Primary Hepatic Neuroendocrine Carcinoma with Metastasis to the Mesentery: A Case Report.

Primary hepatic neuroendocrine carcinomas (PHNECs) are extremely rare, with only about 90 cases having been reported in the English-language literature. Among all neuroendocrine neoplasms, primary hepatic neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs) are extremely rare, accounting for 0.3% of NETs and 0.28-0.46% of malignant liver tumors. Additionally, primary hepatic NECs occur infrequently. The clinical diagnosis of primary hepatic NEC remains challenging because of its rarity and the lack of information about its characteristic appearance on images. Consequently, pathological examination through the performance of a preoperative liver tumor biopsy is essential for diagnosis. Due to the lack of availability of substantial high-quality data, there is no standard therapy for primary hepatic NEC. We present the first case of PHNEC metastasized to the mesentery reported in the English-language literature.

Mandibular Metastasis as the First Manifestation of Hepatocellular Carcinoma: A Case Report.

Metastatic malignancies of the oral cavity are rare lesions, accounting for only 1-4% of all oral malignancies, and can occur in the jaw bones, the oral soft tissues, or even both. Although hepatocellular carcinoma is the most common primary hepatic tumor, no more than 1% of the cases show oral involvement. When metastatic tumor involves the oral cavity, the most frequent site is the posterior angle of the mandible. Histologically, hence, immunohistochemical markers are used for diagnosis. Glypican-3 and HepPar1 are the markers that can be used to confirm the microscopic diagnosis of HCC. Very rarely, hepatocellular carcinoma (HCC) metastasizes to the oral cavity, and such cases have a poor prognosis due to delay in diagnosis. We present a 74-year-old male with a metastasis of HCC in the left mandibular body as the first manifestation. Histologic examination confirmed metastatic hepatocellular carcinoma in the oral mucosa with immunohistochemical (IHC) markers. A review of pertinent literature was performed. Given the rarity of the disease, treatment principles are based mainly on retrospective series and case reports. We report an exceptionally unusual presentation with few cases (<70) reported in the literature, thus representing a diagnostic challenge.

Calcitonin-Negative Neuroendocrine Carcinoma of the Thyroid Gland: Case Report and Literature Review.

Calcitonin-negative neuroendocrine tumor (CNNET) of the thyroid is an extremely rare entity. In some of the previously reported cases within the literature, the terms "atypical medullary thyroid carcinoma," "calcitonin-free oat cell carcinoma," and "a distinct clinical entity" were applied to NETs without definitive evidence of calcitonin production. In the English-language literature, not only are there only few reported cases of CNNET, but the criteria for diagnosis in these cases are also controversial. Most of the current published cases were also treated surgically for local disease. We describe a case of NET of the thyroid with calcitonin, chromogranin A and thyroglobulin negativity, synaptophysin and TTF-1 positivity, and a high Ki-67 proliferation index with metastases in the cervical region as well as mediastinal adenopathies. This case was considered an unresectable thyroid carcinoma, and chemotherapy including cisplatin and etoposide was started as neoadjuvant treatment at the department of medical oncology. Total thyroidectomy plus bilateral and central cervical dissection was performed, and the patient underwent 2 cycles of adjuvant radiotherapy. Currently, the patient's 18F-FDG-PET/CT findings show a complete response 17 months after diagnosis. In conclusion, CNNET of the thyroid is very rare and there is limited evidence regarding treatment in patients with metastases. Chemotherapy including cisplatin and etoposide as well as early aggressive surgical resection appears to positively impact patients' survival.

Breast Metastasis of Gastric Signet Ring Cell Carcinoma: A Case Report and Literature Review.

Breast metastasis from gastric signet ring cell carcinoma is extremely rare in clinical practice. The estimated incidence is 0.5-1.3%. There are few cases reported in the literature (approx. less than 60) of breast metastasis from gastric signet ring cell carcinoma, and due to the rare association between gastric cancer and its extension to the breast, it is difficult to establish the diagnosis. Clinical history, histological findings, and immunohistochemical markers are helpful in distinguishing primary breast cancer from breast metastasis of gastric cancer. The treatment for breast metastasis from gastric carcinoma remains controversial. The prognosis of breast metastasis from gastric carcinoma is generally poor. We report a case of breast metastasis of gastric signet ring cell carcinoma in a 38-year-old woman. She started chemotherapy with ramucirumab, paclitaxel, and irinotecan. Three months later, a combined 2-[18F]-fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography showed a complete response. This is the first reported case of breast metastasis from gastric signet ring cell carcinoma with a complete response.

Myoepithelial Carcinoma Arising in a Plasmacytoid Myoepithelioma of the Parotid Gland Synchronized with Melanoma: A Case Report and Review of the Literature.

