Acute sensorimotor paraneoplastic neuropathy in a patient with small cell prostate cancer.

The authors describe a patient with a background of metastatic small cell prostate cancer who presented with a rapidly evolving sensorimotor neuropathy with bulbar features closely resembling Guillain-Barré syndrome, with a good initial response to intravenous immunoglobulins and platinum-based chemotherapy. This represented a likely paraneoplastic manifestation of the patient's urological malignancy.

Glaucoma neovascular causado por metástasis de carcinoma microcítico de pulmón. Presentación de un caso / Neovascular glaucoma caused by small-cell lung cancer iris metastasis: A case presentation

El carcinoma microcítico de pulmón puede afectar al ojo directamente mediante proliferación metastásica o indirectamente a través de síndromes paraneoplásicos. La coroides es el lugar más afectado entre las metástasis uveales; no obstante, el iris puede estar envuelto en una proporción pequeña de casos (<10%). Visión borrosa, dolor, hiperemia, fotofobia, glaucoma, hipema o defectos en el campo visual pueden originarse por la afectación metastásica. La mediana de supervivencia en pacientes con metástasis iridianas es de 4 meses. El glaucoma secundario que se origina puede ser manejado con tratamiento tópico y oral, ciclo fotoablación transescleral, trabeculoplastia con láser, anti-VEGF, MIGS, cirugía filtrante o enucleación. Se presenta un caso único que desarrolló un cáncer microcítico de pulmón primario con metástasis iridianas. Las metástasis provocaron un cierre angular, refractario al tratamiento tópico. Se realizó radioterapia local para conseguir una respuesta local (AU)

Small-cell lung cancer may directly affect the eye by metastatic proliferation or indirectly by paraneoplastic syndromes. The choroid is the most common site for uveal metastasis (90%); however, the iris can be involved in a smaller proportion of cases (incidence <10%). Blurred vision, pain, redness, photophobia, glaucoma, hyphema and visual field defects can arise from this metastatic involvement. The median survival time for patients with iris metastasis is reported to be 4 months. Secondary glaucoma can be managed with topical and oral treatment, transscleral cyclophotocoagulation, laser trabeculoplasty, anti-VEGF, minimally invasive glaucoma surgery (MIGS), filtering surgery, shunting surgery or enucleation. A case of primary small-cell lung cancer with iris metastasis is presented. The metastases produced an angle-closure glaucoma, which was refractory to topical treatment. Local radiotherapy was administered, obtaining a good local response (AU)

Metastatic Small Cell Neuroendocrine Carcinoma of Endometrium: Rare Entity, Rare Presentation.

ABSTRACT: Primary small cell neuroendocrine carcinoma of endometrium is very rare and aggressive carcinoma. Most patients present with metastases at the time of diagnosis and have very poor prognosis. Only very few cases are reported in literature. Here we present a case of 67-year-old woman, who on evaluation for mild pain abdomen was subsequently diagnosed to have metastatic small cell neuroendocrine carcinoma of endometrium on PET/CT scan and biopsy.

Foreign accent syndrome as a heralding manifestation of transformation to small cell neuroendocrine prostate cancer.

A man in his 50s with metastatic hormone-sensitive prostate cancer, receiving androgen deprivation therapy and abiraterone acetate/prednisone, presented with an uncontrollable 'Irish brogue' accent despite no Irish background, consistent with foreign accent syndrome (FAS). He had no neurological examination abnormalities, psychiatric history or MRI of the brain abnormalities at symptom onset. Imaging revealed progression of his prostate cancer, despite undetectable prostate-specific antigen levels. Biopsy confirmed transformation to small cell neuroendocrine prostate cancer (NEPC). Despite chemotherapy, his NEPC progressed resulting in multifocal brain metastases and a likely paraneoplastic ascending paralysis leading to his death. We report FAS as the presenting manifestation of transformation to small cell NEPC, a previously undescribed phenomenon. His presentation was most consistent with an underlying paraneoplastic neurological disorder (PND), despite a negative serum paraneoplastic panel. This report enhances the minimal existing literature on FAS and PNDs associated with transformed NEPC.

