Team Approach: Extremity Soft Tissue Sarcoma.

¼ Synovial sarcoma is a soft tissue sarcoma that most commonly presents in the extremity in a periarticular location.¼ As the history and physical examination of patients with synovial sarcoma can overlap considerably with those of patients with non-oncologic orthopedic conditions, it is important that orthopedic surgeons maintain a high level of suspicion when caring for patients with extremity masses.¼ Soft tissue sarcomas are best treated using a team approach. Early recognition and referral to a multidisciplinary sarcoma team are crucial to ensure the best clinical outcome for the patient.

Inflammatory Hibernoma of the Renal Hilum Mimicking a Renal Pelvis Tumor.

We report on an enhancing, heterogenous renal pelvis mass growing over 2 years which was found to be a benign hibernoma with inflammatory and lipomatous features originating from the renal hilum. To our knowledge, this is the first case reported on a hibernoma compressing on the renal pelvis and second case of a hibernoma with the inflammatory variant.

Chondromyxoid Fibroma of the Sacral Spine.

Background and Importance. Chondromyxoid fibromas are rare tumors of cartilaginous origin typically found in long bones. They usually present during the second and third decades of life. In this case report, we describe the case of a 60-year-old male who presented with back pain and was later found to have a sacral mass. Further immunohistochemical analysis determined that the mass was a chondromyxoid fibroma. Clinical Presentation. A 60-year-old male presented with worsening lower back pain over the course of 2 years. Magnetic resonance imaging demonstrated a destructive mass centered at S4 with heterogeneous enhancement. The patient underwent an S3-to-Co1 laminectomy for gross total resection of the tumor. Histology demonstrated a biphasic, lobulated appearance, characteristic of a chondromyxoid fibroma. The tissue stained faintly positive for multicytokeratin, and it was negative for markers S-100 protein and brachyury, indicating that it was not a chordoma. Conclusion. Only 8 cases have documented sacral chondromyxoid fibroma. As these are exceedingly rare tumors, especially within the sacral spine, the differential diagnosis includes other malignant tumors more likely to be found in the spine, particularly chordomas and chondrosarcomas. Immunohistochemistry and histology are essential in making a definitive diagnosis. This case provides a comprehensive illustration of the clinical presentation, radiographic findings, and immunohistochemistry of sacral chondromyxoid fibroma.

A rare case of primary dermal clear cell sarcoma with focal epidermotropism: An entity difficult to distinguish from melanoma.

Clear cell sarcoma (CCS) is an uncommon soft-tissue sarcoma that only rarely arises within the dermis. It is challenging to distinguish dermal CCS from nodular, primary dermal, or metastatic melanoma, as they share morphologic features and immunoprofiles. We describe a dermal CCS in a 25-year-old man with a cutaneous groin mass. The lesion was initially diagnosed as melanoma, likely metastatic. On consultation, in addition to a melanoma-like tumor in the dermis, we identified focal infiltration of tumor cells into the overlying epidermis (epidermotropism), resembling primary nodular or metastatic melanoma. Given the patient's age and absence of a history of primary melanoma, fluorescence in situ hybridization (FISH) was performed, which revealed separation of the 5' and 3' EWSR1 probe signals on chromosome 22q12, prompting a diagnosis of CCS. Our case highlights the histopathological, immunohistochemical, and ultrastructural similarities between CCS and melanoma, and the consequent potential for major diagnostic confusion. In such cases, FISH analysis remains the key to diagnosis. CCS should be considered in patients with a melanoma-like tumor in the dermis or subcutaneous tissue without epidermal (or with minimal) involvement, or prior to diagnosing metastatic melanoma in the absence of a known history of primary melanoma, especially in young individuals.

#EBUSTwitter: Novel Use of Social Media for Conception, Coordination, and Completion of an International, Multicenter Pathology Study.

CONTEXT.­: Social media sites are increasingly used for education, networking, and rapid dissemination of medical information, but their utility for facilitating research has remained largely untapped. OBJECTIVE.­: To describe in detail our experience using a social media platform (Twitter) for the successful initiation, coordination, and completion of an international, multi-institution pathology research study. DESIGN.­: Following a tweet describing a hitherto-unreported biopsy-related histologic finding in a mediastinal lymph node following endobronchial ultrasound-guided transbronchial needle aspiration, a tweet was posted to invite pathologists to participate in a validation study. Twitter's direct messaging feature was used to create a group to facilitate communication among participating pathologists. Contributing pathologists reviewed consecutive cases of mediastinal lymph node resection following endobronchial ultrasound-guided transbronchial needle aspiration and examined them specifically for biopsy site changes. Data spreadsheets containing deidentified data and digital photomicrographs of suspected biopsy site changes were submitted via an online file hosting service for central review by 5 pathologists from different institutions. RESULTS.­: A total of 24 pathologists from 14 institutions in 5 countries participated in the study within 143 days of study conception, and a total of 297 cases were collected and analyzed. The time interval between study conception and acceptance of the manuscript for publication was 346 days. CONCLUSIONS.­: To our knowledge, this is the first time that a social media platform has been used to generate a research idea based on a tweet, recruit coinvestigators publicly, communicate with collaborating pathologists, and successfully complete a pathology study.

