications , Cholecystitis, Acute/diagnostic imaging , Cholecystitis, Acute/surgery , Cholecystolithiasis/complications , Cholecystolithiasis/diagnostic imaging , Cholecystolithiasis/surgery , Choledocholithiasis/complications , Choledocholithiasis/diagnostic imaging , Choledocholithiasis/surgery , Chondrosarcoma/complications , Chondrosarcoma/diagnostic imaging , Chondrosarcoma/metabolism , Female , Gallbladder Neoplasms/complications , Gallbladder Neoplasms/diagnostic imaging , Gallbladder Neoplasms/metabolism , Humans , Ki-67 Antigen/metabolism , Mixed Tumor, Malignant/complications , Mixed Tumor, Malignant/diagnostic imaging , Mixed Tumor, Malignant/metabolism , Mucin-1/metabolism , Proto-Oncogene Proteins c-kit/metabolism , S100 Proteins/metabolism , Tomography, X-Ray Computed
12.
Clin J Gastroenterol ; 13(1): 37-45, 2020 Feb.
Article En | MEDLINE | ID: mdl-31342462

Mixed adenoneuroendocrine carcinoma (MANEC) is defined as a tumor composed of both adenocarcinoma and neuroendocrine components. Here, we report the case of a 75-year-old woman with ampullary MANEC. She visited a physician with the chief complaint of dark urine and was diagnosed with advanced jaundice. Subsequently, she was referred to our hospital. Contrast-enhanced computed tomography scan revealed a neoplastic lesion measuring approximately 2 cm with a contrast effect at the duodenal papilla. Upper endoscopy showed a non-exposed tumor at the duodenal papilla. After biliary drainage, a subtotal stomach-preserving pancreaticoduodenectomy was performed. Histopathological examination revealed that the tumor components were composed of circular-to-oval atypical cells admixed with tubular adenocarcinoma tissue. These atypical cells were immunohistochemically positive for synaptophysin and diagnosed as neuroendocrine carcinoma with a Ki-67 labeling index of 63%. The patient was diagnosed with MANEC with a neuroendocrine carcinoma component of approximately 40%. The neuroendocrine carcinoma component had metastasized to the posterior pancreatic lymph nodes. Despite starting adjuvant chemotherapy with S-1, computed tomography revealed the presence of multiple liver metastases within 4 months after surgery. MANEC with neuroendocrine carcinoma is well known to have an extremely poor prognosis. Therefore, establishing a multidisciplinary therapy including chemotherapy is crucial.


Adenocarcinoma/pathology , Ampulla of Vater , Carcinoma, Neuroendocrine/pathology , Common Bile Duct Neoplasms/pathology , Mixed Tumor, Malignant/pathology , Adenocarcinoma/complications , Adenocarcinoma/diagnosis , Adenocarcinoma/surgery , Aged , Carcinoma, Neuroendocrine/complications , Carcinoma, Neuroendocrine/diagnosis , Carcinoma, Neuroendocrine/surgery , Cholangiopancreatography, Endoscopic Retrograde , Common Bile Duct Neoplasms/complications , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/surgery , Female , Humans , Jaundice, Obstructive/etiology , Mixed Tumor, Malignant/complications , Mixed Tumor, Malignant/diagnosis , Mixed Tumor, Malignant/surgery , Pancreaticoduodenectomy , Tomography, X-Ray Computed
14.
World Neurosurg ; 132: 87-92, 2019 Dec.
Article En | MEDLINE | ID: mdl-31470154

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.


