Langerhans cell sarcoma of the skin in association with superficial atypical Langerhans cell proliferation
We report a case of Langerhans cell sarcoma of the skin representing a probable transformation from Langerhans cell histiocytosis. The lesion had a clinical and pathological biphasic pattern, presenting as a plaque and nodule in the lower leg of a 66 year-old female. The plaque area showed a superficial epidermotropic proliferation of atypical Langerhans cells. The associated deep nodule had an overtly sarcomatous Langerhans cell proliferation. The immunohistochemistry profile was characteristic of Langerhans cells in both components. We consider this case a unique example of deep nodular Langerhans cell sarcoma of the ski...
Source: Rare Tumors - October 23, 2017 Category: Cancer & Oncology Source Type: research

Magnetic resonance imaging of pineal tumors and drop metastases: a review approach
Pineal region tumors represent less than 1% and 3-8% of brain tumors in adults and children respectively. There is a wide range of pineal masses, with the majority being germ cell and pineal parenchymal tumors. Magnetic resonance imaging (MRI) is the modality of choice for the assessment of pineal masses. It is considered as the gold standard for the evaluation of the central nervous system. MRI has the ability to produce very detailed images of the brain anatomy and is used to distinguish true pineal masses from parapineal with invasion of the gland. Specific MRI findings are helpful to the differential diagnosis of pinea...
Source: Rare Tumors - October 23, 2017 Category: Cancer & Oncology Authors: Aikaterini G. Solomou Source Type: research

Langerhans cell sarcoma of the skin in association with superficial atypical Langerhans cell proliferation
We report a case of Langerhans cell sarcoma of the skin representing a probable transformation from Langerhans cell histiocytosis. The lesion had a clinical and pathological biphasic pattern, presenting as a plaque and nodule in the lower leg of a 66 year-old female. The plaque area showed a superficial epidermotropic proliferation of atypical Langerhans cells. The associated deep nodule had an overtly sarcomatous Langerhans cell proliferation. The immunohistochemistry profile was characteristic of Langerhans cells in both components. We consider this case a unique example of deep nodular Langerhans cell sarcoma of the ski...
Source: Rare Tumors - October 23, 2017 Category: Cancer & Oncology Authors: Alejandro Peralta Soler, Michael Miller, Thomas L. Smith Source Type: research

Squamous cell lung carcinoma presenting as melena: a case report and review of the literature
Lung cancer has a predilection to widely metastasize to the liver, bone, brain and adrenal glands. Metastasis of primary lung tumors to the stomach is infrequent, with only sporadic cases reported. Most cases are asymptomatic and diagnosed post-mortem on autopsy. The incidence of symptomatic gastrointestinal metastases is extremely rare. Herein, we describe a case of gastric metastasis by squamous cell lung carcinoma, presenting as melena and diagnosed by esophagogastroduodenoscopy. To the best of our knowledge, only twenty other cases in the English literature have reported symptomatic gastric metastasis of lung cancer di...
Source: Rare Tumors - October 4, 2017 Category: Cancer & Oncology Source Type: research

Mucinous cystadenocarcinoma of the breast: the challenge of diagnosing a rare entity
Mucinous cystadenocarcinoma is an extremely rare variant of primary breast tumor which is histologically similar to mucinous cystadenocarcinoma of the ovary and pancreas. Herein we report a case of a 63 years old woman diagnosed with diverse histological types of non-synchronous rare primary breast tumors, a medullary carcinoma of the right breast and a mucinous cystadenocarcinoma of the left breast. Macroscopically the neoplasm appeared multilocular filled with mucoid material. Under light microscopy the cystic areas were lined by columnar cells with abundant intracellular and extracellular mucin. Solid areas were compose...
Source: Rare Tumors - October 3, 2017 Category: Cancer & Oncology Authors: Nektarios Koufopoulos, Christina Goudeli, John Syrios, Evangelos Filopoulos, Lubna Khaldi Source Type: research

Atelectasis in pediatrics: a case of carcinoid tumor
Carcinoid pulmonary tumors occur in the fourth to sixth decades of life. Usually, typical carcinoid arise a decade earlier when compared to atypical carcinoid (45 years and 55 years, respectively). Typical carcinoid tumors are the most common primary lung neoplasm in children and late adolescents, but there are less than 40 cases described in the literature. The clinical presentation is nonspecific and usually the symptoms are due to bronchial obstruction, sometimes with recurrent pneumonia. Its rarity may delay diagnosis but in most cases a favorable course after treatment is observed. The authors describe the case of a 1...
Source: Rare Tumors - October 3, 2017 Category: Cancer & Oncology Source Type: research

Sertoli-Leydig cell tumor of the ovary masquerading as a mucinous adenocarcinoma: a frozen section pitfall
Sertoli-Leydig cells tumors are rare ovarian neoplasms that can be managed with conservative resection given their generally excellent prognosis. Here we report a case of Sertoli-Leydig cell tumor mistakenly diagnosed as an invasive mucinous adenocarcinoma at time of intraoperative consultation because of its blue-mucinous appearance in the frozen section material. The patient subsequently underwent an extensive staging procedure revealing unilateral, ovarian confined disease. The mucinous features seen on frozen section were lost on the slides prepared from formalin fixed tissues. Immunohistochemical work up confirmed the...
Source: Rare Tumors - October 3, 2017 Category: Cancer & Oncology Source Type: research

