Primary malignant melanoma of the genitourinary tract: case series of a rare form of primary mucosal melanoma.

Primary malignant melanoma of the genitourinary tract is extremely rare. We present two such cases in elderly Caucasian females. An 81-year-old female with urinary retention and polypoid urinary bladder mass and a 72-year-old female with gross hematuria and urethral caruncle. After thorough evaluation, they were both eventually diagnosed with primary urogenital melanoma (SOX10 and MART1-positive in tumor cells). In both cases, the presence of melanoma-in-situ and absence of primary melanoma in other sites were consistent with primary urogenital melanoma. Immunotherapy with PD-1 inhibitors and use of neoadjuvant and adjuvant treatment are promising, as treatment guidelines remain unclear and overall survival is low. Additional clinical reporting of primary urogenital melanomas can help in better understanding and ultimately treating it.

Primary melanomas of the bladder and urinary tract are rare and usually deadly. They represent only 0.2% of all melanomas, including melanomas of skin. They can be difficult to diagnose and treat due to how rare they are and the lack of clear treatment guidelines. We present two cases of elderly Caucasian women who were unexpectedly diagnosed with primary melanoma cancers of the bladder and urinary tract after having surgery and analyzing tissue that was removed. Both tissue samples had features specific to melanoma and there was no cancer in any other organ, thus making them primary melanomas of the bladder and urinary tract. Current treatment approaches with surgery and chemotherapy have not improved the survival outcomes and prognosis associated with this disease, but treatment before and after surgery as well as cancer treatments that harness the person's own immune system are promising. By reporting additional clinical experiences of this often fatal disease, we hope it can be better understood and appropriately managed in the future.

Thirteen Synchronous Multifocal Calcifying Epithelial Odontogenic Tumors (CEOT): Case Report and Review of the Literature.

BACKGROUND: Calcifying epithelial odontogenic tumor (CEOT, Pindborg tumor) is a rare, benign, locally aggressive neoplasm of the jaws that accounts for approximately 1% of all odontogenic tumors. It was first defined by Pindborg in 1955 and has been reported approximately 350 times in the literature; 7 reported multiple (up to 4) synchronous lesions. MATERIALS AND METHODS: We report an individual with the largest number of CEOTs reported to date and provide a literature review of multifocal CEOT cases. RESULTS: A 30-year-old male presented to the Oral and Maxillofacial Surgery Department of the West Virginia University School of Dentistry (WVU SoD) to extract multiple impacted teeth previous to construction of a complete denture. A pantograph showed 15 impacted teeth, almost all associated with well-demarcated cyst-like radiolucencies, some with small, ill-defined radiopaque flecks. Microscopically, the lesions showed sheets and strands of polygonal epithelial cells with eosinophilic cytoplasm. Spread throughout the epithelium and connective tissue were small, spherical, amorphous, pale purplish calcifications. Each lesion was similar and consistent with a diagnosis of CEOT. CONCLUSION: We report a patient with 13 independent CEOTs scattered throughout all quadrants. This case represents the largest number of Pindborg tumors or any other type of odontogenic tumor yet reported in a single individual.

Outside the Thorax: Doege-Potter Syndrome Presenting as a Retroperitoneal Abdominal Mass.

OBJECTIVE: We present a case of refractory hypoglycemia, weight loss, and retroperitoneal solitary fibrous tumor. Case report. A 68-year-old female presented with symptomatic hypoglycemia, weight loss, and abdominal mass identified on CT scan of the abdomen. Blood work during symptomatic hypoglycemia was consistent with an IGF-2-producing tumor. The abdominal mass pathology was consistent with solitary fibrous tumor surrounding the adrenal gland, and resection resulted in complete resolution of hypoglycemia. Discussion. Understanding the biochemical mechanisms behind glucose regulation is necessary to diagnose and adequately treat Doege-Potter syndrome, a paraneoplastic syndrome observed in patients with solitary fibrous tumors. Solitary fibrous tumors can be characterized by specific histologic and immunohistochemical studies. CONCLUSION: This report describes the clinical workup of a patient presenting with hypoglycemia and a retroperitoneal tumor. This case is unique because of its presentation with severe, refractory hypoglycemia and the tumor's location in the retroperitoneum, given the majority of solitary fibrous tumors are found in the lungs originating from the pleura.

Educational Case: Gallstones, Cholelithiasis, and Cholecystitis.

The following fictional case is intended as a learning tool within the Pathology Competencies for Medical Education (PCME), a set of national standards for teaching pathology. These are divided into three basic competencies: Disease Mechanisms and Processes, Organ System Pathology, and Diagnostic Medicine and Therapeutic Pathology. For additional information, and a full list of learning objectives for all three competencies, see http://journals.sagepub.com/doi/10.1177/2374289517715040.1.

Human Papillomavirus Infection of the Skin.