Complete resolution of paraneoplastic syndrome of inappropriate antidiuretic hormone secretion following thymic small-cell carcinoma thoracoscopic resection.

Thymic neuroendocrine tumours are rare anterior mediastinal neoplasms often associated with paraneoplastic syndromes. A patient presented with intractable hyponatraemia and a DOTATATE-avid mediastinal mass. Following medical optimization, she underwent thoracoscopic thymectomy with en bloc thymic small-cell carcinoma resection. Her symptoms resolved and her sodium levels normalized. In localized disease, curative-intent, minimally invasive thymic neuroendocrine tumour resection is safe and effective following preoperative staging and paraneoplastic syndrome management.

Subacute sensory neuronopathy associated with Merkel cell carcinoma with unknown primary: a case report with literature review.

Paraneoplastic neurologic syndromes (PNSs) are a wide spectrum of neurologic diseases characterized by different clinical features, associated with a neoplasia, and triggered by an immune-mediated process. In most cases, it is possible to detect specific neuronal antibodies and the Hu protein is one of the most frequently recognized intracellular antigens in patients with PNSs. Small-cell lung cancer is the most common cancer associated with PNSs, followed by urological, gynecological and hematological malignancies. Otherwise, extra-pulmonary small-cell carcinomas, including Merkel cell carcinoma (MCC), have been rarely described as related to PNSs. In this article we report, for the first time in the published literature, a case of anti-Hu antibody-related subacute sensory neuronopathy in association with MCC.

BK virus associated with small cell carcinoma of bladder in a patient with renal transplant.

A man in his 70s with a complex medical history, including cadaveric renal transplant, presented with recurrent urinary tract infections. Investigation revealed recurrent urinary pathogens, including Enterobacter cloacae and persistent BK viruria. Cystoscopy revealed a pedunculated mass in the right posterior-lateral wall, inferior to the transplant urethral orifice, and biopsy of this mass showed invasive small cell carcinoma with a prominent adenocarcinoma component. The tumour was treated with complete transurethral resection followed by carboplatin, etoposide and radiation. Laboratory analysis of biopsied samples showed immunostaining and molecular evidence of BK virus DNA in the cancer cells. Follow-up cystoscopies have shown no recurrence of the cancer.

Endocrine paraneoplastic syndromes in lung cancer: a respiratory physician's perspective.

Lung malignancy is known to be one of the leading causes of cancer-related mortality. Endocrine paraneoplastic syndromes in lung cancer are common. These are due to secretion of various substances and not because of direct tumour invasion or me-tastasis. These syndromes have also been associated with lung cancer prognosis. This review describes the many endocrine paraneoplastic syndromes seen in lung cancer and narrates their incidence, biology, clinical features, diagnosis, and management.

