Constantine A. Axiotis

An In-Depth Look at Leydig Cell Tumor of the Testis

Leydig cell tumor (LCT) is a rare tumor of the male testicular interstitium. This article provides an overview of the major pathologic manifestations of LCT of the testis; patient characteristics; clinical, radiologic, and laboratory features; prognosis; and management. LCTs of the testis are frequently hormonally active, leading to either feminizing or virilizing syndromes. The tumor is usually benign, but malignant variants can occur. The pathologic diagnosis of LCT is usually made based on morphologic characteristics of the tumor cells. The significance of Reinke crystals in the diagnosis of LCT both cytologically and histologically is underscored. Pathologists have to be familiar with the diagnostic histopathologic features, immunohistochemical panel of this tumor, and its principal differential diagnoses to prevent tumor misdiagnosis.

Thyroid-type carcinoma of struma ovarii.

Struma ovarii is a monodermal variant of ovarian teratoma. Thyroid-type carcinoma arising in struma ovarii is rare. The most common type is papillary carcinoma, followed by typical follicular carcinoma, and the new entity of follicular carcinoma-highly differentiated follicular carcinoma of ovarian origin; other forms occur rarely. Consensus on the surgical and postoperative treatment of patients with thyroid-type carcinoma arising in struma ovarii has not been reached. Surgical treatment ranges from total abdominal hysterectomy, plus bilateral salpingo-oophorectomy with omentectomy, to conservative surgery, involving unilateral oophorectomy or strumectomy (cystectomy) for fertility preservation. Adjuvant therapy includes external radiotherapy, chemotherapy, thyroidectomy, and radioactive iodine ablation. Thyroid-type carcinomas arising in struma ovarii, especially the newly recognized entity-highly differentiated follicular carcinoma of ovarian origin-have a favorable prognosis.

Phase II Study of Interferon Gamma in Malignant Carcinoid Tumors (E9292): A Trial of the Eastern Cooperative Oncology Group

Purpose: To determine the safety and efficacy of treatment with gamma interferon (IFNγ) in patients with metastatic carcinoid tumor. Patients and methods: 51 patients were enrolled on this Phase II Eastern Cooperative Oncology Group (ECOG) study. Seventy five percent of them had hormonally active tumors. Treatment consisted of IFNγ subcutaneously at a daily dose of 0.1 mg/m2. Patents were evaluated for toxicity weekly for the first month and monthly thereafter; response was determined radiologically every 8 weeks. Results: Patients received treatment with IFNγ for a median of 17.9 weeks (range 2–175). Toxicity was generally mild and expected: 61% experienced noninfected fever and 21% developed granulocytopenia. Three patients (6%) had a partial response; there were no complete responses. Median time to progression was 5.5 months (95% confidence interval 3.9–11.1). The 1-year progression free rate was 28% (13.4–43.4%). Median survival was 42 months, with a 1-year survival rate of 67% (53.3–80%). Discussion: This Phase II study demonstrated that therapy with IFNγ in patients with metastatic carcinoid tumor was well-tolerated, but did not produce significant antitumor effects. The overall results were somewhat comparable to those previously seen with alpha interferons as well as cytotoxic drugs.

Plasma cell tumors in HIV-positive patients: Report of a case and review of the literature

Plasma cell tumors show an increased incidence in HIV-positive patients. The cases reported in the literature suggest that plasma cell tumors occur in a younger age group than that encountered in the general population. Pathologically, many of these tumors show a plasmablastic morphology. Plasma cell tumors in HIV-positive patients may present at unusual sites and progress rapidly to involve multiple sites, including the soft tissues and viscera. The prognosis is generally poor. These features may be related to a combination of factors, including immunodeficiency, oncogenic viruses, and altered cytokine milieu in these patients. A case of plasma cell tumor in an HIV-positive patient is presented.

Sebaceous carcinoma of the oral cavity: a case report and review of the literature.

We report the clinical and pathologic features of an intraoral sebaceous carcinoma and review the literature. The intraoral buccal mucosal tumor from a 50-year-old man was a poorly differentiated carcinoma with sebaceous differentiation and expression of androgen receptor. Fordyce granules and a minor salivary gland with a duct were identified in vicinity to the tumor. We discuss the differential diagnosis and histogenesis. The patient underwent surgery and adjuvant radiation therapy.

Overexpression of neural cell adhesion molecule in regenerative muscle fibers in 3-hydroxy-3-methylglutaryl coenzyme: A reductase inhibitor-induced rhabdomyolysis.

