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1.
Cancer Genomics Proteomics ; 16(5): 345-351, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31467228

RESUMO

BACKGROUND/AIM: Malignant chondroid syringoma is a rare tumor of unknown pathogenesis. MATERIALS AND METHODS: Genetic analyses were performed on a malignant chondroid syringoma. RESULTS: G-banding analysis of short-term cultured tumor cells yielded the karyotype 46,Y,t(X;6)(p11;p21)[15]/46,XY[2]. RNA sequencing detected an in-frame fusion of PHF1 from 6p21 with TFE3 from Xp11, verified by RT-PCR and Sanger sequencing. Genomic PCR showed that the PHF1-TFE3 junction was identical to the fusion found by RNA sequencing and RT-PCR. CONCLUSION: Malignant chondroid syringoma is genetically related to tumors with PHF1 rearrangements such as low-grade endometrial sarcoma and ossifying fibromyxoid tumor, but also with tumors having TFE3 rearrangements such as renal cell carcinoma, alveolar soft part sarcoma, PEComa, and epithelioid hemangioendothelioma. Further investigations on malignant chondroid syringomas are needed in order to determine whether genetic heterogeneity exists among them and the clinical impact of the PHF1-TFE3 fusion.


Assuntos
Adenoma Pleomorfo/genética , Fatores de Transcrição de Zíper de Leucina e Hélice-Alça-Hélix Básicos/genética , Proteínas de Ligação a DNA/genética , Tumor Misto Maligno/genética , Proteínas do Grupo Polycomb/genética , Neoplasias Cutâneas/genética , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Cutâneas/patologia
2.
World Neurosurg ; 132: 87-92, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31470154

RESUMO

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.


Assuntos
Neoplasias Encefálicas/patologia , Tumor do Seio Endodérmico/patologia , Tumor Misto Maligno/patologia , Glândula Pineal , Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/terapia , Transtornos da Consciência/etiologia , Tumor do Seio Endodérmico/complicações , Tumor do Seio Endodérmico/diagnóstico por imagem , Tumor do Seio Endodérmico/terapia , Evolução Fatal , Transtornos Neurológicos da Marcha/etiologia , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Tumor Misto Maligno/complicações , Tumor Misto Maligno/diagnóstico por imagem , Tumor Misto Maligno/terapia , Neoplasias Embrionárias de Células Germinativas/complicações , Neoplasias Embrionárias de Células Germinativas/diagnóstico por imagem , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Embrionárias de Células Germinativas/terapia , Transtornos da Motilidade Ocular/etiologia , Tomografia por Emissão de Pósitrons , Tomografia Computadorizada por Raios X
3.
Medicine (Baltimore) ; 98(23): e16000, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31169743

RESUMO

RATIONALE: Mixed adenoneuroendocrine carcinoma (MANEC) is a rare neoplasm, and consensus on the treatment is unavailable. PATIENT CONCERN: A 60-year-old Chinese man presented with obstructive symptoms while eating and paroxysmal stomach pain for more than a month. DIAGNOSIS: MANEC was diagnosed based on clinical manifestations, imaging findings, and pathological examinations. INTERVENTIONS: The patient underwent radical gastrectomy and received XELOX adjuvant chemotherapy (oxaliplatin 200 mg day 1 + capecitabine 1.5 g twice a day) after surgery. OUTCOMES: After 4 cycles of XELOX adjuvant chemotherapy were administered, abdominal computerized tomography and liver magnetic resonance showed liver metastasis. LESSONS: The therapy of gastric MANEC is based on surgical operation, and adjuvant chemotherapy program has an important influence on its prognosis. Therefore, further studying the effectiveness of XELOX adjuvant chemotherapy for gastric MANEC is necessary.