Brain Neoplasms/pathology , Endodermal Sinus Tumor/pathology , Mixed Tumor, Malignant/pathology , Pineal Gland , Brain Neoplasms/complications , Brain Neoplasms/diagnostic imaging , Brain Neoplasms/therapy , Consciousness Disorders/etiology , Endodermal Sinus Tumor/complications , Endodermal Sinus Tumor/diagnostic imaging , Endodermal Sinus Tumor/therapy , Fatal Outcome , Gait Disorders, Neurologic/etiology , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Mixed Tumor, Malignant/complications , Mixed Tumor, Malignant/diagnostic imaging , Mixed Tumor, Malignant/therapy , Neoplasms, Germ Cell and Embryonal/complications , Neoplasms, Germ Cell and Embryonal/diagnostic imaging , Neoplasms, Germ Cell and Embryonal/pathology , Neoplasms, Germ Cell and Embryonal/therapy , Ocular Motility Disorders/etiology , Positron-Emission Tomography , Tomography, X-Ray Computed
15.
Prog. obstet. ginecol. (Ed. impr.) ; 62(2): 150-155, mar.-abr. 2019. ilus, tab
Article Es | IBECS | ID: ibc-184911

El diagnóstico de un tumor phyllodes asociado a un carcinoma de mama es muy raro (1-2% de todos los tumores phyllodes). Este tipo de asociación se produce tras la transformación maligna del componente epitelial del tumor phyllodes. Se presenta el caso de una mujer de 46 años, que consulta al presentar un nódulo en mama derecha de rápido crecimiento. Tras valoración clínica, radiológica e histológica, y con el diagnóstico de tumor phyllodes benigno, se practicó una excisión local amplia. El estudio anatomopatológico definitivo informó de la presencia de una tumoración bifásica con un tumor phyllodes de bajo grado de malignidad en el componente estromal y un carcinoma ductal infiltrante en el componente epitelial, presente únicamente en forma de émbolos tumorales en la cápsula del tumor phyllodes


The diagnosis of a phyllodes tumor associated with a breast carcinoma is very rare (1-2% of all phyllodes tumors). This type of association occurs after the malignant transformation of the epitelial component of the phyllodes tumor. We present the case of a 46-year-old woman who consulted for a fast-growing nodule in the right breast. After clinical, radiological and histological evaluation, and with the diagnosis of benign phyllodes tumor, a wide local excision was performed. The anatomopathological study reported the presence of a biphasic tumor with a phyllodes tumor of low grade of malignancy in the stromal component, and an infiltrating ductal carcinoma in the epitelial component, in the form of tumor emboli in the phyllodes tumor capsule


Humans , Female , Middle Aged , Phyllodes Tumor/pathology , Carcinoma, Ductal, Breast/pathology , Breast Neoplasms/pathology , Mixed Tumor, Malignant/pathology , Mastectomy, Simple/methods , Biopsy, Large-Core Needle/methods
16.
World J Surg Oncol ; 17(1): 55, 2019 Mar 22.
Article En | MEDLINE | ID: mdl-30902091

BACKGROUND: Mixed neuroendocrine-non-neuroendocrine tumors (MINEN) of the gallbladder are extremely rare; indeed, the English expert literature reports a mere handful of case reports and case series on this topic. According to the WHO classification of 2010, MINEN are considered to be tumors consisting of two major components, neuroendocrine and non-neuroendocrine, each of which hosts at least 30% of the total cellular population. To date, the etiology and pathogenesis of MINEN have not been precisely determined and the non-specific symptoms generally result in late diagnosis (mainly in the terminal stages of the condition) and contribute to the generally poor prognosis. As far as the management of the disease is concerned, radical surgery plays a crucial role; however, the significance of surgical debulking and biological therapy applying somatostatin analogues has not yet been determined. CASE PRESENTATION: A 56-year-old female was referred to our department for a rapidly progressing tumor in the subhepatic area along with the infiltration of S5 and S6 liver segments. With regard to preoperative findings, the tumor appeared as operable, although, during the surgery, an extensive involvement of the hepatoduodenal ligament by the tumor through the lymph nodes was revealed. Due to acute perioperative bleeding from the necrotic tumor, we decided to perform modified resection. Histologically, the tumor was confirmed as MINEN of gallbladder, where the neuroendocrine component was dominant over the non-neuroendocrine component. Six weeks after the discharge, the patient underwent a follow-up CT revealing large recurrence of the disease. Thereafter, the patient was started on systemic therapy with etoposide and carboplatin in combination with somatostatin analogues. Thirteen months after the surgery, the patient is in good clinical condition, and while a recently performed PET/MRI scan revealed a hepatic lesion and hilar lymphadenopathy in full regression, there was a spread of small peritoneal and pleural metastases. The patient remains in the follow-up care. CONCLUSIONS: The occurrence of mixed neuroendocrine-non-neuroendocrine neoplasms is extremely rare. Radical surgery remains the only potentially effective approach to the cure of this disease. The role of biological therapy and debulking in the management of the disease has not yet been precisely defined. In our experience, both of these methods have the potential to positively influence overall survival rates and the postoperational quality of life of patients.


Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Carcinoma, Neuroendocrine/therapy , Cholecystectomy/methods , Gallbladder Neoplasms/therapy , Mixed Tumor, Malignant/therapy , Neoplasm Recurrence, Local/therapy , Carcinoma, Neuroendocrine/diagnosis , Carcinoma, Neuroendocrine/pathology , Female , Gallbladder/diagnostic imaging , Gallbladder/pathology , Gallbladder/surgery , Gallbladder Neoplasms/diagnosis , Gallbladder Neoplasms/pathology , Humans , Middle Aged , Mixed Tumor, Malignant/diagnosis , Mixed Tumor, Malignant/pathology , Neoplasm Recurrence, Local/diagnostic imaging , Tomography, X-Ray Computed , Treatment Outcome
17.
Pathol Int ; 68(11): 618-623, 2018 Nov.
Article En | MEDLINE | ID: mdl-30311995

Small cell lung carcinoma (SCLC) usually grows in a pure form with no other associated histological components. However, combined small cell lung carcinoma (cSCLC), which is accompanied by other histological components (cSCLCs) may sometimes occur. Herein, we analyzed the tumorigenesis of cSCLC containing a demarcated area of pure SCLC. A 76-year-old man had a 25-mm mass in the perihilar portion of his right upper lung. Histologically, the cSCLC contained two relatively demarcated areas: one area composed of pure SCLC cells and another area of SCLC, squamous-like component (SLC), and spindle cell carcinoma (SpCC) cells. Loss of heterozygosity (LOH) was observed at allele 3p in all tumor components and at 22q in the pure SCLC component. Histological and immunohistochemical analysis and LOH study suggested that all components were likely to be monoclonal in origin and revealed that the pure SCLC component was not the precursor of the cSCLC. In the tumorigenesis of this case, the pure SCLC and the cSCLC may have originated from a common pluripotent tumor cell and then diverged, although we cannot state this conclusively. Further studies with more cases are necessary to test this theory.


Lung Neoplasms/pathology , Mixed Tumor, Malignant/pathology , Small Cell Lung Carcinoma/pathology , Aged , Carcinogenesis/pathology , Humans , Male
18.
Zhonghua Bing Li Xue Za Zhi ; 47(7): 536-541, 2018 Jul 08.
Article Zh | MEDLINE | ID: mdl-29996320

Objective: To investigate the clinicopathologic features, immunophenotype, pathological diagnosis and treatment of malignant mixed tumor (MMT). Methods: Clinical and pathological features including immunohistochemical phenotypes were analyzed in a case of MMT accompanied with eccrine porocarcinoma (EP) involving both hands, diagnosed definitely in January 2018 along with review of relevant literature. Results: A 64-year-old man presented with multiple rash on both hands for 4 years. Three lesions of 0.5 to 2.2 cm were removed for pathological evaluation. The pathological changes on little finger of left and right hands were MMT with EP, whereas that removed from the right ring finger was EP. MMT showed infiltrative growth with vascular wall invasion and consisted of epithelial (glandular or tube differentiation) and mesenchymal components (mucinous and/or cartilage stroma). The endothelial cells showed moderate to severe cytological atypia, nuclear pleomorphism and increased mitotic activity. The glandular component had histological characteristics of syringocarcinoma with moderately atypical chondrocytes but without myoepithelium. EP was composed of basal cells with visible vacuoles in cytoplasm and the presence of tubular and squamous differentiation, along with obvious atypia. Immunohistochemically cavosurface epithelium of glandular differentiation of MMT showed positivity for CK7, EMA and CD117. Myoepithelium showed S-100, CK5/6 and p63 positivity and stromal cells were positive for S-100. Differential diagnoses included metaplastic carcinoma, malignant myoepithelioma and atypical mixed tumor of skin. Conclusions: MMT with EP is extremely rare.The diagnosis of MMT depends on the morphologic features. Immunohistochemical staining is helpful for differential diagnosis. Surgical excision with safety margins is the treatment of choice. Complementary radiotherapy and/or chemotherapy is still controversial. The clinical course of MMT is deemed unpredictable and long-term follow-up is necessary.


