Adenomatoid tumor of the adrenal gland in young woman: from clinical and radiological to pathological study
We present a case of a 30 years old female, with incidental ultrasound finding of unilateral tumor in the right adrenal gland. Multi slices CT scan was of value in localizing this tumor, but not in the precise diagnosis. The tumor ranged from 5.6 cm to 6.4 cm in greatest diameter. Clinical and hormonal examinations excluded Sy. Cushing, M. Conn and pheochromocytoma. The patient underwent laparoscopic right adrenalectomy. A large tumor (d: 8 ×7×3 cm) was removed showing no infiltration of the adrenal cortex or medulla, or extra-adrenal extension into the periadrenal adipose tissue. Histological examination showed numerous...
Source: Rare Tumors - December 7, 2016 Category: Cancer & Oncology Source Type: research

Primary intraosseous smooth muscle tumor of uncertain malignant potential: original report and molecular characterization
We report the first case of primary intraosseous smooth muscle tumor of uncertain malignant potential (STUMP) which is analogous to borderline malignant uterine smooth muscle tumors so designated. The tumor presented in the femur of an otherwise healthy 30-year-old woman. Over a 3-year period, the patient underwent 11 biopsies or resections and 2 cytologic procedures. Multiple pathologists reviewed the histologic material including musculoskeletal pathologists but could not reach a definitive diagnosis. However, metastases eventually developed and were rapidly progressive and responsive to gemcitabine and docetaxel. Molecu...
Source: Rare Tumors - November 17, 2016 Category: Cancer & Oncology Source Type: research

Primary intimal sarcoma of the left atrium: an incidental finding on routine echocardiography
The objective of this report is to describe a case of this rare disease entity, and to discuss its presentation, pathological findings and management. (Source: Rare Tumors)
Source: Rare Tumors - November 17, 2016 Category: Cancer & Oncology Source Type: research

Mayo Clinic Cancer Center experience of metastatic extramammary Paget disease 1998-2012
This study sought to evaluate the treatment regimens and outcomes of patients treated at a Mayo Clinic Center from 1998-2012. Cancer registry inquiry revealed 261 patients with report advanced Paget disease during these years. Ten cases of metastatic EPMD were identified with sufficient documentation for review. This review reveals support for utilizing localized radiation therapy for bulky disease sequentially with systemic chemotherapy consisting of carboplatin and paclitaxel or irinotecan. Further studies are necessary to define the optimal treatment regimen. (Source: Rare Tumors)
Source: Rare Tumors - November 17, 2016 Category: Cancer & Oncology Source Type: research

Soft tissue myoepithelial carcinoma of the neck with spinal invasion
We report the hospital course of a 33-year-old female who presented to our institution with a posterior neck mass with spinal invasion, diagnosed as myoepithelial cancer. A literature review of these rare tumors is discussed here. (Source: Rare Tumors)
Source: Rare Tumors - October 6, 2016 Category: Cancer & Oncology Source Type: research

A giant phyllodes tumor of the breast
Phyllodes tumors of the breast are rare, accounting for less than 1% of the breast tumors. They are mostly seen in women between 45 and 49 years old. These are fast growing tumors with a large spectrum of behavior (from benign to metastatic) and can resemble fibroadenomas. Correct diagnosis mostly through core needle biopsy is important to decide whether a surgical excision has to be done. Here we report a case of a 57-year-old woman with a fast growing, ulcerated tumor in the left breast. Core needle biopsy suggested a malignant phyllodes tumor with heterologous liposarcomatous differentiation. Treatment with total mastec...
Source: Rare Tumors - October 6, 2016 Category: Cancer & Oncology Source Type: research

Gastric metastasis of breast cancer: a case series
Gastric metastasis is rare but it can be the initial symptom of cancer. The second leading cause of this type of metastasis is breast cancer. A lack of clinical signs and nonspecific side effects of the treatment of primary tumors can lead to the misdiagnosis of metastatic gastric cancer. Upper gastrointestinal endoscopy with biopsy and immunohistochemistry should be used for diagnosis. Treatment is palliative; it includes chemo, endocrine, and radiation therapies. Four patients with breast cancer and gastric metastasis were identified. All the patients tested positive for estrogen and progesterone receptors, and received ...
Source: Rare Tumors - October 6, 2016 Category: Cancer & Oncology Source Type: research

Unusual ultrasound presentation of testicular metastasis from renal clear cell carcinoma
We report a rare case of testicular metastasis from RCC. A 69-year-old patient presented with discomfort and pain in his left testis. He had undergone laparoscopic left radical nephrectomy at another institution. Scrotal ultrasonography revealed a non-palpable lesion at the upper pole of the left testis with hypoechoic aspect, highly suspicious for malignancy. We performed a left inguinal orchiectomy. The testicular lesion was diagnosed as a metastasis from RCC. After orchiectomy, a computed tomography of the chest and abdomen revealed no other metastatic lesions. The patient remains free of clinical recurrence after 20 mo...
Source: Rare Tumors - October 6, 2016 Category: Cancer & Oncology Source Type: research