Síndrome miasteniforme de Lambert-Eaton / Lambert-Eaton myasthenic syndrome

Introducción: El síndrome miasteniforme de Lambert-Eaton (LEMS) es una patología paraneoplásica (T-LEMS) o idiopática autoinmunitaria (NT-LEMS) ocasionada por autoanticuerpos contra los canales de calcio dependientes del voltaje presinápticos de la unión neuromuscular. El 60% de los T-LEMS se asocia a carcinoma de pulmón de células pequeñas. Una puntuación Dutch-English LEMS Tumor Association Prediction (DELTA-P) mayor de 3 denota un riesgo elevado de dicha asociación. El diagnóstico precoz fundado en los hallazgos clínicos, estudios neurofisiológicos y dosificación de títulos de anticuerpos en el suero permite iniciar tempranamente el tratamiento sintomático y la búsqueda oncológica. Son escasos los informes de pacientes con LEMS en Latinoamérica. Objetivo: Describir las características de pacientes con LEMS de un centro privado de Buenos Aires, Argentina, y compararlas con las de otras series publicadas. Pacientes y métodos: Se revisaron historias clínicas de 13 pacientes con LEMS con hallazgos clínicos, electromiograma compatible y/o anticuerpos positivos. Se realizó seguimiento hasta descartar o confirmar una neoplasia asociada de acuerdo con los algoritmos recomendados. Resultados: Cuatro pacientes presentaron diagnóstico de T-LEMS, dos de ellos con carcinoma de pulmón de células pequeñas. De los nueve pacientes con NT-LEMS, cinco presentaron una puntuación DELTA-P de 3 y 4. Nueve pacientes presentaron la tríada clínica clásica desde el inicio. Todos los pacientes presentaron en el electromiograma hallazgos compatibles con defecto de placa neuromuscular presináptico. El 70% mejoró sintomáticamente con piridostigmina. Conclusiones: Los hallazgos clínicos, junto con los estudios neurofisiológicos compatibles, resultan suficientes para el diagnóstico de LEMS. No pudo replicarse la relación entre puntuación DELTA-P y riesgo de carcinoma de pulmón de células pequeñas...(AU)

Introduction: Early diagnosis based on clinical findings, neurophysiological studies and serum antibody titres allows early initiation of symptomatic treatment and oncological screening. Reports of patients with LEMS in Latin America are scarce. Aim: This article aims to describe the characteristics of patients with LEMS from a private centre in Buenos Aires, Argentina, and to compare them with those of other series that have been published. Patients and methods: The medical records of 13 patients with LEMS with clinical findings, compatible electromyogram and/or positive antibodies were reviewed. Follow-up was performed until associated neoplasia was ruled out or confirmed according to the recommended algorithms. Results: Four patients were diagnosed with T-LEMS, two of them with small-cell lung carcinoma. Of the nine patients with NT-LEMS, five had a DELTA-P score of 3 and 4. Nine patients presented with the classic clinical triad from the onset of the disease. All patients had electromyogram findings compatible with presynaptic neuromuscular plaque defect. Of the total, 70% improved symptomatically with pyridostigmine. Conclusions: The clinical findings, together with compatible neurophysiological studies, are sufficient for the diagnosis of LEMS. The relationship between the DELTA-P score and the risk of small-cell lung carcinoma could not be replicated. Symptomatic treatment with pyridostigmine represents an effective therapeutic alternative.(AU)

Hypercalcaemia due to ovarian small cell carcinoma of the hypercalcaemic type.

A 37-year-old woman presented with a few days' history of lower abdominal pain and an incidental finding of hypercalcaemia. A thorough workup ensued, and the cause was found to be an exceptionally rare ovarian tumour-ovarian small cell carcinoma of the hypercalcaemic type. Acute treatment of hypercalcaemia consisted of aggressive intravenous fluids and bisphosphonates. She underwent surgery to remove the tumour and is currently receiving systemic platinum/etoposide chemotherapy combination to be followed by pelvic radiotherapy. This case highlights the wide range of differential diagnosis for hypercalcaemia and the importance of a stepwise and thorough approach during investigations. We discuss the pathophysiology of malignancy-related hypercalcaemia, focusing especially on parathyroid hormone-related peptide-associated hypercalcaemia.

[Lambert-Eaton myasthenic syndrome]. / Síndrome miasteniforme de Lambert-Eaton.