Primary adrenal leiomyosarcoma with inferior vena cava extension in a 70-year-old man.

Primary adrenal leiomyosarcoma, a malignant soft tissue tumour originating from the smooth muscle of the inferior vena cava and adrenal vein, is rarely described in the literature. Cases are often diagnosed at an advanced stage as the tumour is not hormone-producing. We describe a 70-year-old man who presented with lower extremity swelling and abdominal varices and was subsequently found to have a large adrenal mass on imaging. Our case is among the few reported primary adrenal leiomyosarcomas in which a CT-guided biopsy was used to aid in diagnosis.

Glomangiomatosis: a case report.

Glomangiomatosis is a benign vascular variant of a glomus tumor. The lesion represents only 5% of glomus tumors with unusual or atypical features and even fewer glomus tumors with typical features. The lesions are most commonly located in the distal extremities and are multiple, deep, extensive, and often pain producing. They develop from small arteriovenous anastamoses and are most often identified in young adults. The lesions may recur. We present a case of a 33-year-old male who presented clinically with multiple slowly enlarging masses of the leg over a 5-year period, of which one caused significant pain. One of the lesions was reported to be present at birth. Imaging studies were performed and were suggestive of neurofibromatosis. Biopsies from multiple lesions led to an eventual diagnosis of multiple glomangiomatosis.

Leiomyoma of deep soft tissue mimicking calcific myonecrosis.

Leiomyoma of deep soft tissue is an unusual entity reported in less than 60 cases in the English literature. The lesion is now accepted as a rare neoplasm. Leiomyomata of deep soft tissue have been divided into 2 groups, those occurring primarily in women in the retroperitoneum, histologically similar to leiomyomata of the uterus, and those occurring equally in both sexes in the somatic deep soft tissues. Irrespective of location, these lesions can demonstrate calcification, and even less commonly ossification. We report a unique case of a leiomyoma of deep soft tissue that mimicked the clinical and radiographic features of calcific myonecrosis, also a rare mass forming soft-tissue lesion. Clinical and radiographic information are often critical in the diagnosis of soft-tissue lesions; however, this case demonstrates that a lesion can deviate from the standard clinical and radiographic interpretations most commonly attributed to it.

Rhabdoid Tumor Predisposition Syndrome and Pleuropulmonary Blastoma Syndrome.

In recent years, hereditary cancer syndromes have developed greater interest in the scientific community. Two such syndromes, rhabdoid tumor syndrome and pleuropulmonary blastoma (DICER1) syndrome, have appeared increasingly in the literature. This review will discuss these two syndromes in terms of clinical parameters, associated tumors, and genetic associations.

Multiple Giant Cell Tumors of Tendon Sheath Found within a Single Digit of a 9-Year-Old.

Giant cell tumor of tendon sheath is one of the most common soft tissue tumors of the hand. These tumors typically occur in the third or fourth decade of life and present as solitary nodules on a single digit. Currently, the greatest reported number of lesions found within a single digit is five. Although uncommon, giant cell tumor of tendon sheath does occur in the pediatric population. Herein we present a report of a rare case of GCTTS in a child in which seven lesions were identified within a single digit-the greatest number of lesions within a single digit reported to date.

Abdominal wall endometriosis: a rarely anticipated diagnosis: a 16-year experience and brief literature review.

OBJECTIVE: To evaluate cases of endometriosis of the abdominal wall to determine whether the diagnosis was anticipated preoperatively and whether or not the patient had undergone prior surgery. STUDY DESIGN: This was a retrospective review of records of patients undergoing surgical resection of abdominal wall endometriosis from 1996-2011. RESULTS: Nine cases were identified. For the 8 cases with a recorded preoperative diagnosis, the clinical diagnosis of endometriosis was recorded preoperatively in only 25% of cases. The most common preoperative diagnosis was neoplasia. CONCLUSION: Abdominal wall endometriosis remains a difficult diagnosis to make preoperatively. Awareness among gynecologists and consideration of the diagnosis in the differential will potentially decrease the number of cases suspected of malignancy and may alleviate some stress on the patients' part, due to the possibility of a benign diagnosis.

Massive localized lymphedema of the vulva: report of a case and review of the literature.

BACKGROUND: Massive localized lymphedema (MLL) is a recently described entity associated with obesity, most often on the thighs. It rarely occurs on the vulva, and it may pose diagnostic difficulty for both clinician and pathologist in this setting. CASE: An obese 55-year-old woman underwent excision of bilateral polypoid vulvar masses. Histology confirmed the diagnosis of MLL. CONCLUSIONS: Although MLL is not common on the vulva, with the increasing incidence of obesity in the population, more cases can be anticipated, and an awareness of this lesion is important.

Giant cell tumor of bone with secondary aneurysmal bone cyst-like change producing ß-human chorionic gonadotropin.