Primary adenocarcinoma of the seminal vesicle
This report presents a case of adenocarcinoma of the seminal vesicle with lung metastases in which surgical and chemotherapeutic treatments have been carried out. The MVAC dose dense regimen following local resection seems effective in this scenario and may be used in the treatment of this disease. (Source: Rare Tumors)
Source: Rare Tumors - October 3, 2017 Category: Cancer & Oncology Source Type: research

Mucinous cystic neoplasms in male patients: two cases
Mucinous cystic neoplasms (MCNs) of the pancreas in male patients are very rare. Though few cases of MCNs in male patients have been reported, it is difficult to reveal them preoperatively. The first patient was a 50-year-old man and was incidentally found to have a cystic mass of 51 mm in the tail of the pancreas. We performed a distal pancreatectomy. The second patient was a 73-year-old man, incidentally found to have a cystic mass of 25 mm in diameter in the tail of the pancreas and a carcinoma of the bile duct. We performed a subtotal stomachpreserving pancreaticoduodenectomy and distal pancreatectomy. In both cases, t...
Source: Rare Tumors - October 3, 2017 Category: Cancer & Oncology Source Type: research

Magnetic resonance imaging of pineal tumors and drop metastases: a review approach
Pineal region tumors represent less than 1% and 3-8% of brain tumors in adults and children respectively. There is a wide range of pineal masses, with the majority being germ cell and pineal parenchymal tumors. Magnetic resonance imaging (MRI) is the modality of choice for the assessment of pineal masses. It is considered as the gold standard for the evaluation of the central nervous system. MRI has the ability to produce very detailed images of the brain anatomy and is used to distinguish true pineal masses from parapineal with invasion of the gland. Specific MRI findings are helpful to the differential diagnosis of pinea...
Source: Rare Tumors - October 3, 2017 Category: Cancer & Oncology Source Type: research

Mammary analogue secretory carcinoma of salivary glands: diagnostic pitfall with distinct immunohistochemical profile and molecular features
Mammary analogue secretory carcinoma (MASC) is a newly defined entity among salivary gland malignancies which has just been established in the 4th edition of the WHO classification of head and neck tumors. MASC (synonym: secretory carcinoma) are characterized by a specific rearangement of theETV6 gene locus. Here, we present a series of 3 MASC cases including clinical data with follow-up for up to 26 months. All tumours immunhistochemically displayed strong positivity for cytokeratin 7, and mammaglobin, focal positivity for S100, cytokeratin 5/6 and muc-4. In contrast, immunhistochemical stainings against cytokeratin 14, h...
Source: Rare Tumors - October 3, 2017 Category: Cancer & Oncology Source Type: research

Mucinous cystadenocarcinoma of the breast: the challenge of diagnosing a rare entity
Mucinous cystadenocarcinoma is an extremely rare variant of primary breast tumor which is histologically similar to mucinous cystadenocarcinoma of the ovary and pancreas. Herein we report a case of a 63 years old woman diagnosed with diverse histological types of non-synchronous rare primary breast tumors, a medullary carcinoma of the right breast and a mucinous cystadenocarcinoma of the left breast. Macroscopically the neoplasm appeared multilocular filled with mucoid material. Under light microscopy the cystic areas were lined by columnar cells with abundant intracellular and extracellular mucin. Solid areas were compose...
Source: Rare Tumors - October 3, 2017 Category: Cancer & Oncology Source Type: research

Mammary analogue secretory carcinoma of salivary glands: diagnostic pitfall with distinct immunohistochemical profile and molecular features
Mammary analogue secretory carcinoma (MASC) is a newly defined entity among salivary gland malignancies which has just been established in the 4th edition of the WHO classification of head and neck tumors. MASC (synonym: secretory carcinoma) are characterized by a specific rearangement of theETV6 gene locus. Here, we present a series of 3 MASC cases including clinical data with follow-up for up to 26 months. All tumours immunhistochemically displayed strong positivity for cytokeratin 7, and mammaglobin, focal positivity for S100, cytokeratin 5/6 and muc-4. In contrast, immunhistochemical stainings against cytokeratin 14, h...
Source: Rare Tumors - October 3, 2017 Category: Cancer & Oncology Authors: Oliver Bissinger, Carolin G ötz, Andreas Kolk, Henning A. Bier, Abbas Agaimy, Henning Frenzel, Sven Perner, Julika Ribbat-Idel, Klaus Dietrich Wolff, Wilko Weichert, Caroline Mogler Source Type: research

Atelectasis in pediatrics: a case of carcinoid tumor
Carcinoid pulmonary tumors occur in the fourth to sixth decades of life. Usually, typical carcinoid arise a decade earlier when compared to atypical carcinoid (45 years and 55 years, respectively). Typical carcinoid tumors are the most common primary lung neoplasm in children and late adolescents, but there are less than 40 cases described in the literature. The clinical presentation is nonspecific and usually the symptoms are due to bronchial obstruction, sometimes with recurrent pneumonia. Its rarity may delay diagnosis but in most cases a favorable course after treatment is observed. The authors describe the case of a 1...
Source: Rare Tumors - October 3, 2017 Category: Cancer & Oncology Authors: Cl áudia Guerreiro, Paula Ornelas, Luísa Pereira, Nuno Abecasis, Maria Teresa Almodovar Source Type: research