Langerhan’s cell sarcoma: two case reports
Langerhan’s cell sarcoma (LCS) is a rare neoplasm with a poor prognosis. To our knowledge, only sixty-six cases have been published. We discuss two patients who presented very differently with LCS, as well as a recently published review of all sixty-six cases. Our first case had a complicated history of metastatic, high-grade myxofibrosarcomas and presented with a single skin lesion of LCS which was treated with resection to a positive margin and adjuvant radiotherapy. The LCS recurred locoregionally and was again resected. The patient is alive two years after initial diagnosis. The second case presented with bone marrow...
Source: Rare Tumors - April 6, 2016 Category: Cancer & Oncology Source Type: research

Cancers in young patients in Uyo (Niger-delta region of Nigeria): magnitude of the problem and histopathological prolife
This study shows that embryonal cancer was the most common malignancy in patients aged 0 to 14, while carcinoma of the breast and soft tissue sarcoma were the two most common cancers in patients from 5 to 30 years-old. (Source: Rare Tumors)
Source: Rare Tumors - April 6, 2016 Category: Cancer & Oncology Source Type: research

Histopathologic prolife of primary gastrointestinal malignancies in Uyo city (Niger-delta region of Nigeria)
Incidence of gastrointestinal malignancy is gradually increasing. The aim of the study is to investigate age, sex and relative frequencies of various gastrointestinal malignancies diagnosed between January 2007 and December 2014 in the University of Uyo Teaching Hospital, and in a Private Specialist Laboratory, Uyo, Akwa Ibom State, Nigeria. All histological-diagnosed cases of gastrointestinal malignancies seen during the study period were recruited noting their bio-data and histopathological characteristics. A total of 67 patients aged 6-77 years (mean 58.0, SD 7.4) were enrolled; a male to female ratio of 1.3:1 was recor...
Source: Rare Tumors - April 6, 2016 Category: Cancer & Oncology Source Type: research

Solitary bone plasmacytoma of the pelvis: a rare tumor
We report a case of a solitary bone plasmacytoma, found on the ala of the left ilium of a patient during a routine consult due to hip pain. (Source: Rare Tumors)
Source: Rare Tumors - April 6, 2016 Category: Cancer & Oncology Source Type: research

Importance of an early diagnosis in primary adenocarcinoma of the seminal vesicle
We present the case of a 61-year-old Caucasian man with a left SV mass detected by transrectal ultrasound. SV ultrasound-guided biopsy showed an adenocarcinoma. The tumor was uniformly strongly immunoreactive for cytokeratin-7 and carcinoembryonic antigen. There was no immunoreactivity for prostate-specific acid phosphatase (PSAP) and CK-20. These tumors have been reported to be also positive for CA- 125. Therefore a combination of positive staining for CK-7, CEA and CA-125; with negative staining for CK-20, PSA and PSAP is the pattern of immunohistochemical findings noted for this rare tumor. The computed tomography of th...
Source: Rare Tumors - April 6, 2016 Category: Cancer & Oncology Source Type: research

Soft tissue myoepithelial carcinoma metastatic to the cecum: highlighting an unusual metastatic pattern and the need for diagnostic awareness
We present the case of a 36 year old man with myoepithelial carcinoma arising as a primary tumor within the soft tissues of the neck, which metastasized to the cecum, causing intussusception. This spindle cell neoplasm showed the classic S100 protein, smooth muscle actin and pancytokeratin-positive immunoprofile. Metastasis of myoepithelial carcinoma to the cecum has not been previously described, and coupled with the spindle cell morphology, may cause significant diagnostic difficulty in the absence of clinical familiarity, particularly as there is morphologic overlap with spindle cell neoplasms arising more commonly in g...
Source: Rare Tumors - March 31, 2016 Category: Cancer & Oncology Source Type: research

Diagnostic challenge of desmoplastic melanoma
Desmoplastic melanoma (DM) is a rare variant of spindle-cell malignant melanoma. DM is easily misdiagnosed at an early stage because it can be confused with benign entities. Histological analysis, including careful attention to the presence of atypical spindle cells, as well as to lymphocytic aggregates in an abundant fibrotic stroma in the dermis, provides clues for diagnosis. The adjunction of an immunohistochemical panel, and particularly testing for S-100 protein, is needed for the final diagnosis. (Source: Rare Tumors)
Source: Rare Tumors - March 31, 2016 Category: Cancer & Oncology Source Type: research

Rare carcinoma ex-pleomorphic adenoma of buccal mucosa: case report and review of literature
We present a case of CXPA arising from buccal mucosa in a 44-year-old male patient. The man presented to surgery outpatient department with right buccal mucosa swelling. Clinical impression suggests a neoplasm of buccal mucosa and the patient was sent for fine needle aspiration cytology. By examining the cytological smears, possibility of carcinoma was revealed. The mass was dissected and excised with safety margins. Examining Hematoxylin and Eosin slides, final diagnosis of CXPA was given. Patient did not turn for regular follow-ups and presented 1 year after surgery with recurrence at the same site. CXPA is an uncommon m...
Source: Rare Tumors - March 21, 2016 Category: Cancer & Oncology Source Type: research