CONTEXT: - Human papillomavirus is implicated in the pathogenesis of benign and malignant neoplasms of the skin. OBJECTIVE: - To review the role of human papillomavirus in the development of malignancies and their precursor lesions in skin. DATA SOURCES: - The study comprised a review of the literature. CONCLUSIONS: - The use of low-grade squamous intraepithelial lesion and high-grade squamous intraepithelial lesion terminology brings order and simplicity to these lesions, correlates with the current understanding of the biology of human papillomavirus infections, and helps to promote accurate diagnosis of and appropriate treatment for these lesions.

Unicentric epithelioid hemangioendothelioma of the calcaneus: a case report and review of literature.

BACKGROUND: This review of the literature combined with a clinical case will allow the illustration of a favorable outcome of this variable low grade malignancy, display a role for limb salvage surgery with intralesional treatment, and offer a clinical example of epithelioid hemangioendothelioma, a rare malignancy. CASE PRESENTATION: The case report presents a case of solitary epithelioid hemangioendothelioma (EHE) of the calcaneus in a 60-year-old male. Primary vascular tumors of the bone are rare; however, EHE is one of the most common primary malignant vascular tumors to occur in bone. A review of the literature found few cases that involved the calcaneus; those cases found that involved the calcaneus were either part of a multifocal or metastatic disease process. Our case presents a 45-month clinical follow-up of solitary EHE in the calcaneus treated with surgical excision by curettage and cementing. CONCLUSION: This case has clinical follow-up greater than 2 years post-operatively and could be a guide for treatment of a rare disorder with a substantial paucity of literature.

Retroauricular Pleomorphic Adenoma Arising from Heterotopic Salivary Gland Tissue.

A 38-year-old woman is described who presented with a slowly growing mass on the posterior aspect of the left ear. Excision and histopathologic evaluation revealed a pleomorphic adenoma (PA) originating from heterotopic salivary gland tissue. Many authors have presented cases of PAs originating from ceruminous glands in the external auditory canal or of so-called chondroid syringoma originating from apocrine and eccrine sweat glands. This is the only case in the recent literature of a PA originating from a heterotopic rest of salivary gland tissue in the retroauricular region. The 3 main sources of PAs, their embryologic derivation, and treatment are described.

IgA Nephropathy in a Patient Presenting with Pseudotumor Cerebri.

IgA nephropathy is the most common glomerulonephritis worldwide and typically has minimal signs for chronicity in histopathology at the time of initial presentation. Pseudotumor cerebri (PTC) is characterized by increased intracranial pressure in the absence of any intracranial lesions, inflammation, or obstruction. PTC has been reported in renal transplant and dialysis patients, but we are unaware of any reports of pseudotumor cerebri in patients with IgA nephropathy. We report a case of a young female who presented with signs and symptoms of pseudotumor cerebri and was subsequently diagnosed with IgA nephropathy and end-stage renal disease. To our knowledge this is the first report of IgA nephropathy presenting as end-stage renal disease in a patient who presented with pseudotumor cerebri.

Myeloid Sarcoma of the Bladder in the Setting of Refractory Anemia with Excess Blasts-2 (RAEB-2).

Myeloid sarcoma is an extramedullary tumor consisting of immature hematopoietic cells of granulocytic or monocytic differentiation. While rare, it can be seen in a variety of clinical settings and is most commonly associated with acute myeloid leukemia (AML), myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN). We present a rare case of myeloid sarcoma occurring in the bladder of a 56 year old male. Myeloid sarcoma may be difficult to recognize due to its rarity and clinical and morphologic similarity to many other conditions; however, swift diagnosis is necessary as it is considered equivalent to AML. Prognostic indicators for myeloid sarcoma have not been well established, but survival may be improved by undergoing chemotherapy designed to treat AML.

Bullous Henoch-Schönlein purpura in children.

Henoch-Schönlein purpura (HSP) is a systemic vasculitis that is common in the pediatric population and often presents with the classical triad of palpable purpura, arthralgia, and abdominal pain. We describe a case of HSP in a 14-year-old adolescent girl who presented with atypical features of painful hemorrhagic bullae. The patient was treated with high-dose steroids, dapsone, and supportive therapy with remarkable improvement.

Perirenal extra-adrenal myelolipoma.

Myelolipomas are rare tumors consisting of both adipose and hematopoietic tissue and are typically found within the adrenal gland. Extra-adrenal involvement is rare, especially those tumors involving the perirenal space and collecting system. We report a case of a patient with an incidentally discovered perirenal mass that was initially concerning for a retroperitoneal liposarcoma. Following surgical resection and pathological analysis, the lesion was found to be an extra-adrenal myelolipoma. This case report and review of the literature demonstrates the importance of the proper work-up and management of perirenal lipoma variants while addressing the issues of tissue biopsy, surgical intervention, and pre- and post-operative surveillance.