Assuntos
Adenoma/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Carcinoma Neuroendócrino/tratamento farmacológico , Desoxicitidina/análogos & derivados , Fluoruracila/análogos & derivados , Tumor Misto Maligno/tratamento farmacológico , Neoplasias Gástricas/tratamento farmacológico , Quimioterapia Adjuvante , Desoxicitidina/administração & dosagem , Fluoruracila/administração & dosagem , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
4.
World J Surg Oncol ; 17(1): 55, 2019 Mar 22.
Artigo em Inglês | MEDLINE | ID: mdl-30902091

RESUMO

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.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Carcinoma Neuroendócrino/terapia , Colecistectomia/métodos , Neoplasias da Vesícula Biliar/terapia , Tumor Misto Maligno/terapia , Recidiva Local de Neoplasia/terapia , Carcinoma Neuroendócrino/diagnóstico , Carcinoma Neuroendócrino/patologia , Feminino , Vesícula Biliar/diagnóstico por imagem , Vesícula Biliar/patologia , Vesícula Biliar/cirurgia , Neoplasias da Vesícula Biliar/diagnóstico , Neoplasias da Vesícula Biliar/patologia , Humanos , Pessoa de Meia-Idade , Tumor Misto Maligno/diagnóstico , Tumor Misto Maligno/patologia , Recidiva Local de Neoplasia/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Resultado do Tratamento
5.
Urology ; 124: 91-97, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30452964

RESUMO

OBJECTIVE: To describe a family in which 3 members presented with mixed epithelial tumor of the kidney (MEST) and were found to possess a germline mutation in CDC73, a gene which is associated with hyperparathyroidism-jaw tumor syndrome (HPT-JT). MATERIALS AND METHODS: Blood and tumor DNA from three family members who presented with a primary diagnosis of MEST was subjected to targeted gene sequencing to identify potential genetic components. RESULTS: A germline start codon mutation (p.M1I) in CDC73 was identified in all 3 family members who presented with MEST and 2 tumors from 1 patient demonstrated somatic copy-neutral loss of heterozygosity. Patients presented with no evidence of hyperparathyroidism or jaw tumors, but both female patients had hysterectomies at an early age due to excessive bleeding and numerous fibroids, which is common in HPT-JT. A germline p.M1I mutation has been previously reported in a family with clinical features of HPT-JT. CONCLUSION: Patients with MEST may be at risk for HPT-JT and CDC73 germline mutation testing of MEST patients should be considered.


Assuntos
Mutação em Linhagem Germinativa , Neoplasias Renais/genética , Tumor Misto Maligno/genética , Proteínas Supressoras de Tumor/genética , Adenoma/genética , Idoso , Feminino , Fibroma/genética , Humanos , Hiperparatireoidismo/genética , Neoplasias Maxilomandibulares/genética , Masculino , Pessoa de Meia-Idade , Linhagem
6.
Pathol Int ; 68(11): 618-623, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30311995

RESUMO

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.


Assuntos
Neoplasias Pulmonares/patologia , Tumor Misto Maligno/patologia , Carcinoma de Pequenas Células do Pulmão/patologia , Idoso , Carcinogênese/patologia , Humanos , Masculino
7.
Zhonghua Bing Li Xue Za Zhi ; 47(7): 536-541, 2018 Jul 08.
Artigo em Chinês | MEDLINE | ID: mdl-29996320

RESUMO

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.


Assuntos
Porocarcinoma Écrino/patologia , Tumor Misto Maligno/patologia , Neoplasias Cutâneas/patologia , Neoplasias das Glândulas Sudoríparas/patologia , Diagnóstico Diferencial , Porocarcinoma Écrino/química , Epitélio , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Tumor Misto Maligno/química , Mioepitelioma/química , Mioepitelioma/patologia , Proteínas Proto-Oncogênicas c-kit/análise , Neoplasias Cutâneas/química , Neoplasias das Glândulas Sudoríparas/química
8.
Rofo ; 190(10): 955-966, 2018 10.
Artigo em Inglês, Alemão | MEDLINE | ID: mdl-29913520

RESUMO

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.