Myoepithelial carcinoma, also known as malignant myoepithelioma, is considered an extremely rare (0.45-1%) malignant salivary gland neoplasm. Approximately 100 cases have been reported in the English-language literature on myoepithelial carcinoma. The majority of the myoepitheliomas described in the literature have been benign, and the malignant counterpart is considered rare (<1%). Such a tumor may appear de novo or rarely develop from a preexisting pleomorphic adenoma (<20%), and in exceedingly rare cases (<0.5%), it has arisen from a benign myoepithelioma (i.e., plasmacytoid myoepithelioma). To our knowledge, no case of myoepithelial carcinoma of the parotid gland arising in a plasmacytoid myoepithelioma synchronized with melanoma has been reported to date. The treatment of myoepithelial carcinoma has been mainly surgical, including wide excision with free margins, with or without nodal dissection. The roles of chemotherapy and radiotherapy have not yet been established. We report a case of myoepithelial carcinoma of the parotid gland arising in a plasmacytoid myoepithelioma synchronized with melanoma in a 40-year-old woman. In our case, a complete response was achieved with surgery followed by adjuvant chemotherapy based on carboplatin and paclitaxel concurrent with radiotherapy.

Giant Benign Mammary Phyllodes Tumor: Report of a Case and Review of the Literature.

Phyllodes tumor of the breast is an infrequently encountered fibroepithelial neoplasm, which accounts for 0.3-1% of all tumors. Few case reports have described the occurrence of giant phyllodes tumor. To our knowledge, about 20% of phyllodes tumors would be considered giant benign. Complete surgical excision is the standard of care for giant benign phyllodes tumors; axillary lymph node metastasis is rare, and dissection should be limited to patients with pathologic evidence of tumor in the lymph nodes. We report the case of a 40-year-old Mexican woman with giant mammary tumor who underwent a right total mastectomy. The pathology results showed a benign phyllodes tumor 4,857 g in weight and 40.2 × 36.3 × 15 cm in size. We do not suggest adjuvant radiation therapy for patients with benign phyllodes tumors that are widely excised. A review of the pertinent literature was performed.

Neuroendocrine Tumor of the Common Bile Duct: Case Report.

Carcinoma of the extrahepatic biliary tract accounts for <2% of all cancers. Neuroendocrine tumor of the extrahepatic bile duct is very rare, and there are <200 cases reported since 1959. The preoperative diagnosis is infrequent (5.12%). The definite diagnosis relies on postoperative pathology which utilized immunohistochemistry study on many biomarkers to diagnose the histological subtypes of neuroendocrine neoplasms, such as chromogranin A, synaptophysin, and neuron-specific enolase. When the primary tumor has no metastases, radical removal of the lesion appears as curative treatment. The treatment of the carcinoid syndrome or other functioning syndrome is the first priority. We report a case of a 12-year-old Mexican woman with neuroendocrine tumor of the extrahepatic bile duct (common bile duct neuroendocrine tumor) seen in our hospital. Resection of the common bile duct, cholecystectomy, end to side Roux-en-y hepaticojejunostomy, and portal lymphadenectomy was performed. A review of the pertinent literature was performed. Given the rarity of the disease, treatment principles are based mainly on retrospective series and case reports. We present the eighth case in adolescence in the literature.

Acute Cardiac Tamponade Secondary to Cardiac Vascular-Type Pleomorphic Leiomyosarcoma: Case Report.

Soft tissue sarcomas represent <1% of all neoplasms. Leiomyosarcomas comprise only 5-7% of cases, and only 2% of these are vascular. Vascular leiomyosarcomas are extremely rare and represent only 0.001% of all neoplasms, the venous type being up to 5 times more frequent. Arterial leiomyosarcomas most frequently affect the great vessels, being fatal in most cases. In the reported cases of arterial leiomyosarcomas, the most frequently affected site is the pulmonary artery. We present the clinical case of 2 patients (a 42-year-old woman and a 36-year-old man) with a diagnosis of arterial pleomorphic leiomyosarcoma that conditioned cardiac tamponade as the initial manifestation. As it is an exceptionally rare neoplasm and with few cases reported in the literature, it is important to identify and describe this pathology which, due to the impossibility of offering surgical treatment, represents a therapeutic challenge.

Epithelial-Cadherin Expression Is Associated With Better Recurrence-Free and Overall Survival in Invasive Lobular Breast Cancer in Mexican Women.