Eccrine Porocarcinoma/pathology , Mixed Tumor, Malignant/pathology , Skin Neoplasms/pathology , Sweat Gland Neoplasms/pathology , Diagnosis, Differential , Eccrine Porocarcinoma/chemistry , Epithelium , Humans , Immunohistochemistry , Male , Middle Aged , Mixed Tumor, Malignant/chemistry , Myoepithelioma/chemistry , Myoepithelioma/pathology , Proto-Oncogene Proteins c-kit/analysis , Skin Neoplasms/chemistry , Sweat Gland Neoplasms/chemistry
19.
Rofo ; 190(10): 955-966, 2018 10.
Article En, De | MEDLINE | ID: mdl-29913520

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.


Cerebral Ventricle Neoplasms/diagnostic imaging , Glioma, Subependymal/diagnostic imaging , Magnetic Resonance Imaging , Tomography, X-Ray Computed , Adult , Aged , Aged, 80 and over , Cerebral Ventricle Neoplasms/pathology , Cerebral Ventricles/diagnostic imaging , Cerebral Ventricles/pathology , Contrast Media/administration & dosage , Ependymoma/diagnostic imaging , Ependymoma/pathology , Female , Follow-Up Studies , Glioma, Subependymal/pathology , Humans , Male , Middle Aged , Mixed Tumor, Malignant/diagnostic imaging , Mixed Tumor, Malignant/pathology , Organometallic Compounds , Retrospective Studies , Tumor Burden
20.
Breast Dis ; 37(3): 169-175, 2018.
Article En | MEDLINE | ID: mdl-29504519

Metaplastic carcinoma of the breast with mesenchymal differentiation (MCMD), previously known as carcinosarcoma, is a very rare and aggressive tumor that has been recently classified as a subtype of metaplastic breast carcinoma. It accounts for 0.08%-0.2% of all breast cancers, with only a few cases reported in the literature. Histologically, MCMD is characterized by a biphasic pattern of malignant epithelial and sarcomatous components without evidence of a transition zone between the two elements. We herein describe a unique case of metaplastic carcinoma of the breast with chondrosarcomatous differentiation in a postmenopausal woman who presented with a large, rapidly growing, ulcerated, bleeding mass and signs of impending sepsis. Metaplastic breast carcinomas (MBC) are rare and aggressive tumors. They are characterized by larger size, lower rates of axillary node involvement, higher rates of triple negativity and distal metastases, earlier local recurrence and poorer survival compared with classic invasive breast cancer. Because of the rarity of MBC, the optimal treatment has not been well defined. Surgery is the main curative treatment modality since MBC has shown a suboptimal response to standard chemotherapy. Patients with MBC may be appropriate candidates for novel targeted therapies.


Breast Neoplasms/diagnosis , Carcinoma, Ductal, Breast/diagnosis , Mixed Tumor, Malignant/diagnosis , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Breast Neoplasms/drug therapy , Breast Neoplasms/pathology , Breast Neoplasms/surgery , Carcinoma, Ductal, Breast/drug therapy , Carcinoma, Ductal, Breast/pathology , Carcinoma, Ductal, Breast/surgery , Chemotherapy, Adjuvant , Diagnosis, Differential , Disease-Free Survival , Female , Humans , Mastectomy , Metaplasia/pathology , Middle Aged , Mixed Tumor, Malignant/drug therapy , Mixed Tumor, Malignant/pathology , Mixed Tumor, Malignant/surgery , Neoplasm Staging , Receptor, ErbB-2/metabolism
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