Positron emission tomography findings in atypical polypoid adenomyoma
Atypical polypoid adenomyoma (APAM) is a rare polypoid tumor of the uterus composed of atypical endometrial glands surrounded by smooth muscle. A 29-year-old nulligravida, was clinically diagnosed with endocervical myoma and underwent trans-uterine cervical resection with hysteroscope. The histopathological diagnosis of specimens was APAM. Eight months later, she diagnosed recurrent uterine tumor. The positron emission tomography (PET-CT) imaging showed an increased fluorodeoxyglucose uptake. She has performed hysterectomy and was diagnosed APAM. Therapy for APAM depends on multiple factors such as age at presentation and ...
Source: Rare Tumors - March 21, 2016 Category: Cancer & Oncology Source Type: research

Primary multifocal gliosarcoma of the spinal cord
This report confirms that GS is not limited to supratentorial involvement and can primarily affect the spinal cord. (Source: Rare Tumors)
Source: Rare Tumors - March 21, 2016 Category: Cancer & Oncology Source Type: research

Clinical characteristics and outcomes of pediatric patients with desmoplastic small round cell tumor
Desmoplastic small round cell tumor (DSRCT) is a rare malignancy that typically affects pediatric and young adult patients. There are limited data on the clinical features of pediatric DSRCT. We selected patients aged 0-21 years reported to the Surveillance, Epidemiology and End Results Program from 1991-2011. We estimated overall survival using Kaplan-Meier approaches and compared outcomes using the log rank test. The median age of the 95 pediatric patients was 15.3 years (range: 0-21). The majority of tumors originated in the abdomen and pelvis (84.4%) and the majority of patients had distant metastasis (72.6%). A minori...
Source: Rare Tumors - March 21, 2016 Category: Cancer & Oncology Source Type: research

A case of patch stage of Kaposi’s sarcoma and discussion of the differential diagnosis
A 55-year-old HIV positive male had a skin lesion biopsy which showed atypical vascular proliferation within the superficial and deep dermis with mild atypia of lining endothelial cells. A sparse lymphoplasmacytic infiltrate surrounding the irregular vascular channels was noted. Immunohistochemistry highlighted the atypical blood vessels with the vascular markers CD31, CD34 and Factor VIII. The differential diagnosis included unusual vascular or lymphatic proliferations, stasis dermatitis, kaposiform hemangioendothelioma, progressive lymphangioma and angiosarcoma with focal Kaposi’s sarcoma features. Characteristic human...
Source: Rare Tumors - March 21, 2016 Category: Cancer & Oncology Source Type: research

Intracystic papillary carcinoma of the breast in a male patient
We report a case of intracystic papillary carcinoma of the right breast in a 59-year old man presenting with bloody nipple discharge for 1 week prior to presentation. Mammography, ultrasonography, and core needle aspiration were consistent with intracystic papillary carcinoma. The patient underwent right simple mastectomy. Pathology was also consistent with low grade intracystic papillary carcinoma. A 21-gene assay revealed a recurrence score of 0, corresponding to a 3% risk of distant recurrence at 10 years. The patient did not receive chemotherapy or post-mastectomy radiotherapy. The patient was placed on tamoxifen and h...
Source: Rare Tumors - March 21, 2016 Category: Cancer & Oncology Source Type: research

Benign phyllodes tumor of the vulva: a case report and literature review
We report a rare primary case of this tumor arising within the vulva. A 34-year old African American female presented with a 3 cm slow-growing vulvar mass initially thought to be an inclusion cyst. The lesion was excised and histologic examination demonstrated this lesion to be a rare case of benign phyllodes tumor with morphologic features similar to those arising from breast tissue. Patient received no further treatment and did not exhibit any recurrence or metastasis. Nearly two years after excision, the patient died due to an unrelated medical cause. This rare tumor should be considered in the differential diagnosis fo...
Source: Rare Tumors - December 30, 2015 Category: Cancer & Oncology Source Type: research

Undifferentiated embryonal sarcoma of liver
Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant hepatic tumor. A 47 year old male presented with symptoms of sour taste in his mouth, occasional nausea, indigestion and 15-pound weight loss over two months. He had an unremarkable upper gastrointestinal endoscopy. Imaging showed a large liver mass in the left hepatic lobe that was resected and then reported as UESL. He went on to develop lung metastases and was initially treated with doxorubicin and ifosfamide followed by switching of therapy to gemcitabine and docetaxel due to progression of disease. He had a good response after two cycles and we...
Source: Rare Tumors - December 30, 2015 Category: Cancer & Oncology Source Type: research