Assuntos
Neoplasias do Ventrículo Cerebral/diagnóstico por imagem , Glioma Subependimal/diagnóstico por imagem , Imagem por Ressonância Magnética , Tomografia Computadorizada por Raios X , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias do Ventrículo Cerebral/patologia , Ventrículos Cerebrais/diagnóstico por imagem , Ventrículos Cerebrais/patologia , Meios de Contraste/administração & dosagem , Ependimoma/diagnóstico por imagem , Ependimoma/patologia , Feminino , Seguimentos , Glioma Subependimal/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Tumor Misto Maligno/diagnóstico por imagem , Tumor Misto Maligno/patologia , Compostos Organometálicos , Estudos Retrospectivos , Carga Tumoral
9.
Orv Hetil ; 159(19): 741-747, 2018 May.
Artigo em Húngaro | MEDLINE | ID: mdl-29730944

RESUMO

INTRODUCTION AND AIM: The aim of our study was to evaluate the prognostic factors and treatment options of a very rare and highly aggressive type of uterine neoplasms, the malignant mixed Müllerian tumor, known as carcinosarcoma. METHOD: Between 2009 and 2017, 29 patients were treated with malignant mixed Müllerian tumor. At stage I, surgery and postoperative radiotherapy were performed. At stages II-IV, trimodal treatment (surgery, chemotherapy and radiotherapy) was administered. RESULTS: The average age of patients was 68.51 (49-90) years, mean body mass index was 30.22 (20.90-37.22). We have experienced recurrence of disease after complete resection in 6 cases (4 of 6 patients did not accept radiation therapy). Local recurrence has occurred after an average 15.52 (6-36) months, distant metastasis with an average 19.2 (8-32) months. Overall survival was 11.92 (1-75) months. Six patients are free of tumours at the moment. CONCLUSIONS: As overall survival has not increased in recent decades by using combined chemotherapy, there is no congruent consensus associated with the optimal treatment. The standard surgical treatment is total abdominal hysterectomy with bilateral oophorectomy, although due to high rates of recurrence and metastases, the necessity of lymphadenectomy and postoperative treatment is in the focus of recent studies. Though postoperative irradiation improves local control, the beneficial effect on overall survival is still not proven. Adjuvant chemotherapy decreases the rate of both pelvic and extrapelvic recurrence at the same time, although there is no recommendation for the optimal chemoterapeutic agent. Multimodal therapy should lead to better outcomes. Recently there are many ongoing studies with biologic and target therapies to improve efficiency, however, the relevant results will be disclosed in many years only, due to the small number of patients. Orv Hetil. 2018; 159(19): 741-7747.


Assuntos
Tumor Misto Maligno/mortalidade , Tumor Mulleriano Misto/mortalidade , Neoplasias Uterinas/mortalidade , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Tumor Misto Maligno/tratamento farmacológico , Tumor Misto Maligno/radioterapia , Tumor Misto Maligno/cirurgia , Tumor Mulleriano Misto/tratamento farmacológico , Tumor Mulleriano Misto/radioterapia , Tumor Mulleriano Misto/cirurgia , Prognóstico , Resultado do Tratamento , Neoplasias Uterinas/tratamento farmacológico , Neoplasias Uterinas/radioterapia , Neoplasias Uterinas/cirurgia
10.
Breast Dis ; 37(3): 169-175, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29504519

RESUMO

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.


Assuntos
Neoplasias da Mama/diagnóstico , Carcinoma Ductal de Mama/diagnóstico , Tumor Misto Maligno/diagnóstico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias da Mama/tratamento farmacológico , Neoplasias da Mama/patologia , Neoplasias da Mama/cirurgia , Carcinoma Ductal de Mama/tratamento farmacológico , Carcinoma Ductal de Mama/patologia , Carcinoma Ductal de Mama/cirurgia , Quimioterapia Adjuvante , Diagnóstico Diferencial , Intervalo Livre de Doença , Feminino , Humanos , Mastectomia , Metaplasia/patologia , Pessoa de Meia-Idade , Tumor Misto Maligno/tratamento farmacológico , Tumor Misto Maligno/patologia , Tumor Misto Maligno/cirurgia , Estadiamento de Neoplasias , Receptor ErbB-2/metabolismo
11.
J Stomatol Oral Maxillofac Surg ; 119(3): 232-235, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29477759

