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 Authors: Jonathan E. Zuckerman, Neda A. Moatamed 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 Authors: Safae Terrisse, Maria Eugenia Comblor, J érôme Vérine, Hélène Gauthier, Pierre Mongiat-Artus, Stéphane Culine 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 Authors: Shunsuke Tamura, Hirotaka Yamamoto, Shinichiro Ushida, Kazufumi Suzuki Source Type: research
Comprehensive genomic profiling of a rare thyroid follicular dendritic cell sarcoma
We previously reported an extremely rare case of follicular dendritic cell sarcoma (FDCS) presented as a thyroid mass. Given the rarity of this disease, there are no personalized and molecularly targeted treatment options due to the lack of knowledge in the genomic makeup of the tumor. A 44- year-old white woman was diagnosed with an extranodal FDCS in thyroid. The patient underwent a total thyroidectomy, central compartment dissection, parathyroid reimplantation, and adjuvant radiation therapy. Tumor DNA sequencing of 236 genes by FoundationOne panel found truncating mutations in PTEN and missense mutations in RET and TP5...
Source: Rare Tumors - September 15, 2017 Category: Cancer & Oncology Source Type: research
Variegated colors of pediatric glioblastoma multiforme: what to expect?
Malignant gliomas account for 35-45% of primary brain tumors; among these glioblastoma multiforme (GBM) is the most common adult brain tumor constituting approximately 85%. Its incidence is quite less in the pediatric population and treatment of these patients is particularly challenging. Exposure to ionizing radiation is the only environmental factor found to have any significant association with GBM. Several genetic alterations associated with GBM in adults have been well documented such as epidermal growth factor receptor amplification, overexpression of mouse double minute 2 homolog also known as E3 ubiquitin-protein l...
Source: Rare Tumors - August 29, 2017 Category: Cancer & Oncology Source Type: research
Granular cell tumor of cecum: a common tumor in a rare site with diagnostic challenge
Granular cell tumor (GCT) also known as Abrikossoff ’s tumor is a benign neoplasm that is usually seen in the fourth to sixth decades of life with slight female preponderance. It is most frequently seen in the oral cavity, skin, and subcutaneous tissue. Gastrointestinal tract involvement is uncommon, in which esophagus is the most commonly affected site. There are case reports of GCT in stomach, appendix, colon and rectum. In this article, we report a case of GCT involving cecum. The cell of origin in GCT is controversial. There are various pools of thoughts regarding its histogenesis, the details of which are reviewed i...
Source: Rare Tumors - August 29, 2017 Category: Cancer & Oncology Source Type: research
Novel therapies in platinumrefractory metastatic germ cell tumor: a case report with a focus on a PD-1 inhibitor
Testicular germ cell tumor (GCT) is the most common malignancy in young males between the ages of 15 to 35 years. Although the overall cure rate of GCTs approaches 95%, almost 25% of patients with distant metastases die from the cancer. Active investigations on novel treatment options for platinum-refractory GCTs include immunotherapies such as programdeath 1 (PD-1)/program death-ligand 1 (PD-L1) inhibitors. In this case, we report a patient with metastatic GCTs who was treated with pembrolizumab, a PD-L1 inhibitor, in a phase II study after failing several lines of chemotherapy. We highlighted the rationale for the use of...
Source: Rare Tumors - July 3, 2017 Category: Cancer & Oncology Source Type: research
A case of neuroendocrine carcinoma developing from the broad ligament of the uterus
Neuroendocrine carcinoma (NEC), also called small cell carcinoma or large cell carcinoma, is a rare and aggressive tumor that develops mainly in the lung and intestine. More rarely, NEC develops in gynecologic organs, with poor prognoses. We experienced a case of NEC in the broad ligament of the uterus. The patient was a 74-year-old woman with symptoms of abdominal distension and constipation. Ultrasound sonography detected an abdominal tumor larger than 10 cm. She was then admitted to our hospital. She underwent surgery under the diagnosis of ovarian cancer, but the bilateral ovaries and uterus were normal in appearance, ...
