RESUMO
BACKGROUND: Primary endometrial squamous cell carcinoma (PSECC) is a rare neoplasm. Squamous epithelium derived from endometrioid cancer or from cervical squamous cell carcinoma. The prevalence is about 0.1%. The genesis, histogenesis and biological behavior are unknown. CASE PRESENTATION: A 48-year old woman in postmenopause, referring pelvic pain and vaginal bleeding. Transvaginal ultrasound showed a bulky uterus with the endometrium containing an hyperecogenic area. Endometrial biopsy found an epidermoid carcinoma. MRI showed a 4 x 2 x 1.2 cm mass occupying the uterine cavity. The patient underwent radical treatment. Pathological examination showed features of PSECC. The mutation of p53 tumor suppressor protein was disclosed in 15% of neoplastic cells. PCR revealed the absence of HPV DNA. CONCLUSIONS: The findings of our case move us to underline that the pathogenesis of this tumor is still unclear. Moreover, preoperative diagnosis and staging of PESCC is extremely difficult. Most patients do not show characteristic symptoms and predisposing factors, making it almost impossible to diagnose the precise localization of tumor origin.
Assuntos
Carcinoma de Células Escamosas/patologia , Neoplasias do Endométrio/patologia , Carcinoma de Células Escamosas/genética , Carcinoma de Células Escamosas/cirurgia , Neoplasias do Endométrio/genética , Neoplasias do Endométrio/cirurgia , Feminino , Humanos , Pessoa de Meia-Idade , Pós-Menopausa , Resultado do Tratamento , Proteína Supressora de Tumor p53/genéticaRESUMO
The Silent Corticotroph Adenoma (SCA) is a pituitary adenoma variant characterized by the immunoreactivity for adrenocorticotropic hormone (ACTH) and related peptides, without the clinical signs of Cushing's disease. SCA has been postulated to either secrete structurally abnormal ACTH that is inactive but detectable by immunohistochemistry or radioimmunoassay, or to secrete ACTH intermittently or at low levels continuously. Excess of ACTH has been associated to type II muscle atrophy. We describe a case of type II muscle fibers atrophy associated with silent corticotroph adenoma in a dog. The dog showed moderate to severe proximal muscle wasting and weakness with normal levels of muscle-associated enzymes. In the limb muscle biopsies, type II fibers were uniformly smaller than type I fibers. In temporalis muscles, there were few atrophic fibers, and several irregular areas of loss of enzymatic activity observed in NADH, SDH and COX stains. The tumour showed a trabecular growth pattern and immunohistochemical analysis demonstrated the presence of cytoplasmic immunoreactivity for ACTH. The muscle atrophy was considered to be related to an excess of inactive ACTH. Studying spontaneous occurring rare diseases in animals could help to understand the mechanism of similar diseases in human has well.
Assuntos
Adenoma Hipofisário Secretor de ACT/veterinária , Doenças do Cão/patologia , Fibras Musculares de Contração Rápida/patologia , Atrofia Muscular/veterinária , Neoplasias Hipofisárias/veterinária , Adenoma Hipofisário Secretor de ACT/diagnóstico , Adenoma Hipofisário Secretor de ACT/patologia , Hormônio Adrenocorticotrópico , Animais , Doenças do Cão/enzimologia , Doenças do Cão/metabolismo , Cães , Imuno-Histoquímica/veterinária , Masculino , Atrofia Muscular/patologia , NADH Tetrazólio Redutase/metabolismo , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/patologia , Prostaglandina-Endoperóxido Sintases/metabolismo , Radioimunoensaio/veterinária , Coloração e Rotulagem/métodos , Coloração e Rotulagem/veterinária , Succinato Desidrogenase/metabolismoRESUMO
BACKGROUND: Mucinous ovarian cancer raises problems of differential diagnoses because it is often difficult to distinguish the primary from the metastatic form. Most metastatic ovarian tumors originate from the gastrointestinal tract, mainly colorectal, gastric, pancreatic; the gallbladder is a very rare source of ovarian metastases. CASE: We report a case of ovarian metastases from a gallbladder cancer, incidentally diagnosed more than 2.5 years earlier during a laparoscopic intervention for biliary lithiasis. CONCLUSION: The interest of this case lies in the long progression-free survival, the venous thromboembolism syndrome that preceded by a few months the diagnosis of the ovarian mass and the discrepancy between the radiologic and the laparoscopic stage assessment.
