RESUMEN
Juxtaglomerular cell tumors and glomus tumors both arise from perivascular mesenchymal cells. Juxtaglomerular cells are specialized renin-secreting myoendocrine cells in the afferent arterioles adjacent to glomeruli, and juxtaglomerular tumors derived from these cells are therefore unique to the kidney. In contrast, glomus tumors have been described at numerous anatomic sites and may show significant morphologic and immunophenotypic overlap with juxtaglomerular tumors when occurring in the kidney. Although ultrastructural studies and immunohistochemistry for renin may distinguish these entities, these diagnostic modalities are often unavailable in routine clinical practice. Herein, we studied the clinicopathologic features of a large series of juxtaglomerular tumors (n = 15) and glomus tumors of the kidney (n = 9) to identify features helpful in their separation, including immunohistochemistry for smooth muscle actin (SMA), CD34, collagen IV, CD117, GATA3, synaptophysin, and renin. Markers such as SMA (juxtaglomerular tumors: 12/13, 92%; glomus tumors: 9/9, 100%), CD34 (juxtaglomerular tumors: 14/14, 100%; glomus tumors: 7/9, 78%), and collagen IV (juxtaglomerular tumors: 5/6, 83%; glomus tumors: 3/3, 100%) were not helpful in separating these entities. In contrast to prior reports, all juxtaglomerular tumors were CD117 negative (0/12, 0%), as were glomus tumors (0/5, 0%). Our results show that juxtaglomerular tumors have a younger age at presentation (median age: 27 years), female predilection, and frequently exhibit diffuse positivity for renin (10/10, 100%) and GATA3 (7/9, 78%), in contrast to glomus tumors (median age: 51 years; renin: 0/6, 0%; GATA3: 0/6, 0%). These findings may be helpful in distinguishing these tumors when they exhibit significant morphologic overlap.
Asunto(s)
Adenoma , Tumor Glómico , Neoplasias Renales , Actinas/análisis , Adenoma/patología , Adulto , Antígenos CD34/análisis , Colágeno Tipo IV/análisis , Femenino , Factor de Transcripción GATA3/análisis , Tumor Glómico/química , Tumor Glómico/diagnóstico , Humanos , Aparato Yuxtaglomerular/metabolismo , Aparato Yuxtaglomerular/patología , Aparato Yuxtaglomerular/ultraestructura , Riñón/patología , Neoplasias Renales/química , Persona de Mediana Edad , Renina/análisis , Renina/metabolismo , Sinaptofisina/análisisRESUMEN
CASE PRESENTATION: A 54-year-old man presented with 6 months' history of dry cough and dyspnea on exertion. He also reported intermittent joint pain and orthopnea. He denied fevers, chills, and rashes. His medical history was significant for rheumatoid arthritis, for which he was taking 20 mg of prednisone daily. He had not been receiving adalimumab or methotrexate for several months. He never smoked and drank alcohol occasionally. Family history was significant for rheumatoid arthritis.
Asunto(s)
Dolor en el Pecho/etiología , Tos/etiología , Disnea/etiología , Esfuerzo Físico , Pleura/diagnóstico por imagen , Pleuresia/complicaciones , Biopsia , Dolor en el Pecho/diagnóstico , Tos/diagnóstico , Diagnóstico Diferencial , Disnea/diagnóstico , Humanos , Masculino , Persona de Mediana Edad , Pleuresia/diagnóstico , Tomografía Computarizada por Rayos X , XantomatosisRESUMEN
BACKGROUND: Granulomatous disease in the thyroid gland has been linked to viral, bacterial and autoimmune etiologies. The most common granulomatous disease of the thyroid is subacute granulomatous thyroiditis, which is presumed to have a viral or post-viral inflammatory cause. Bacterial etiologies include tuberculosis, actinomycosis, and nocardiosis, but are extremely rare. Disseminated actinomycosis and nocardiosis more commonly affect organ-transplant patients with the highest susceptibility within the first year after transplant surgery. CASE: A 45-year-old African American male, who received his third kidney transplant for renal failure secondary to Alport Syndrome, presented with numerous subcutaneous nodules and diffuse muscle pain in the neck. Further workup revealed bilateral nodularity of the thyroid. Fine needle aspiration of these nodules demonstrated suppurative granulomatous thyroiditis. Subsequent right thyroid lobectomy showed granulomatous thyroiditis with filamentous micro-organisms, morphologically resembling Nocardia or Actinomyces. CONCLUSION: Disseminated granulomatous disease presenting in the thyroid is very rare, and typically afflicts immune-compromised patients. The overall clinical, cytologic and histologic picture of this patient strongly points to an infectious etiology, likely Nocardia, in the setting of recent organ transplantation within the last year.