INTRODUCTION: Early diagnosis based on clinical findings, neurophysiological studies and serum antibody titres allows early initiation of symptomatic treatment and oncological screening. Reports of patients with LEMS in Latin America are scarce. AIM: This article aims to describe the characteristics of patients with LEMS from a private centre in Buenos Aires, Argentina, and to compare them with those of other series that have been published. PATIENTS AND METHODS: The medical records of 13 patients with LEMS with clinical findings, compatible electromyogram and/or positive antibodies were reviewed. Follow-up was performed until associated neoplasia was ruled out or confirmed according to the recommended algorithms. RESULTS: Four patients were diagnosed with T-LEMS, two of them with small-cell lung carcinoma. Of the nine patients with NT-LEMS, five had a DELTA-P score of 3 and 4. Nine patients presented with the classic clinical triad from the onset of the disease. All patients had electromyogram findings compatible with presynaptic neuromuscular plaque defect. Of the total, 70% improved symptomatically with pyridostigmine. CONCLUSIONS: The clinical findings, together with compatible neurophysiological studies, are sufficient for the diagnosis of LEMS. The relationship between the DELTA-P score and the risk of small-cell lung carcinoma could not be replicated. Symptomatic treatment with pyridostigmine represents an effective therapeutic alternative.

TITLE: Síndrome miasteniforme de Lambert-Eaton.Introducción. El síndrome miasteniforme de Lambert-Eaton (LEMS) es una patología paraneoplásica (T-LEMS) o idiopática autoinmunitaria (NT-LEMS) ocasionada por autoanticuerpos contra los canales de calcio dependientes del voltaje presinápticos de la unión neuromuscular. El 60% de los T-LEMS se asocia a carcinoma de pulmón de células pequeñas. Una puntuación Dutch-English LEMS Tumor Association Prediction (DELTA-P) mayor de 3 denota un riesgo elevado de dicha asociación. El diagnóstico precoz fundado en los hallazgos clínicos, estudios neurofisiológicos y dosificación de títulos de anticuerpos en el suero permite iniciar tempranamente el tratamiento sintomático y la búsqueda oncológica. Son escasos los informes de pacientes con LEMS en Latinoamérica. Objetivo. Describir las características de pacientes con LEMS de un centro privado de Buenos Aires, Argentina, y compararlas con las de otras series publicadas. Pacientes y métodos. Se revisaron historias clínicas de 13 pacientes con LEMS con hallazgos clínicos, electromiograma compatible y/o anticuerpos positivos. Se realizó seguimiento hasta descartar o confirmar una neoplasia asociada de acuerdo con los algoritmos recomendados. Resultados. Cuatro pacientes presentaron diagnóstico de T-LEMS, dos de ellos con carcinoma de pulmón de células pequeñas. De los nueve pacientes con NT-LEMS, cinco presentaron una puntuación DELTA-P de 3 y 4. Nueve pacientes presentaron la tríada clínica clásica desde el inicio. Todos los pacientes presentaron en el electromiograma hallazgos compatibles con defecto de placa neuromuscular presináptico. El 70% mejoró sintomáticamente con piridostigmina. Conclusiones. Los hallazgos clínicos, junto con los estudios neurofisiológicos compatibles, resultan suficientes para el diagnóstico de LEMS. No pudo replicarse la relación entre puntuación DELTA-P y riesgo de carcinoma de pulmón de células pequeñas. El tratamiento sintomático con piridostigmina representa una alternativa terapéutica eficaz.

Neuroendocrine carcinoma of prostate presenting with ascites.

Prostate cancer is a very common cause of morbidity and mortality in India. The commonest type of prostate carcinoma is adenocarcinoma, most of them are confined to prostate at the time of presentation. Bone is the preferred site for metastasis. The following is a case of neuroendocrine carcinoma of prostate arising in a 36 years old male who presented with ascitis and jaundice to the emergency department. The ascitic tap was positive for malignant cells. CECT done to detect primary showed osteoblastic secondaries in the spine along with lesions in the liver. DRE revealed grade 2 prostatomegaly. A TRUS guided biopsy showed neuroendocrine carcinoma of the prostate. Neuroendocrine carcinoma is a very rare type of prostatic carcinoma, with presentation of the same as ascitis is very uncommon. The following case is presented due to its rarity.

Small Cell Carcinoma of the Ovary, Hypercalcemic Type, in a 12-Month-Old Girl.

Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is a highly aggressive malignant tumor affecting predominantly young adults and adolescents with an average age of 23.9 at time of diagnosis. Up to two thirds of patients have paraneoplastic hypercalcemia. The molecular signature of these tumors is SMARCA4 mutations, with somatic and germline pathogenic variants previously described. We report a case of a previously healthy one-year-old girl who was noticed to have mild anemia and an abdominal mass during a well-child visit. Further laboratory testing revealed hypercalcemia. A computerized tomography scan showed a left-sided ovarian mass (9.3 x 7.3 x 7 cm). The resection specimen showed a large ovarian tumor with solid tan-yellow cut surfaces and small foci of necrosis. Microscopically, the tumor was composed of sheets of small, hyperchromatic epithelioid cells with focal rhabdoid large cell morphology. The tumor cells were strongly and diffusely positive for WT1 (N-terminal antibodies) with focal EMA and Pan-keratin positivity. Absent SMARCA4 (BRG1) protein expression by immunohistochemistry ultimately established the diagnosis of small cell carcinoma of the ovary, hypercalcemic type. To our knowledge, this is the youngest patient reported in the literature.

Small cell carcinoma of the cervix complicated by syndrome of inappropriate antidiuretic hormone secretion: a case report.

Small cell carcinoma of the cervix is a rare malignant tumor in the clinical setting. Clinical manifestations of this tumor are mostly similar to those of normal types of cervical cancer. Small cell carcinoma of the cervix only shows symptoms of neuroendocrine tumors, such as syndrome of inappropriate antidiuretic hormone secretion (SIADH). Most of the hyponatremia caused by SIADH can be managed after removal of the cause. Hyponatremia is a predictor of poor prognosis and can be used as an indicator of partial recurrence. We report a case of small cell carcinoma of the cervix complicated by SIADH. Our patient presented with irregular vaginal bleeding after menopause. After one cycle of chemotherapy, there was trembling of the limbs, and a laboratory examination showed low Na+ and low Cl- levels. After limited water intake, intravenous hypertonic saline, and intermittent diuretic treatment, the patient's blood Na+ levels returned to normal. After a radical operation, the above-mentioned symptoms disappeared.

Hypercalcemia and Unilateral Ovarian Mass in a Young Adult: A Case Report of Small Cell Ovarian Carcinoma.

BACKGROUND Small cell ovarian carcinoma, hypercalcemic type is an uncommon malignant ovarian tumor entity with an unfavorable prognosis and a short overall survival rate. It mainly affects women of childbearing age. CASE REPORT We report a case in which a 31-year-old woman with small cell ovarian carcinoma, hypercalcemic type presented with unspecific symptoms. We emphasize the importance of treatment planning and address fertility-sparing surgical procedures, which remain a therapeutic dilemma. CONCLUSIONS The occurrence of unspecific abdominal symptoms, unilateral tumor masses, and hypercalcemia may indicate the presence of malignant ovarian neoplasm in young adults. Histopathological examination of the mass should be performed by an experienced gynecological pathologist. A misdiagnosis can lead to inadequate surgical and adjuvant treatment. Adjuvant multi-agent chemotherapy and high-dose chemotherapy with autologous stem cell rescue may prolong the progression-free interval and overall survival.

Mediastinal small cell carcinoma with a metastasis to the orbit: A rare carcinoma with an unusual metastatic progression.

Extraocular muscle (EOM) is a rare site for orbital metastasis. We presented a case of solitary EOM metastasis from mediastinal small cell cancer (MSCC) for the first time. A 49-year-old man presented with hoarseness. Thorax computed tomography (CT) revealed a mediastinal mass. A fine-needle aspiration biopsy (FNAB) confirmed the diagnosis of MSCC. The patient staged as limited-stage MSCC with a positron emission computed tomography (PET-CT). The patient received radical chemo-radiotherapy (CRT). PET-CT showed a complete response after CRT. Afterward, the patient presented with double vision and a headache. Brain magnetic resonance imaging (MRI) demonstrated a 2 cm metastatic lesion at the left inferior rectus muscle. A 30 Gy palliative RT was applied. The full regression of the mass was achieved 3 months after the palliative RT. Although solitary EOM metastasis is rare, the timing of accurate diagnosis and appropriate treatment can help to preserve the patient's vision and relieve complaints related to the mass.