Source: Rare Tumors - July 3, 2017 Category: Cancer & Oncology Source Type: research
Osteosclerosis secondary to metastatic oligodendroglioma
This paper reviews a case of metastatic 1p/19q codeleted oligodendrioglioma causing diffuse osteosclerosis and pain. Primary central nervous system (CNS) tumors rarely metastasize outside the CNS, and metastatic oligodendroglioma is rarer still. The patient in this study had relief of pain after being treated with temozolomide. We discuss this rare presentation and potential treatment options, and review the literature in regards to metastatic oligodendrogliomas. (Source: Rare Tumors)
Source: Rare Tumors - March 31, 2017 Category: Cancer & Oncology Source Type: research
Myoepithelial carcinoma of the paracecal mesentery: aggressive behavior of a rare neoplasm at an unusual anatomic site
We describe a case of a 44-year-old man who was diagnosed with a primary myoepithelial neoplasm of the paracecal mesentery, which showed aggressive local recurrence after four years. The tumor was composed of cords of ovoid cells within chondromyxoid stroma, and displayed a characteristic pancytokeratin, S100 protein and smooth muscle actin-positive myoepithelial immunoprofile. Primary myoepithelioma has not been previously described at this site, and this case highlights this varied family of tumors, emphasizes the need to consider myoepithelial tumor in the differential diagnoses of carcinoma variants occurring in the bo...
Source: Rare Tumors - March 31, 2017 Category: Cancer & Oncology Source Type: research
Microsatellite instability in medullary carcinoma of the colon
We present the case of a 44 years old Caucasian woman, who referred to the Emergency Room with symptoms mimicking an acute appendicitis. Computed tomography and colonoscopy demonstrated an ulcerated and stenotic lesion of the caecum without signs of metastasis and peritoneal carcinosis. Patient underwent a laparoscopic right colectomy. The final pathologic findings provided the diagnosis of medullary carcinoma with MSI. Patient then underwent adjuvant chemotherapy according to the FOLFOX- 4 protocol (association of 5-Fluorouracil, Leucovorin, and Oxaliplatin) for twelve cycles. At two-years follow-up, patient is disease fr...
Source: Rare Tumors - March 30, 2017 Category: Cancer & Oncology Source Type: research
Large plasmacytoma occupying the upper limb in a myeloma patient
Extramedullary disease (EMD) is an issue for patients with multiple myeloma (MM), since extramedullary spread of MM is associated with an aggressive course and a poor prognosis. Moreover, the mechanism of EMD development is uncertain. Here, we present extensive extramedullary plasmacytoma occupying the left upper limb of a 66- year-old female patient with MM with an extremely aggressive course and multiple visceral organ involvement without bone marrow infiltration or plasma cell leukemia. EMD of this large size is extremely rare and this case may provide a clue for better understanding of clinical features of EMD in MM. (Source: Rare Tumors)
Source: Rare Tumors - March 30, 2017 Category: Cancer & Oncology Source Type: research
Angiofibrolipoma of posterior mediastinum with transforaminal extension
Angiofibrolipoma is a histological variant of lipoma, which commonly occurs in subcutaneous tissues. In the present report we illustrate the case of an angiofibrolipoma of the posterior upper mediastinum in a 75-year-old man presented with progressive chest pain. Xray chest showed a homogeneous opacity vertically oriented along the right lateral aspect of thoracic vertebrae with an obtuse angle to the mediastinum. The upper extremity of the mass extended above the superior clavicle, suggestive of a posterior mediastinal lesion. Thoracic magnetic resonance imaging revealed a posterior mediastinal mass, in keeping with a non...
Source: Rare Tumors - March 29, 2017 Category: Cancer & Oncology Source Type: research
Immunohistochemical diagnosis of primary cardiac leiomyosarcoma in a Latin American patient
Primary cardiac malignancies are rare entities. Although sarcomas enclosed the main group of malignant heart neoplasms, primary cardiac leiomyosarcomas are extremely rare and constitutes less than 8% of cardiac tumors. Leiomyosarcoma usually originates from the pulmonary veins and have a worm-like shaped structure. In this article, we present a case of a 40-year-old Hispanic man diagnosed with a cardiac tumor who underwent surgical resection, during pathological examination the tissue samples were consistent with a malignancy of mesenchymal origin that contained irregular bundles of spindle cells. Subsequent immunohistoche...
Source: Rare Tumors - March 28, 2017 Category: Cancer & Oncology Source Type: research
Hairy cell lymphoma: a potentially under-recognized entity
We present an additional case presenting as a thoracic mass, and discuss potential diagnostic pitfalls and management of these rare cases. (Source: Rare Tumors)
Source: Rare Tumors - March 28, 2017 Category: Cancer & Oncology Source Type: research