Skeletal muscle degeneration is a side effect of cholesterol-lowering hydroxymethylglutaryl coenzyme A reductase inhibitors. The expression of the cell–cell adhesion proteins, neural cell adhesion molecule and neural-cadherin was studied in a case of rhabdomyolysis induced by the hydroxymethylglutaryl coenzyme A reductase inhibitor cerivastatin. Neural cell adhesion molecule and N-cadherin participate in the interactions of muscle cells during skeletal myogenesis. In the adult muscle, neural cell adhesion molecule is restricted to neuromuscular sites but is re-expressed in denervated muscle and in rhabdomyolysis. Our results show expression of neural cell adhesion molecule in regenerative skeletal muscle fibers but not in degenerated or unaffected fibers in cerivastatin-induced rhabdomyolysis. In contrast, N-cadherin was not expressed. The presence of apoptotic cells was studied by a fluorescence-based Tdt-mediated dUTP nick-end labeling in the same sections. Apoptosis was detected in degenerative fibers and inflammatory cells but not in regenerative fibers. We hypothesize that the expression of neural cell adhesion molecule in regenerative fibers may have a protective role against apoptosis during rhabdomyolysis. Cerivastatin-induced rhabdomyolysis appears to have common features with rhabdomyolysis of other causes. The immunohistochemical study of neural cell adhesion molecule can serve as an additional tool in the evaluation of muscle regeneration in rhabdomyolysis.

A retrospective analysis of ten symptomatic multiple myeloma patients with HIV infection: A potential therapeutic effect of HAART in multiple myeloma

The impact of highly active anti-retroviral therapy (HAART) in multiple myeloma (MM) is unknown. Ten HIV+ and 28 HIV-negative patients were retrospectively identified out of 262 cases of MM diagnosed at Kings County Hospital Center since the introduction of HAART in 1996. The HIV+ MM patients on HAART had superior overall survival (OS) (Fisher exact, p=0.008; log-rank, p=0.012) and progression free survival (PFS) (Fisher exact, p=0.007; log-rank, p=0.009) than the HIV-negative MM patients. HAART alone blocked the production of serum M-protein. We propose that HARRT should be explored for the treatment of both HIV+ and HIV-negative MM patients.

Haymaker Gene Expression in Malignant and Normal Gynecologic Tissues

We previously reported that cell lines established from human carcinomas and leukemias/lymphomas expressed high levels of an intracellular membrane-bound protein, Haymaker, whereas cell lines derived from non-malignant connective tissue cells and lymphoid cells expressed low levels of this gene product. To determine whether these findings reflect neoplastic transformation or, alternatively, tissue specificity, we examined by immunohistochemical and molecular methods the expression of Haymaker in gynecologic organs with and without tumor. A highly specific, affinity-purified rabbit polyclonal antibody against a 25-mer Haymaker peptide was used for immunohistochemical staining and morphometric analysis of 85 tissue specimens. Immunohistochemical studies demonstrate, for the first time, that Haymaker protein is highly expressed in epithelial cells of the endometrium of the normal uterus and to a somewhat lesser extent in the mucosa of the normal vagina and cervix, but is poorly expressed or absent in cells of the connective tissue and smooth muscle strata of these organs (p < 0.005). Significant differences in Haymaker expression, as assessed by immunohistochemistry, between malignant and normal gynecologic tissues were not observed (p = 0.27). The expression of Haymaker protein does not appear, therefore, to be a marker of malignant transformation of the epithelium of gynecologic organs but rather distinguishes both normal and malignant epithelial cells from normal connective tissue and smooth muscle cells

Isolated fibromuscular dysplasia of the coronary ostium: a rare cause of sudden death. Case report and review of the literature

We present a unique case of sudden death in a 21-year-old man with history of cocaine use and a solitary fibromuscular dysplastic lesion completely occluding the left coronary artery ostium. We document intimal proliferation of myofibroblasts at the opening of the left coronary ostium without other concomitant lesions. This report discusses the gross and histologic features of the lesion, explores in careful detail the possible etiologies, and gives a comprehensive literature review of isolated coronary ostial fibromuscular dysplasia presenting with sudden death.

Postirradiation Leiomyosarcoma of Rectum Presenting as a Polyp Case Report and Review of the Literature

Radiation-induced leiomyosarcomas of the gastrointestinal tract are rare. Very few cases have been documented to date. The histological similarity to gastrointestinal stromal tumor has raised doubts if many of the cases originally reported to be leiomyosarcoma before the widespread use of CD117 were indeed gastrointestinal stromal tumors. We present a case of post-irradiation leiomyosarcoma presenting as a rectal polyp and review the literature.