Assuntos
Adenocarcinoma/secundário , Neoplasias da Vesícula Biliar/patologia , Neoplasias Ovarianas/secundário , Adenocarcinoma/tratamento farmacológico , Adenocarcinoma/cirurgia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Desoxicitidina/administração & dosagem , Desoxicitidina/análogos & derivados , Intervalo Livre de Doença , Feminino , Neoplasias da Vesícula Biliar/tratamento farmacológico , Neoplasias da Vesícula Biliar/cirurgia , Humanos , Achados Incidentais , Excisão de Linfonodo , Pessoa de Meia-Idade , Compostos Organoplatínicos/administração & dosagem , Neoplasias Ovarianas/tratamento farmacológico , Neoplasias Ovarianas/cirurgia , Ovariectomia , Oxaliplatina , Resultado do Tratamento , GencitabinaRESUMO
This study reports a series of 19 extrapleural solitary fibrous tumors. The patients included 6 men and 13 women with age ranging from 27 to 86 years. Three patients showed local recurrence. In 2 tumors, a diagnosis of malignancy was made. All of the tumors were strongly positive for CD34, and 3 of them expressed high levels of progesterone receptor. Solitary fibrous tumors are fairly rare, occurring in many parts of the body, and their behavior is unpredictable.
Assuntos
Tumores Fibrosos Solitários/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Antígenos CD34/biossíntese , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Receptores de Progesterona/biossíntese , Tumores Fibrosos Solitários/metabolismoRESUMO
The authors report a case of soft tissue chondroma of the masseter muscle in a 49-year-old man. The tumour was entirely composed of lobules of hyaline cartilage. The literature on head and neck soft tissue chondroma is also reviewed. To the authors' knowledge, this is the first case of muscular soft tissue chondroma in the head and neck region.
Assuntos
Condroma/patologia , Músculo Masseter/patologia , Neoplasias Musculares/patologia , Biópsia por Agulha Fina , Condrócitos/patologia , Colágeno/análise , Diagnóstico Diferencial , Humanos , Cartilagem Hialina/patologia , Masculino , Pessoa de Meia-Idade , Proteínas S100/análise , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Primary retroperitoneal mucinous cystadenoma is a rare tumor only 48 cases have been reported in international literature. Patients affected by primary retroperitoneal mucinous cystadenoma/cystadenocarcinoma ranged in age from 17 to 86 years (median, 42.3 years) and the size of the cystis ranged from 5 to 35 cm (median, 16.1 cm). There is no unanimous opinion on the genesis of these tumors and, due to their extreme rarity, its histogenesis, biological behavior and the optimal management strategy remain at a speculative level. CASE REPORT: We report the case of a huge borderline primary retroperitoneal mucinous cystadenoma (24 x 25 cm) in a 35-year-old woman and the strategies adopted for the diagnosis and surgical management. CONCLUSION: Primary mucinous cystic tumor of the retroperitoneum was correctly diagnosed only at the time of surgery. As well as in the majority of cases reported in the literature, preoperative investigations were not able to give information about the tumor site. In spite of the short follow-up (two years), the patient's favorable course supports the hypothesis that primary retroperitoneal mucinous cystadenoma may be treated in the same manner as a primary ovarian tumor of the same grade and comparable stage.
Assuntos
Cistadenoma Mucinoso/diagnóstico , Neoplasias Ovarianas/diagnóstico , Neoplasias Retroperitoneais/diagnóstico , Adulto , Cistadenoma Mucinoso/patologia , Cistadenoma Mucinoso/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Neoplasias Ovarianas/patologia , Neoplasias Ovarianas/cirurgia , Neoplasias Retroperitoneais/patologia , Neoplasias Retroperitoneais/cirurgiaRESUMO
Haemangiomas are benign vascular neoplasms characterized by an abnormal proliferation of blood vessels. They may occur in any vascularized tissue including skin, subcutaneous tissue muscle and bone. These tumours are common in infancy and childhood and commonly involve subcutaneous or mucosal tissues. Intramuscular haemangiomas, a distinctive type of haemangioma occurring within skeletal muscle, account for less than 1% of all haemangiomas. They occur more often in trunk and extremity muscles, whereas involvement of the temporal muscle is extremely rare. Herein, the case is reported of a 38-year-old male who presented with a round, painless mass in the left temporal fossa, which was interpreted as an intramuscular haemangioma after a magnetic resonance imaging scan. In this report, clinico-pathological findings are described in an additional case of haemangioma involving the temporal muscle, and a review is made of the international literature on this subject.