New lung mass in a patient with granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (GPA) is a potentially lethal ANCA-associated small-vessel vasculitis characterized by a typical triad of upper respiratory tract, lung, and kidney involvement. Lung involvement in GPA occurs in 25-80% of cases. The most common radiographic and computed tomography (CT) abnormalities of pulmonary GPA are lung nodules and masses, very often multiple and with cavitation. As there are various clinical presentations, the diagnosis of GPA can be challenging, and the illness is difficult to distinguish from other diseases such as infection or malignancy. Following the improved survival rates in patients with GPA, there is accumulating evidence to suggest an increased occurrence of different types of cancer. Exposure to cyclophosphamide seems to be one of its main causes. We present the case of a patient with chronic GPA who was hospitalized owing to a new infiltrate in the lung, suggesting relapse of the disease, and finally diagnosed with small cell lung cancer. Data regarding lung cancer in GPA patients are limited. While there are some case reports and short case series in the literature, there are no detailed data regarding an association between CYC exposure and lung cancer development in vasculitis. It is necessary to consider the causes of pulmonary masses other than a GPA relapse. Bronchoscopy with biopsy and histopathological examination are crucial in proper differential diagnosis. GPA patients require long-term follow-up to monitor for the development of complications during treatment.

Neuroendocrine carcinoma of the endometrium: A very rare gynecologic malignancy.

OBJECTIVE: To investigate the clinicopathologic characteristics, prognostic factors, outcome, and treatment of the neuroendocrine carcinoma (NEC) of the endometrium. MATERIALS AND METHODS: We retrospectively reviewed the clinicopathologic and survival data of 10 patients who underwent surgery for NEC. The patients were collected between 1999 and 2017 from four referral centers in Turkey. RESULTS: The median age of patients was 67 years (range: 34-75 years). The NEC of endometrium consist of 9 cases with small cell carcinoma (SC) NEC (two with mixed histotypes), and one with a large cell (LC) NEC. According to FIGO 2009 criteria, 70 % (7/10) of patients had advanced stage (III and IV) disease. All patients except one underwent surgical staging, eight patients received platinum-based chemotherapy (CTX) and of 6 those were additionally treated with radiotherapy (RT). Four patients died of disease ranging from 2 to 10 months and six were alive 12-72 months with no evidence of disease. In addition, 4 SC NEC cases raised in polypoid features had no evidence of disease from 24 to 72 months. DISCUSSION: NEC of the endometrium is a rare disease with poor prognosis, which frequently diagnosed in advanced stages. The main treatment modality was the administration of platinum-based CTX as an adjuvant to surgery or surgery and RT. Our result suggests that the polypoid feature of the tumor might be one of the best predictors for the prognosis of SC NEC.

Neuroendocrine tumors of the urinary bladder - Report of two cases

Primary paraganglioma and small cell neuroendocrine carcinoma of the urinary bladder are rare tumors, comprising 0.05% of all bladder tumors and <1% of all malignant bladder tumors, respectively. These tumors can be the cause of a diagnostic dilemma or misdiagnosis on morphology. Paraganglioma is often mistaken for urothelial carcinoma and small cell carcinoma for poorly differentiated carcinoma or lymphoma. Herein, we report a case of primary paraganglioma and another of a small cell carcinoma of the urinary bladder and discuss their closest differential diagnoses. The diagnostic pitfalls should be kept in mind so that correct, timely diagnosis of these entities can be made due to implications in the management and prognosis.