RESUMO

INTRODUCTION: Malignant chondroid syringomas, also known as cutaneous malignant mixed tumors, are rare neoplasms that most frequently occur on the torso or extremities of women. Here, we present an illustrated case of a facial malignant chondroid syringoma. MATERIALS AND METHODS: A 32-year-old female patient with no notable medical history presented with an approximately 1cm-wide, painless, palpably-mobile subcutaneous nodule, suggestive of a sebaceous cyst, just above the middle third of the right eyebrow. The nodule had grown steadily over six months. She had no palpable cervical lymphadenopathies. RESULTS: Anatomic pathology of the enucleated nodule found an adnexal sudoriparous tumor measuring 6×10mm and indicative of a malignant chondroid syringoma. Cervicofacial computed tomography and positron emission tomography scans showed no near or distant lymph node involvement. A second intervention for wide excision around the original enucleation lesion (+1cm) was validated in a multidisciplinary, cancerology-dermatology consultation. The eyebrow was reconstructed with a temporally-harvested fasciocutaneous island flap. DISCUSSION: Malignant chondroid syringomas are very rare and thus no standardized treatment has been established for them. Only 12 craniofacial localizations have been described to date. Radiation therapy and chemotherapy have not been shown effective for this malignancy, leaving only wide excision as a therapeutic option. A high and sustained (as much as 20 years after the initial diagnosis) risk of recurrence or metastasis necessitates prolonged patient follow-up.


Assuntos
Adenoma Pleomorfo , Tumor Misto Maligno , Neoplasias das Glândulas Sudoríparas , Adulto , Sobrancelhas , Feminino , Humanos , Recidiva Local de Neoplasia
13.
Aktuelle Urol ; 49(5): 429-432, 2018 Sep.
Artigo em Alemão | MEDLINE | ID: mdl-28905347

RESUMO

Mixed epithelial and stromal tumours of the kidney are rare. Histologically, they are characterised by a complex of epithelium and stroma with cystic and solid areas. They usually occur in perimenopausal women receiving hormone replacement with oestrogen. Typical symptoms are haematuria, flank pain and a palpable mass, with more and more authors reporting incidentally diagnosed tumours. This also applies to our case report. We are reporting the first published case in Germany: a 60-year-old female with a mixed epithelial and stromal tumour of the right kidney. The tumour was removed through complete nephrectomy.


Assuntos
Neoplasias Renais/cirurgia , Tumor Misto Maligno/cirurgia , Neoplasias Primárias Múltiplas/cirurgia , Feminino , Seguimentos , Humanos , Rim/patologia , Neoplasias Renais/diagnóstico , Neoplasias Renais/patologia , Laparoscopia , Imagem por Ressonância Magnética , Pessoa de Meia-Idade , Tumor Misto Maligno/diagnóstico , Tumor Misto Maligno/patologia , Neoplasias Primárias Múltiplas/diagnóstico , Neoplasias Primárias Múltiplas/patologia , Nefrectomia
15.
J Vet Med Sci ; 80(1): 112-115, 2018 Jan 27.
Artigo em Inglês | MEDLINE | ID: mdl-29199245

RESUMO

Renal mixed tumor characterized by the absence of nephrogenic blastema and the presence of predominant osteoid-producing osteoblast-like cells occurred in the kidney of a 6-month-old, hybrid, female pig. At the post-mortem examination, the tumor was found as a calcified grayish-white mass at the cranial end of the left kidney. Histologically the tumor consisted of 3 growth areas of poorly differentiated spindle cells, osteoid-producing osteoblast-like cells, and luminal epithelial cells. Transition from the spindle cells to the osteoblast-like cells or the luminal epithelial cells was observed. Immunohistochemically, the spindle cells and the osteoblast-like cells were consistently positive for ß-catenin. Although the luminal epithelial cells and adjacent spindle cells were positive for cytokeratin, these 3 types of tumor cells were consistently negative for WT1. The tumor was diagnosed as primary renal mixed tumor characterized by marked proliferation of osteoblast-like cells with osteoid formation.