Assuntos
Hemangioma Cavernoso/patologia , Hemangioma/patologia , Neoplasias Musculares/patologia , Músculo Temporal/patologia , Adulto , Hemangioma/complicações , Hemangioma Cavernoso/complicações , Humanos , Imageamento por Ressonância Magnética , Masculino , Neoplasias Musculares/complicaçõesRESUMO
BACKGROUND: The uterine cervix represents an exceptional localization of signet-ring cell adenocarcinoma (SRCA). Most commonly, endocervical tumors with such morphology are metastatic from the breast or gastrointestinal tract while primary pure or almost pure tumors are extremely rare. No previous case of primary pure endocervical SRCA with follow-up longer than three years has been found in the literature. CASE REPORT: The present report describes such a case of a 46-year-old woman without evidence of recurrence eight years after the diagnosis. The patient was referred to the Gynecology Department for persistent abnormal vaginal bleeding of three months duration. Specular examination and colposcopy revealed a cervical polypoid lesion occupying the posterior lip of the cervix and protruding from the external uterine orifice. A biopsy of the lesion was interpreted by the pathologist as SRCA. An extensive search for an extrapelvic primary cancer was undertaken but revealed no evidence of malignancy. The patient underwent radical hysterectomy with bilateral salpingo-oophorectomy and pelvic and paraaortic lymph node sampling for FIGO Stage 1B1 cervical cancer without any adjuvant chemo- or radiotherapy. The histological diagnosis showed neoplastic signet-ring cells confined in the head of the cervical polyps with minimal stromal infiltration. After surgery the patient underwent close followup including periodic cervicovaginal smears, bimanual vaginal examination, complete laboratory tests, ultrasound and abdominopelvic computed tomography. CONCLUSIONS: The prognostic relevance of primitive pure SRCA in the uterine cervix is unclear because of the relatively small number of cases. However the two early deaths out of six reported cases and the absence of follow-up longer than three years for the other affected patients, seem to suggest an aggressive behavior. The present case represents an exceptional long-term survival, probably related to an early diagnosis and a prominent polypoid growth of the neoplasia outside the uterus.
Assuntos
Carcinoma de Células em Anel de Sinete/patologia , Neoplasias do Colo do Útero/patologia , Carcinoma de Células em Anel de Sinete/cirurgia , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Histerectomia , Pessoa de Meia-Idade , Neoplasias do Colo do Útero/terapiaRESUMO
Imatinib, a tyrosine kinase inhibitor directed against the enzymatic domain of KIT protein, was found to produce dramatic clinical responses in metastatic gastrointestinal stromal tumors (GISTs). However, resistance usually develops thus determining treatment failure. The present study was performed to analyse the expression of somatostatin receptor (SSTR) subtypes, modulators of tissue transglutaminase, in a series of GISTs and leiomyosarcomas by immunohistochemistry to identify a new potential therapeutic target. Sixteen cases (8 males and 8 females, age range: 38-73; 11 GISTs, 4 leiomyosarcomas, 1 leiomyoma) were studied. Immunohistochemical detection of the relevant SSTRs was performed on paraffin-embedded tissue sections, stained with polyclonal antibodies directed against the five somatostatin receptor subtypes. We found 7 out of 16 (44%) tumors expressing all SSTRs and 14 out of 16 (87%) tumors positive for at least 3 subtypes. SSTR2A was the most represented subtype in the tumors studied, being expressed in approximately 70% of cases exhibiting an intense labeling in most of these cases. The significant expression of SSTRs shown in this series of GISTs and gastrointestinal leiomyosarcomas suggests a potential therapeutic target to be explored alone and/or in combination with other therapeutic agents in the setting of refractory GI stromal tumors.
Assuntos
Neoplasias Gastrointestinais/tratamento farmacológico , Neoplasias Gastrointestinais/enzimologia , Leiomioma/tratamento farmacológico , Leiomioma/enzimologia , Leiomiossarcoma/tratamento farmacológico , Leiomiossarcoma/enzimologia , Somatostatina/uso terapêutico , Transglutaminases/biossíntese , Adulto , Idoso , Benzamidas , Resistencia a Medicamentos Antineoplásicos/efeitos dos fármacos , Ativação Enzimática/efeitos dos fármacos , Indução Enzimática/efeitos dos fármacos , Feminino , Neoplasias Gastrointestinais/patologia , Regulação Neoplásica da Expressão Gênica/efeitos dos fármacos , Humanos , Mesilato de Imatinib , Leiomioma/patologia , Leiomiossarcoma/patologia , Masculino , Mesoderma/enzimologia , Mesoderma/patologia , Pessoa de Meia-Idade , Piperazinas/uso terapêutico , Inibidores de Proteínas Quinases/metabolismo , Proteínas Proto-Oncogênicas c-kit/metabolismo , Pirimidinas/uso terapêutico , Receptores de Somatostatina/biossíntese , Somatostatina/análogos & derivadosRESUMO
Chondromyxoid fibroma is a rare benign tumour of chondral origin. It usually involves the long bones of the lower extremity, whilst involvement of craniofacial skeleton is extremely unusual. The second case of chondromyxoid fibroma of the zygoma described in literature is presented and the surgical resection of the lesion with tumour-free margins as the key factor for avoiding local recurrence of this tumour is emphasised.