Asunto(s)
Huésped Inmunocomprometido , Nocardiosis/inmunología , Nocardia/inmunología , Glándula Tiroides/inmunología , Nódulo Tiroideo/inmunología , Tiroiditis Subaguda/inmunología , Tiroiditis Supurativa/inmunología , Biopsia con Aguja Fina , Humanos , Fallo Renal Crónico/etiología , Fallo Renal Crónico/cirugía , Trasplante de Riñón/efectos adversos , Masculino , Persona de Mediana Edad , Nefritis Hereditaria/inmunología , Nefritis Hereditaria/fisiopatología , Nocardia/aislamiento & purificación , Nocardiosis/microbiología , Nocardiosis/fisiopatología , Reoperación/efectos adversos , Glándula Tiroides/microbiología , Glándula Tiroides/patología , Glándula Tiroides/cirugía , Nódulo Tiroideo/microbiología , Nódulo Tiroideo/patología , Nódulo Tiroideo/cirugía , Tiroidectomía , Tiroiditis Subaguda/microbiología , Tiroiditis Subaguda/patología , Tiroiditis Subaguda/cirugía , Tiroiditis Supurativa/microbiología , Tiroiditis Supurativa/patología , Tiroiditis Supurativa/cirugía , Resultado del TratamientoRESUMEN
Pulmonary salivary gland-type tumors (SGT) comprise a very small proportion of primary lung neoplasms. The most common tumors among this group are mucoepidermoid carcinoma and adenoid cystic carcinoma. Contrary to the head and neck region, benign SGT such as pleomorphic adenomas are exceedingly rare in the pulmonary system. More recently, 2 additional SGT, namely hyalinizing clear cell carcinoma and salivary duct-like carcinoma were recognized as primary lung tumors expanding the spectrum of SGT that have been described to originate in the tracheobronchial system. Primary pulmonary SGT must be clinically excluded from metastatic salivary gland neoplasms as their morphology is indistinguishable from that of their salivary gland counterparts. Little is known about the clinical behavior and best treatment approach for these unusual tumors. In this review, we provide a comprehensive summary of primary pulmonary SGT with particular emphasis on morphologic characteristics and latest developments in terms of immunohistochemical and molecular techniques.
Asunto(s)
Carcinoma Adenoide Quístico/patología , Carcinoma Mucoepidermoide/patología , Neoplasias Pulmonares/patología , Adenocarcinoma de Células Claras/patología , Adenoma/patología , Adenoma Oxifílico/patología , Adenoma Pleomórfico/patología , Carcinoma de Células Acinares/patología , Humanos , Mioepitelioma/patología , Neoplasias Glandulares y Epiteliales/patologíaRESUMEN
Thymic neuroendocrine carcinomas are the most common mediastinal neuroendocrine tumor. These malignancies are not often diagnosed by fine-needle aspiration (FNA), as they are more commonly diagnosed by biopsy or excision. We describe a case of a FNA of a paratracheal mass from a 38-year-old man who presented with Cushing syndrome. A low-grade neuroendocrine carcinoma with oncocytic features was diagnosed, which was later confirmed by excision of the thymus, anterior mediastinal and paratracheal soft tissue, and lymph nodes. Oncocytic features in these tumors are a rare finding and bring metastatic medullary thyroid carcinomas as well as other metastases into the differential diagnosis. The prognosis of neuroendocrine carcinomas in this location is worse than neuroendocrine carcinomas in other areas, and close follow-up is recommended.
Asunto(s)
Carcinoma Neuroendocrino/patología , Síndrome de Cushing/patología , Timoma/patología , Neoplasias del Timo/patología , Hormona Adrenocorticotrópica/biosíntesis , Adulto , Biopsia con Aguja Fina/métodos , Carcinoma Neuroendocrino/metabolismo , Carcinoma Neuroendocrino/cirugía , Síndrome de Cushing/metabolismo , Diagnóstico Diferencial , Humanos , Masculino , Pronóstico , Timoma/metabolismo , Timoma/cirugía , Neoplasias del Timo/metabolismo , Neoplasias del Timo/cirugíaRESUMEN
We present an adult autopsy case of primary hyperoxaluria type 1. Diagnosis was established with skin biopsy and subsequent genetic analysis one month prior to death. At autopsy, calcium oxalate crystals refringent to polarized light were found systemically. Interestingly, however, calcium oxalate crystals were not identified in the bone. Additionally, we have included a review of the literature for previous autopsy cases, presentations, diagnosis, complications, and treatment of this rare genetic systemic process.