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Primary neuroendocrine tumors of the larynx are rare, with moderately differentiated neuroendocrine carcinoma (MDNC) being the most frequent histologic type. We report a MDNC in a 57-year-old gentleman with an enlarging right-sided neck mass. Flexible fiberoptic exam revealed a right arytenoid lesion. Histology from excisional biopsy was concerning for medullary thyroid carcinoma (MTC) versus NET of the larynx. Immunohistochemistry was diffusely positive for calcitonin and CEA and focally positive for TTF-1. Serum calcitonin was elevated. Thyroid ultrasound was unremarkable. The patient underwent laryngectomy, thyroidectomy, and neck dissection. Pathology showed neuroendocrine carcinoma of right arytenoid with positive cervical lymph nodes. A 4 mm deposit of NET was present in right thyroid with adjacent intravascular tumor consistent with thyroidal metastasis from a primary laryngeal NET (MDNC). MDNC and MTC can be microscopically indistinguishable. Both tumors can stain positively for calcitonin and CEA. TTF-1 staining has been useful to help distinguish these tumors as it is strongly and diffusely positive in MTC, but usually negative (or only focally positive) in MDNC. We report the fourth case of primary neuroendocrine carcinoma of the larynx associated with elevated serum calcitonin level and the first such case associated with metastasis to the thyroid.

BackgroundRetinoic acid-inducible gene I (RIG-I) is an important regulator of virus-induced antiviral interferons (IFNs) and proinflammatory cytokines which participate in clearing viral infections. Cigarette smoke (CS) exposure increases the frequency and severity of respiratory tract infections.MethodsWe generated a RIG-I transgenic (TG) mouse strain that expresses the RIG-I gene product under the control of the human lung specific surfactant protein C promoter. We compared the mortality and host immune responses of RIG-I TG mice and their litter-matched wild type (WT) mice following challenge with influenza A virus (IAV).ResultsRIG-I overexpression increased survival of IAV-infected mice. CS exposure increased mortality in WT mice infected with IAV. Remarkably, the effect of RIG-I overexpression on survival during IAV infection was enhanced in CS-exposed animals. CS-exposed IAV-infected WT mice had a suppressed innate response profile in the lung compared to sham-exposed IAV-infected WT mice in terms of the protein concentration, total cell count and inflammatory cell composition in the bronchoalveolar lavage fluid. RIG-I overexpression restored the innate immune response in CS-exposed mice to that seen in sham-exposed WT mice during IAV infection, and is likely responsible for enhanced survival in RIG-I TG mice as restoration preceded death of the animals.ConclusionsOur results demonstrate that RIG-I overexpression in mice is protective for CS enhanced susceptibility of smokers to influenza infection, and that CS mediated RIG-I suppression may be partially responsible for the increased morbidity and mortality of the mice exposed to IAV. Thus, optimizing the RIG-I response may be an important treatment strategy for CS-enhanced lung infections, particularly those due to IAV.

Uterine carcinosarcomas are uncommon, aggressive neoplasms usually afflicting postmenopausal women. Histologically, they are characterized by the presence of both malignant epithelial and stromal cells. The latter may be either homologous or heterologous in nature. The cytologic diagnosis of carcinosarcoma via fine‐needle aspiration (FNA) was previously described for primary tumors arising in the parotid gland, breast, lung, and ovary. Although the diagnosis of uterine carcinosarcoma via cervicovaginal, endometrial, and peritoneal fluid cytology has been described, the FNA cytology diagnosis of recurrent uterine carcinosarcoma has only been rarely described. We present a case of recurrent uterine carcinosarcoma in a 59‐yr‐old woman, diagnosed by ultrasound‐guided FNA cytology. Cytologic smears showed a biphasic neoplasm consisting of both malignant columnar epithelial and spindle cells, in a background of tumor diathesis. This case illustrates the diagnostic utility of aspiration cytology in the investigation of recurrent carcinosarcoma in clinically suspicious lesions arising postsurgery. Diagn. Cytopathol. 23:108–113, 2000. © 2000 Wiley‐Liss, Inc.

The fine-needle aspiration biopsy (FNAB) findings in two cases of hemangiopericytoma (HP), arising in the parotid gland and on the inner chest wall, respectively, are reported. Smear preparations in each case showed cytologic features of an undifferentiated spindle-cell neoplasm, whereas a core needle biopsy specimen of the chest wall mass showed a spindle-cell tumor with a "staghornlike" arrangement of endothelium-lined vascular channels. Immunostains performed on this core biopsy, and on the surgical resection specimens in both cases, showed positive staining of tumor cells for vimentin and CD34, with negative staining for a variety of smooth muscle, epithelial, neural, and neuroendocrine markers. Electron microscopy performed in one case further supported the diagnosis of HP. With adequate sampling and appropriate use of ancillary studies, a diagnosis of HP can be reliably suggested on the basis of FNAB and core biopsy of a soft-tissue mass.

Primary cardiac lymphoma (PCL) is defined as a non-Hodgkin lymphoma involving only the heart or pericardium. PCL is extremely rare and often misdiagnosed. We report the case of a healthy 53-year-old male who originally presented with blurred vision secondary to a right intraocular mass. Enucleation of the eye confirmed a ciliary body melanoma and, upon further investigation, the patient was discovered to have a mass in the right atrium suspicious for a myxoma. However, resection of the atrial mass revealed a low-grade B-cell PCL. The occurrence of PCL in an immunocompetent patient being investigated for a visceral malignancy makes this a highly unusual presentation of a rare neoplasm.

Primary pulmonary sarcomas are uncommon neoplasms. Primary fibrosarcoma of the lung is extremely rare, and only 53 cases have been documented in the literature to date. To our knowledge, the diagnosis of primary lung fibrosarcoma by fine-needle aspiration cytology has never been reported. We report a case of pulmonary fibrosarcoma diagnosed by fine-needle aspiration cytology and core biopsy. The neoplasm consisted of interweaving fascicles of minimally atypical spindle cells with slender nuclei and scant cytoplasm. Positive immunohistochemistry for vimentin along with nonreactivity of tumor cells for keratin, S100 protein, desmin, α-smooth muscle actin, and CD34 supported the the diagnosis. The diagnosis was later confirmed by histologic and ultrastructural findings following lobectomy. A meticulous clinical search for a possible primary neoplasm elsewhere was unsuccessful, and lung was established as the primary site. Fine-needle aspiration cytology and core biopsy are reliable methods for establishing a diagnosis of fibrosarcoma.