Assuntos
Condroblastoma/diagnóstico , Neoplasias Cranianas/diagnóstico , Zigoma/patologia , Calcinose/patologia , Seguimentos , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Benign myoepithelioma is a very rare form of salivary gland tumor, composed entirely of myoepithelial cells. It accounts for approximately 1% of all salivary gland tumors and is most frequently located in the parotid gland and in the minor salivary glands of the hard palate. We describe herein the ninth reported case of myoepithelioma of the submandibular gland. Benign myoepithelioma must be differentiated from several benign and malignant epithelial and mesenchymal tumors. Immunohistochemical staining can help differentiate between these conditions, but histopathology remains the gold standard for diagnosing this neoplastic process.
Assuntos
Mioepitelioma/patologia , Neoplasias da Glândula Submandibular/patologia , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-IdadeAssuntos
Anticorpos Monoclonais/uso terapêutico , Doença de Crohn/tratamento farmacológico , Dermatite/tratamento farmacológico , Fármacos Dermatológicos/uso terapêutico , Adulto , Doença de Crohn/complicações , Dermatite/etiologia , Feminino , Humanos , Infliximab , Fístula Retal/tratamento farmacológicoRESUMO
Intravascular lymphomatosis (IVL) is a rare large-cell lymphoproliferative disorder characterized by a widespread lymphoma proliferation within the lumen of medium and small vessels, frequently presenting with skin and/or central nervous system (CNS) manifestations. The tumor is of B-cell origin in most cases. Prognosis is poor with a reported median survival of 5-7 months. We describe here two cases of IVL. The first was that of a 55-year-old woman with a large B-cell lymphoma of the leg, successfully treated with conventional chemotherapy (CHT) followed by autologous peripheral stem cell transplantation. At 3 months from the autograft she relapsed with a picture of hemophagocytic syndrome (HPS) and CNS symptoms. She died before any specific treatment, and post-mortem examination revealed the intravascular proliferation of lymphoma B-cells in the brain and bone marrow. The second case was that of a 60-year-old male with CNS involvement at diagnosis. He responded poorly to CHOP-like CHT, and died 2 months after diagnosis and 6 months after onset of symptoms. Failure of CHT at least in some IVL patients may be related to a delay in the initiation of therapy due to non-specific neurological symptoms. Therefore, early diagnosis based upon aggressive attempts immediately followed by adequate therapy may prove beneficial to these patients. In the present report, we performed an extended medline-based review of the published series of patients with IVL.
Assuntos
Neoplasias do Sistema Nervoso Central/secundário , Linfoma de Células B/patologia , Neoplasias Vasculares/patologia , Autopsia , Evolução Fatal , Feminino , Humanos , Perna (Membro)/irrigação sanguínea , Linfoma de Células B/diagnóstico , Linfoma de Células B/terapia , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica/patologia , Neoplasias Vasculares/diagnóstico , Neoplasias Vasculares/terapiaRESUMO
In the peritoneal cavity, diffuse serosal replacement by tumor is demonstrated usually by extensive carcinomatous involvement from gastric, colonic, or pancreatic tumors or less frequently by mesothelioma. Primary tumors other than mesothelioma are extremely rare in the peritoneum. The computed tomographic appearances of two cases of rare peritoneal tumors, epithelioid hemangioendothelioma and desmoplastic small round cell tumor, are described.
Assuntos
Fibromatose Agressiva/diagnóstico por imagem , Hemangioendotelioma Epitelioide/diagnóstico por imagem , Neoplasias Peritoneais/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto , Humanos , MasculinoRESUMO
AIMS: Monotypic epithelioid angiomyolipoma is a distinct and definable variant of angiomyolipoma, composed of monomorphous epithelioid cells that show HMB45 immunoreactivity. Angiomyolipoma, including its morphological variants, belongs to the family of perivascular epithelioid cell tumour. METHODS AND RESULTS: The tumour was examined using immunohistochemical staining and by transmission electron microscopy. Neoplastic cells showed a cytoplasmic granular positivity for HMB45. CONCLUSIONS: Extrarenal angiomyolipomas are rare and, to the best of our knowledge, this is the first reported case of a primary monotypic epithelioid angiomyolipoma of bone in a patient without evidence of tuberous sclerosis.