BACKGROUND: There are different clinicopathological characteristics that are associated with the prognosis in patients with breast cancer. To date, the prognostic valor of for epithelial cadherin (E cadherin) expression in invasive lobular breast cancer remains unknown. METHODS: A retrospective single-center study that included 207 patients with primary invasive lobular breast cancer was conducted. The primary outcome was to report the correlation of the different clinical pathological characteristics including the expression of epithelial-cadherin (E-cadherin) in invasive lobular breast cancer in Mexican women with recurrence-free survival (RFS) and overall survival (OS). RESULTS: After 11 years of follow-up of patients with invasive lobular breast cancer, RFS was 89.4% and OS of 96.1%. The best prognosis in RFS was in patients with negative nodes 95.2% (P = 0.0001) and OS was 98.6-100% (P = 0.0001). Regarding tumor size, an RFS of 98.3% was observed in those measuring ≤ 2 cm (P = 0.0001) and OS of 99.2% (P = 0.0001). Negative Her2 was related to an RFS of 92.1% (P = 0.0001), and had better OS of 98.3% (P = 0.0001). Ki67 proliferation index ≤ 14% was associated with an RFS of 93.2% (P = 0.005). Negative lymph vascular invasion (LVI) increases the RFS of 91.8% (P = 0.032). The rate of positive expression of E-cadherin was associated with an increase in the RFS of 97.4%, with a mean of 128.6 ± 2.4 months (95% confidence interval (CI): 123.75 - 133.45 months) compared to the absence of expression E-cadherin: signal log ratio (SLR) 68.9%, a mean of 95 ± 6 months (95% CI: 83.28 - 106.88 months), P <0.001. When the OS was analyzed, the presence of E-cadherin expression increased the OS of 100% vs. 86.9% with the absence, P = 0.015. CONCLUSIONS: The prognostic impact of the different clinicopathological characteristics known worldwide was confirmed. Results of the analysis in the presented study indicate that positive expression of E-cadherin correlates with an improvement in OS and RFS in invasive lobular breast cancer in Mexican women.

Intergluteal Cleft Eccrine Porocarcinoma with Metastasis to Inguinal Region and Lung: Case Report and Review of the Literature.

Eccrine porocarcinoma (EPC) is an infrequent cutaneous neoplasm, and was described in 1963 by Pinkus and Mehregan. It is a rare type of skin tumor (0.005-0.01% of all skin tumors). Less than 300 cases have been described in the entire world medical literature. To our knowledge, no case of intergluteal cleft EPC has been reported in the literature in English and Spanish to date, so this would be the first reported case of such pathology. Metastatic EPC is less frequent, since only <10% of metastatic type have been reported and the rest as localized disease. The primary treatment of choice is surgical wide local excision of the tumor with histological confirmation of tumor-free margins. Prognosis is difficult to determine because of the rarity of EPC and the variations in natural history. There are no data to support the use of adjuvant chemotherapy or radiotherapy, and there are currently no agreed criteria to define patients at high risk of relapse. We present a 67-year-old man with intergluteal cleft eccrine tumor by biopsy. Metastasis to left inguinal region and lung was reported by contrasted abdominal and chest computed tomography. He started chemotherapy based on etoposide, vincristine, carboplatin. A review of pertinent literature is provided.

Progressive supranuclear palsy as differential diagnosis of Parkinson's disease in the elderly / Parálisis supranuclear progresiva como diagnóstico diferencial de la enfermedad de Parkinson en los ancianos

Introduction: Progressive supranuclear palsy (PSP) is a syndrome characterized by progressive parkinsonism with early falls due to postural instability, typically vertical gaze supranuclear ophthalmoplegia, pseudobulbar dysfunction, neck dystonia and upper trunk rigidity as well as mild cognitive dysfunction. Progressive supranuclear palsy must be differentiated from Parkinson's disease taking into account several so-called red flags. Materials and methods: We report a case series hallmarked by gait abnormalities, falls and bradykinesia in which Parkinson's disease was the initial diagnosis. Results: Due to a torpid clinical course, magnetic resonance imaging (MRI) was performed demonstrating midbrain atrophy, highly suggestive of progressive supranuclear palsy. Conclusion: The neuroradiological exams (magnetic resonance imaging, single photon emission computer tomography, and positron emission tomography) can be useful for diagnosis of PSP. Treatment with levodopa should be considered, especially in patients with a more parkinsonian phenotype

Introducción: La parálisis supranuclear progresiva (PSP) es un síndrome caracterizado por parkinsonismo progresivo con caídas tempranas secundarias a inestabilidad postural, oftalmoplejía supranuclear típicamente de mirada vertical, disfunción seudobulbar, distonía de cuello y tronco superior, rigidez y deterioro cognitivo moderado. La parálisis supranuclear progresiva debe ser diferenciada de la enfermedad de Parkinson tomando en cuenta las llamadas banderas rojas. Materiales y métodos: Reportamos una serie de casos distinguidos por anormalidad de la marcha, caídas y bradicinesia, en quienes el diagnóstico de inicio fue enfermedad de Parkinson. Resultados: Debido a un curso clínico tórpido se realizaron resonancias magnéticas que demostraron atrofia mesencefálica altamente sugestiva de parálisis supranuclear progresiva. Conclusión: El examen neurorradiológico (resonancia magnética, tomografía por emisión de positrones y tomografía simple) pueden ser útiles para el diagnóstico de PSP. El tratamiento con levodopa debe ser considerado especialmente en pacientes con fenotipo parkinsoniano

Progressive supranuclear palsy as differential diagnosis of Parkinson's disease in the elderly.