Giant cell tumor of bone is a benign, locally aggressive neoplasm that is composed of sheets of neoplastic mononuclear cells interspersed amongst non-neoplastic, uniformly distributed, osteoclast-like giant cells. They represent approximately 4-5% of primary bone tumors. Rarely, bone tumors have been noted to produce human chorionic gonadotropin, a finding most often reported in osteosarcoma. We present the case of a young woman who presented with a low-level human chorionic gonadotropin level which, after resection of her recurrent giant cell tumor of bone with secondary aneurysmal bone cyst-like change, became undetectable in her blood. Furthermore, cells within the aneurysmal bone cyst component were immunohistochemically positive for ß-human chorionic gonadotropin. This is the first report of such a finding in the literature.

Pseudoangiomatous stromal hyperplasia of the vulva presenting as a polypoid vulvar lesion: a lesion associated with anogenital mammary-like tissue-report of a case and review of the literature.

OBJECTIVE: The study aimed to report the case of a patient with a polypoid vulvar lesion clinically thought to be an aggressive angiomyxoma. MATERIALS AND METHODS: On examination, a 4.0 × 2.0 × 1.5-cm polypoid lesion was seen on the right labium majus. RESULTS: Histopathological findings of the excised mass were consistent with pseudoangiomatous stromal hyperplasia of the vulva CONCLUSIONS: Pseudoangiomatous stromal hyperplasia is a lesion of breast tissue but can occur in anogenital mammary-like glands. It must be distinguished from low-grade angiosarcoma.

Significance of divergent expression of prostaglandin EP4 and EP3 receptors in human prostate cancer.

PGE2 has been implicated in prostate cancer tumorigenesis. We hypothesized that abnormal prostaglandin receptor (EPR) expression may contribute to prostate cancer growth. Twenty-six archived radical prostatectomy specimens were evaluated by immunohistochemistry (IHC) and Western blotting for the expression of EP1, EP2, EP3, and EP4. As a corollary, EPR expression in one normal (PZ-HPV7) and four prostate cancer cell lines (CA-HPV10, LNCaP, PC3, and Du145) were assessed by Western blotting. Prostate cancer and normal cell growth were compared in vitro after EPR blockade, siRNA EPR knockdown, or overexpression. EP1, EP2, EP3, and EP4 receptors were detected by IHC in all areas of benign tissue within the clinical prostate cancer specimens. In areas of prostate cancer, EP4 and EP2 were overexpressed in 85% (22 of 26) and 75% (18 of 24) and EP3 expression was reduced in all (26 of 26, 100%) specimens (P < 0.05 vs. benign tissue). EP1 showed no specific differential expression pattern. Increased EP4 and reduced EP3 was confirmed by Western blotting in fresh clinical specimens and in prostate cancer cell lines (CA-HPV10, LNCaP, PC3, and Du145) compared with the normal prostate cell line (PZ-HPV7). EP2 and EP4 siRNA knockdown resulted in reduced in vitro growth and metastasis-related gene expression (MMP9 and Runx2) of prostate cancer lines, and in vitro migration was inhibited by EP4 antagonists. As a corollary, EP3-overexpressing PC3 cells displayed impaired growth in vitro. Human prostate cancer is associated with EP4 and EP2 overexpression and reduced EP3 expression. These data suggest that targeting specific EPR may represent a novel therapeutic approach for prostate cancer.

Epithelioid sarcoma: an unusual presentation in the distal phalanx of the toe.

Epithelioid sarcoma is rare soft tissue sarcoma first described by Enzinger in 1970. The classic variant often occurs in the deep or superficial soft tissue of the distal upper extremities of individuals between the ages of 10 and 35. Due to the tumor's benign clinical presentation, infrequent occurrence, and histological similarities with other disease processes, diagnosing epithelioid sarcoma in its early stages has become extremely difficult. We report a rare case of epithelioid sarcoma of the toe with bone metastasis, as well as the clinical, pathological, and radiological difficulties in correctly diagnosing epithelioid sarcoma.

Vaginal blue nevus: report of a case and review of the literature.

OBJECTIVE: : Blue nevi are extremely rare in the vaginal canal and are suspicious for melanoma, especially when multiple lesions are present. We describe a woman with multiple blue nevi of the vagina. DESIGN: : We describe 1 case of multiple blue nevi of the vagina. RESULTS: : A 37-year-old woman, status after therapy for serous carcinoma of the ovary, presented with multiple blue to black macular lesions present throughout the vagina. Two of the lesions were examined by biopsy and demonstrated dendritic melanocytes. The patient has been closely followed, and the lesions remain unchanged. CONCLUSIONS: : Multiple vaginal blue nevi are an important differential diagnostic consideration for melanoma of the vagina. These lesions, however, are benign and require only clinical follow-up. Excision is unnecessary.

Mesenchymal lesions of the vulva.

Mesenchymal lesions of the vulva may be either regionally specific to the vulva or soft tissue neoplasms that can occur in multiple locations on the body. They may have overlapping histological and immunohistochemical features and, in routine practice, are not likely to be encountered, adding to the diagnostic difficulty. A review of mesenchymal vulvar lesions is presented.