Assuntos
Angiomiolipoma/patologia , Neoplasias Ósseas/patologia , Células Epitelioides/patologia , Adulto , Angiomiolipoma/metabolismo , Antígenos de Neoplasias , Neoplasias Ósseas/metabolismo , Células Epitelioides/química , Humanos , Imuno-Histoquímica , Masculino , Antígenos Específicos de Melanoma , Proteínas de Neoplasias/análiseRESUMO
A case of Kaposi sarcoma with HIV-negative and sequence of HHV-8 positive and exclusive rectosigmoid and descending colon involvement without immunodeficiency is reported. Histologically, in addition to typical features of Kaposi sarcoma, Cowdry type A inclusions were seen. PCR analysis of the tumor showed positivity for human herpesvirus 8. Two of the six reported cases of Kaposi sarcoma limited to the bowel were from African men before the AIDS epidemic. J. Surg. Oncol. 2001;76:197-200.
Assuntos
Neoplasias do Colo/diagnóstico , Soronegatividade para HIV , Sarcoma de Kaposi/diagnóstico , Idoso , Neoplasias do Colo/virologia , DNA Viral/análise , Feminino , Herpesvirus Humano 8/genética , Herpesvirus Humano 8/isolamento & purificação , Humanos , Reação em Cadeia da Polimerase , Sarcoma de Kaposi/virologiaRESUMO
Sarcomatoid carcinoma (SC) is a well defined tumor type that may occur in all organs and anatomic sites but most commonly in the head, neck, respiratory tract, breast, and genitourinary tract. It is a biphasic tumor showing both epithelial- and mesenchymal-like differentiation; however, its carcinomatous nature is widely recognized. SC is rare in the gastrointestinal tract. In the esophagus it accounts for less than 5% of all malignancies and approximately only 35 cases have been described in the stomach. Very few cases have been observed in the small intestine, anorectal junction, liver, and pancreas. To our knowledge only eight cases of SC have been reported in the colon. We report a case of primary colonic SC. Both morphological and immunohistochemical analyses are provided along with an evaluation of the unusual clinical history, therapeutic implications, and controversial differential diagnosis.
Assuntos
Carcinossarcoma/patologia , Neoplasias do Colo/patologia , Feminino , Humanos , Imuno-Histoquímica , Pessoa de Meia-IdadeRESUMO
The cemento-ossifying fibroma is probably the most frequent fibro-osseous lesion seen by oral pathologists. Occasionally, reports of lesions behaving in an aggressive fashion appear in the literature. At the present time, the relationship if these "aggressive" lesions to the "usual" fibromas is unclear. Two cases of "aggressive" cemento-ossifying fibroma are reported. Certainly, clinical, radiological, and histological characteristics of aggressive and usual fibromas do coincide. There are, however, diverging features warranting separation of different forms. These mild differences may be detected during preoperative assessment of the lesion, allowing a diagnosis of aggressive form to be made.
Assuntos
Fibroma Ossificante/patologia , Neoplasias Maxilomandibulares/patologia , Adulto , Feminino , Fibroma Ossificante/cirurgia , Seguimentos , Humanos , Neoplasias Maxilomandibulares/cirurgia , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Intraabdominal desmoplastic small round cell tumor (DSRCT) is a recently recognized type of primitive sarcoma characterized by a predilection for young males, a usually very aggressive course and generally unsuccessful therapy. A primitive histologic appearance with prominent desmoplasia and striking divergent multilineage differentiation are well-described morphologic features of this tumor, along with a consistent fusion of the EWS and WT1 genes at the molecular level. The cytologic literature contains only scattered references to this type of neoplasm. Detailed information on the clinical and fine needle aspiration (FNA) biopsy and the immunocytochemical and ultrastructural findings in a patient with DSRCT is presented. CASE REPORT: A 23-year-old male had a firm abdominal mass with multiple secondary lesions of the liver. An FNA biopsy was performed under ultrasonographic guidance. CONCLUSION: FNA of the liver nodules showed cohesive groups of small cells with hyperchromatic nuclei and inconspicuous nucleoli; immunocytochemically vimentin and desmin showed characteristic perinuclear globular positivity. FNA cytology is an effective means of diagnosing deeply located lesions. The cytologic features of DSRCT need to become familiar to pathologists and must be considered in the differential diagnosis of liver metastasis.