Assuntos
Neoplasias Renais/veterinária , Tumor Misto Maligno/veterinária , Osteoblastos/patologia , Doenças dos Suínos/patologia , Animais , Proliferação de Células , Células Epiteliais/patologia , Feminino , Imuno-Histoquímica , Queratinas/análise , Neoplasias Renais/patologia , Tumor Misto Maligno/patologia , Suínos , beta Catenina/análise
16.
JBJS Case Connect ; 7(4): e90, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-29286973

RESUMO

CASE: A 49-year-old man presented with a rapidly growing thigh mass. Histologic analyses demonstrated separate regions that were consistent with a collision tumor composed of a primary leiomyosarcoma and a metastatic medullary thyroid carcinoma. After responding to chemotherapy, the patient underwent resection of the tumor and a total thyroidectomy; he was disease-free 9 years after the diagnosis. CONCLUSION: A wide diagnostic differential and thorough histologic analysis are necessary in patients presenting with neoplasms of the extremities. A leiomyosarcoma may be a hospitable location for metastatic disease, and the presence of a collision tumor should be considered when pathology findings are equivocal.


Assuntos
Carcinoma Neuroendócrino/patologia , Leiomiossarcoma/patologia , Tumor Misto Maligno/secundário , Neoplasias da Glândula Tireoide/patologia , Carcinoma Neuroendócrino/cirurgia , Humanos , Leiomiossarcoma/cirurgia , Extremidade Inferior/patologia , Masculino , Pessoa de Meia-Idade , Tumor Misto Maligno/cirurgia , Coxa da Perna/patologia , Neoplasias da Glândula Tireoide/cirurgia , Tireoidectomia/métodos
17.
Rom J Morphol Embryol ; 58(3): 977-982, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-29250677

RESUMO

Malignant cutaneous mixed tumor (CMT) is a very rare adnexal tumor with biphasic differentiation. In rare cases, a benign CMT (chondroid syringoma) undergoes malignant transformation. Sebaceous differentiation in a cutaneous malignant mixed tumor has not been previously reported. We present a malignant CMT with sebaceous differentiation, which occurred on the scalp of an 81-year-old man. The tumor showed epithelial elements composed of relatively small and bland-appearing ductal and cord-like structures lined by small, cuboidal-shaped adnexal cells, with a few large, dilated gland-like spaces lined by larger, apocrine-appearing cells with abundant eosinophilic-staining cytoplasm. However, the majority of the epithelial component was composed of nests and islands of markedly enlarged and atypical cells with pale÷clear to ground-glass cytoplasm. Focally, there was sebaceous differentiation identified, in the form of prominent multivacuolated cytoplasm, with nuclear indentations. The stroma showed a mixture of myxoid and hyalinized÷chondroid-appearing areas with focal calcifications. There was strong and diffuse staining of the sebaceous cells by cytokeratin (CK) 7, epithelial membrane antigen (EMA), and androgen receptor (AR). Mismatch repair proteins were investigated by immunohistochemistry, without evidence of loss of expression of MutS protein homolog 6 (MSH6), MutS protein homolog 2 (MSH2), MutL protein homolog 1 (MLH1), or postmeiotic segregation increased 2 (PMS2) in the sebaceous cells.


Assuntos
Tumor Misto Maligno/patologia , Neoplasias Cutâneas/patologia , Neoplasias das Glândulas Sudoríparas/patologia , Idoso de 80 Anos ou mais , Diferenciação Celular , Humanos , Masculino
18.
Bull Cancer ; 104(12): 1001-1012, 2017 Dec.
Artigo em Francês | MEDLINE | ID: mdl-29031505

RESUMO

Endometrial cancer is the fourth cause of cancer in women in France and is the second most common cancer of the gynecologic cancer after breast cancer with 7275 new cases in 2012. The incidence of this neoplasm tends to increase with population aging, diabetes and obesity's augmentation. In rare cases, a hereditary factor has been described: Lynch's syndrome. The therapeutic management of the patient depends on the endometrial biopsy which specifies the histological type and the histo-prognostic grade as well as the MRI which allow the tumor staging. Within the last decade, improvement in technologies such as genomic, transcriptomic and histological analyses, allowed the establishment of new and finer classifications of endometrial carcinomas. The latest classification proposed by The Cancer Genomic Atlas (TCGA), has been made routinely applicable through the international consortium TransPORTEC. It consists of 4 groups listed from good to poor prognosis: (1) ultra-mutated "POLE"; (2) hyper-mutated "MSI"; (3) low copy number "NSMP" and (4) high number of copies "TP53 mutated" (serous-like). This integrated characterization combined with mutational data opens new opportunities for therapeutic strategies.