INTRODUCTION: Progressive supranuclear palsy (PSP) is a syndrome characterized by progressive parkinsonism with early falls due to postural instability, typically vertical gaze supranuclear ophthalmoplegia, pseudobulbar dysfunction, neck dystonia and upper trunk rigidity as well as mild cognitive dysfunction. Progressive supranuclear palsy must be differentiated from Parkinson's disease taking into account several so-called red flags. MATERIALS AND METHODS: We report a case series hallmarked by gait abnormalities, falls and bradykinesia in which Parkinson's disease was the initial diagnosis. RESULTS: Due to a torpid clinical course, magnetic resonance imaging (MRI) was performed demonstrating midbrain atrophy, highly suggestive of progressive supranuclear palsy. CONCLUSION: The neuroradiological exams (magnetic resonance imaging, single photon emission computer tomography, and positron emission tomography) can be useful for diagnosis of PSP. Treatment with levodopa should be considered, especially in patients with a more parkinsonian phenotype.

Cancer immunotherapy experience in the Integral Oncology Centre "Diana Laura Riojas de Colosio", Médica Sur Hospital.

INTRODUCTION: The useof immunotherapy in Mexico has been used since 2012 with ipilimumab and since 2015 with nivolumab and pembrolizumab, so it is a matter of necessity to know the experience of these drugs. MATERIAL AND METHODS: An observational, descriptive, cross-sectional, and retrospective study was performed in Médica Sur Hospital, where with dossiers from 2012 to June 2018 patients with metastatic cancer who received immunotherapy with ipilimumab, nivolumab, and pembrolizumab for six months were evaluated, searching as principal outcomes the adverse effects of those drugs and as secondary outcomes the response to treatment. RESULTS: Seventy subjects fulfilled the inclusion criteria for the study, and 42 (60%) were women with an average age of 60.73 ±13.64 years (16-82 years). The pathologies that received immunotherapy were the following: melanoma and lung cancer. The most frequent clinical and laboratory adverse effects were as follows: fatigue - 32 (45.71%), asthaenia - 30 (42%), nausea - 8 (11.4%), diarrhoea - 8 (11.4%), and rash - 7 (10%). The worst adverse effects were respiratory and endocrinological: pneumonitis - 10 (14.28%), hypothyroidism - 4 (5.71%), hyperglycaemia - 1 (1.4%), and hypophysitis - 2 (2.9%). With respect to treatment response: complete response - 8 (11.4%), partial response - 11 (15.71%), stable disease - 33 (47.14%), and disease progression - 19 (27.14%). CONCLUSIONS: The most common adverse effects did not condition the suspension of treatment or increase in intra-hospital stay, but there were some adverse effects that actually had an impact on evolution, hospital stay, and mortality.

Stress induced cardiomyopathy due to a Mexican earthquake / Cardiomiopatía por estrés inducida por el terremoto en México

Abstract Takotsubo Cardiomyopathy mainly occurs in postmenopausal women, with or with- out cardiovascular disease, and is commonly associated with emotional or physical stress. After nearly 27 years of extensive efforts towards a better understanding of this disorder, current knowledge remains limited. Many people suffer post-traumatic stress, and this situation can be associated to stress cardiomyopathy. The case is presented of a female who suffers stress asso- ciated with the earthquake of 19 September 2017 in Mexico City, and arrived in the Emergency Department in cardiogenic shock.

Resumen La miocardiopatía de Takotsubo ocurre principalmente en mujeres posmenopáusicas con o sin enfermedad cardiovascular, y se asocia comúnmente con estrés emocional o físico. Después de casi 27 an˜os de esfuerzos extensos para una mejor comprensión de este trastorno, el conocimiento actual sigue siendo limitado. Muchas personas sufren estrés postraumático y esta situación puede estar asociada a la cardiomiopatía por estrés. Presentamos el caso clínico de una mujer que sufrió estrés relacionado con el pasado terremoto del 19 de septiembre en la Ciudad de México y llegó al servicio de urgencias en choque cardiogénico.

Stress induced cardiomyopathy due to a Mexican earthquake.

Takotsubo Cardiomyopathy mainly occurs in postmenopausal women, with or without cardiovascular disease, and is commonly associated with emotional or physical stress. After nearly 27 years of extensive efforts towards a better understanding of this disorder, current knowledge remains limited. Many people suffer post-traumatic stress, and this situation can be associated to stress cardiomyopathy. The case is presented of a female who suffers stress associated with the earthquake of 19 September 2017 in Mexico City, and arrived in the Emergency Department in cardiogenic shock.