Primary hepatic lymphoma: a retrospective, multicenter Rare Cancer Network study
Primary hepatic lymphoma (PHL) is a rare malignancy. We aimed to assess the clinical profile, outcome and prognostic factors in PHL through theRare Cancer Network (RCN). A retrospective analysis of 41 patients was performed. Median age was 62 years (range, 23- 86 years) with a male-to-female ratio of 1.9:1.0. Abdominal pain or discomfort was the most common presenting symptom. Regarding B-symptoms, 19.5% of patients had fever, 17.1% weight loss, and 9.8% night sweats. The most common radiological presentation was multiple lesions. Liver function tests were elevated in 56.1% of patients. The most common histopathological di...
Source: Rare Tumors - October 6, 2016 Category: Cancer & Oncology Source Type: research

Locally advanced stage high-grade mucoepidermoid carcinoma of salivary gland in a 9-year-old girl: the controversy of adjuvant therapy
Malignant salivary gland tumors are rare in children, mostly represented by low-grade mucoepidermoid carcinomas. For these patients, long-term survival rates above 95% are reported after surgical resection. Here we report a case of a 9-year-old girl with a high grade locally advanced mucoepidermoid carcinoma undergoing adjuvant radiotherapy and chemotherapy after surgery. We emphasize the controversy and lack of evidence-based indication for these highly toxic adjuvant therapy modalities in children. (Source: Rare Tumors)
Source: Rare Tumors - October 6, 2016 Category: Cancer & Oncology Source Type: research

Erlotinib induced fatal interstitial lung disease in a patient with metastatic non-small cell lung cancer: case report and review of literature
We present the case of a patient diagnosed with stage IV adenocarcinoma of the lung with metastasis to brain. He was found to be positive for the human epidermal growth factor mutation and was hence started on erlotinib. Within a few weeks of starting the medicine the patient was admitted with diarrhea. During the course of this admission he developed acute shortness of breath diagnosed as interstitial pneumonitis. The purpose of this case report is to review the literature associated with erlotinib induced interstitial pneumonitis and make the practicing oncologists aware of this rare yet fatal complication of erlotinib. ...
Source: Rare Tumors - October 5, 2016 Category: Cancer & Oncology Source Type: research

Solitary fibrous tumor of the liver: a rare tumor in a rarer location
We report a case of hepatic solitary fibrous tumor in a 56-year-old female, who presented with right upper abdominal pain. An extended right hepatectomy was performed. Histopathological and immunohistochemical examination revealed solitary fibrous tumor of the liver. (Source: Rare Tumors)
Source: Rare Tumors - October 5, 2016 Category: Cancer & Oncology Source Type: research

Primary retroperitoneal melanoma presented in a rare extracutaneous site for malignant melanoma
Malignant melanoma, as the name implies, is a malignant tumor of melanocytes, found in the skin, eyes, meningeal lining and the mucosal epithelium of the aero-digestive and genitourinary tracts. Malignant melanoma is typically skin malignancy, which rarely presents at extracutaneous site. Here we present a rare case of primary retroperitoneal melanoma and review the findings in comparison with other cases described in literature. (Source: Rare Tumors)
Source: Rare Tumors - October 5, 2016 Category: Cancer & Oncology Source Type: research

Angiosarcoma: a case report of gingival disease with both palatine tonsils localization
We present here, to our knowledge, the first case of a 83-year-old man with gingival and both palatine tonsils localization of a grade-two angiosarcoma discovered after a two months history of a painful lesion followed by hematoma and spontaneous bleeding. Chemotherapy with paclitaxel and hemostatic radiotherapy were inefficient and he died seven months after the first symptoms. It is essential to use the vascular markers, such as CD34, CD31, ERG and FLI1, for a correct histological diagnosis, which remains difficult because it displays a wide range of morphological appearances and multiple patterns may be present in the s...
Source: Rare Tumors - October 5, 2016 Category: Cancer & Oncology Source Type: research

Rare aggressive behavior of MDM2-amplified retroperitoneal dedifferentiated liposarcoma, with brain, lung and subcutaneous metastases
We describe a case of retroperitoneal dedifferentiated liposarcoma in a 63-year-old male, who showedMDM2 amplification with fluorescencein situ hybridization, which displayed unusually aggressive behavior, with brain, lung and subcutaneous soft tissue metastases. As previous reports of metastatic liposarcoma have largely grouped DDL in with other (genetically and clinically distinct) liposarcoma subtypes, we highlight and discuss the rare occurrence of brain metastasis inMDM2-amplified retroperitoneal liposarcoma. (Source: Rare Tumors)
Source: Rare Tumors - October 5, 2016 Category: Cancer & Oncology Source Type: research