Assuntos
Carcinoma Endometrioide , Neoplasias do Endométrio , Tumor Misto Maligno , Biópsia , Carcinoma Endometrioide/classificação , Carcinoma Endometrioide/genética , Carcinoma Endometrioide/patologia , Carcinoma Endometrioide/terapia , Neoplasias do Endométrio/classificação , Neoplasias do Endométrio/genética , Neoplasias do Endométrio/patologia , Neoplasias do Endométrio/terapia , Endométrio/patologia , Feminino , Humanos , Imunoterapia/métodos , Tumor Misto Maligno/classificação , Tumor Misto Maligno/genética , Tumor Misto Maligno/patologia , Tumor Misto Maligno/terapia , Terapia de Alvo Molecular , Prognóstico , Fatores de Risco
19.
Int J Surg Pathol ; 25(7): 585-591, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28552015

RESUMO

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.


Assuntos
Adenocarcinoma/patologia , Ampola Hepatopancreática/patologia , Carcinoma Neuroendócrino/patologia , Neoplasias do Ducto Colédoco/patologia , Tumor Misto Maligno/patologia , Doenças Raras/patologia , Adenocarcinoma/diagnóstico por imagem , Adenocarcinoma/mortalidade , Adenocarcinoma/terapia , Adulto , Ampola Hepatopancreática/diagnóstico por imagem , Ampola Hepatopancreática/cirurgia , Biomarcadores Tumorais/sangue , Carcinoma Neuroendócrino/diagnóstico por imagem , Carcinoma Neuroendócrino/mortalidade , Carcinoma Neuroendócrino/terapia , Quimioterapia Adjuvante , Neoplasias do Ducto Colédoco/diagnóstico por imagem , Neoplasias do Ducto Colédoco/mortalidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Tumor Misto Maligno/diagnóstico por imagem , Tumor Misto Maligno/mortalidade , Tumor Misto Maligno/terapia , Gradação de Tumores , Recidiva Local de Neoplasia/diagnóstico por imagem , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/terapia , Pancreaticoduodenectomia , Prognóstico , Doenças Raras/diagnóstico por imagem , Doenças Raras/mortalidade , Doenças Raras/terapia , Estudos Retrospectivos , Análise de Sobrevida , Tomografia Computadorizada por Raios X
20.
J Vet Med Sci ; 79(3): 670-673, 2017 Mar 28.
Artigo em Inglês | MEDLINE | ID: mdl-28132963

RESUMO

The atypical cutaneous tumor of a 9-year-old mixed breed female dog was examined. The tumor was well-demarcated and histologically composed of a trichoblastic area, tricholemmal area and apocrine glandular area. Neoplastic cells in trichoblastic area and tricholemmal area had PAS-positive granules in the cytoplasm and were positive for pan-cytokeratin, cytokeratin 5/6, 14 and 19 and p63. Neoplastic cells in trichoblastic area were also positive for cytokeratin 15 and CD34. Neoplastic cells in apocrine glandular area were positive for pan-cytokeratin and cytokeratin 7, 18 and 19. Myoepithelial cell proliferation with osteocartilaginous metaplasia was observed in this area. Since neoplastic cells showed multiphenotypic differentiation for hair follicles and apocrine glands, the present case was diagnosed as a cutaneous mixed tumor.


Assuntos
Doenças do Cão/patologia , Tumor Misto Maligno/veterinária , Neoplasias Cutâneas/veterinária , Animais , Cães , Feminino , Tumor Misto Maligno/patologia , Neoplasias Cutâneas/patologia
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