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A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia.

Ofikwu G, Mani VR, Rajabalan A, Adu A, Ahmed L, Vega D - Case Rep Surg (2015)

Bottom Line: Of interest, DIPNECH in our patient was associated with metastatic pulmonary carcinoids, papillary carcinoma of the left breast, oncocytoma and angiomyolipoma of her left kidney, and cortical nodules suggestive of tuberous sclerosis.She had video assisted thoracoscopic surgery (VATS), modified radical mastectomy with reconstruction, and radical nephrectomy.She is currently symptom-free most of the time with over two years of follow-up.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Columbia University College of Physicians and Surgeons at Harlem Hospital, New York, NY 10037, USA.

ABSTRACT
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare clinical condition with only about 100 cases reported in the literature. It is characterized by primary hyperplasia of pulmonary neuroendocrine cells (PNECs) which are specialized epithelial cells located throughout the entire respiratory tract, from the trachea to the terminal airways. DIPNECH appears in various forms that include diffuse proliferation of scattered neuroendocrine cells, small nodules, or a linear proliferation. It is usually seen in middle-aged, nonsmoking women with symptoms of cough, dyspnea, and wheezing. We present a 45-year-old, nonsmoking woman who presented with symptoms of DIPNECH associated with bilateral pulmonary nodules and left hilar adenopathy. Of interest, DIPNECH in our patient was associated with metastatic pulmonary carcinoids, papillary carcinoma of the left breast, oncocytoma and angiomyolipoma of her left kidney, and cortical nodules suggestive of tuberous sclerosis. She had video assisted thoracoscopic surgery (VATS), modified radical mastectomy with reconstruction, and radical nephrectomy. She is currently symptom-free most of the time with over two years of follow-up.

No MeSH data available.


Related in: MedlinePlus

Multidisciplinary review of PET CT.
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Related In: Results  -  Collection


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fig4: Multidisciplinary review of PET CT.

Mentions: A multidisciplinary review of the PET/CT (Figure 4) raised the possibility that the lung lesion was metastatic renal cell or breast cancer versus unknown primary. It was decided that the best way to obtain a diagnosis was to operate and perform wedge resection of the largest lung nodules. She had bilateral video assisted thoracoscopic surgery (VATS) with resection of the right chest 2 cm hilar lymph node, wedge resections of right middle lobe (RML), and excision of right lower lobe (RLL) nodules. The patient did well postoperatively and was discharged home on postoperative day three. The pathology of the left hilar lymph node was positive for carcinoid tumor. The right middle and lower lobe nodules showed carcinoid tumor as well. Also seen within the lung were multiple carcinoid tumorlets, diffuse neuroendocrine cell hyperplasia, and bronchiectasis. Microscopically, the tumor in both the lungs and lymph nodes showed organoid and trabecular growth with salt and pepper chromatin. The highest mitotic count was 1 per 10 high power fields (HPF) and no necrosis was seen. These findings are consistent with typical carcinoid tumors. Given the history of breast cancer and a renal tumor, immunohistochemistry (IHC) studies were performed. IHC staining was positive for cytokeratin (weak), cytokeratin 7, synaptophysin, chromogranin, and TTF1 (weak) but negative for napsin, PAX8, GFDFP15, ER, PR, mammaglobin, and CDX2.


A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia.

Ofikwu G, Mani VR, Rajabalan A, Adu A, Ahmed L, Vega D - Case Rep Surg (2015)

Multidisciplinary review of PET CT.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4644819&req=5

fig4: Multidisciplinary review of PET CT.
Mentions: A multidisciplinary review of the PET/CT (Figure 4) raised the possibility that the lung lesion was metastatic renal cell or breast cancer versus unknown primary. It was decided that the best way to obtain a diagnosis was to operate and perform wedge resection of the largest lung nodules. She had bilateral video assisted thoracoscopic surgery (VATS) with resection of the right chest 2 cm hilar lymph node, wedge resections of right middle lobe (RML), and excision of right lower lobe (RLL) nodules. The patient did well postoperatively and was discharged home on postoperative day three. The pathology of the left hilar lymph node was positive for carcinoid tumor. The right middle and lower lobe nodules showed carcinoid tumor as well. Also seen within the lung were multiple carcinoid tumorlets, diffuse neuroendocrine cell hyperplasia, and bronchiectasis. Microscopically, the tumor in both the lungs and lymph nodes showed organoid and trabecular growth with salt and pepper chromatin. The highest mitotic count was 1 per 10 high power fields (HPF) and no necrosis was seen. These findings are consistent with typical carcinoid tumors. Given the history of breast cancer and a renal tumor, immunohistochemistry (IHC) studies were performed. IHC staining was positive for cytokeratin (weak), cytokeratin 7, synaptophysin, chromogranin, and TTF1 (weak) but negative for napsin, PAX8, GFDFP15, ER, PR, mammaglobin, and CDX2.

Bottom Line: Of interest, DIPNECH in our patient was associated with metastatic pulmonary carcinoids, papillary carcinoma of the left breast, oncocytoma and angiomyolipoma of her left kidney, and cortical nodules suggestive of tuberous sclerosis.She had video assisted thoracoscopic surgery (VATS), modified radical mastectomy with reconstruction, and radical nephrectomy.She is currently symptom-free most of the time with over two years of follow-up.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Columbia University College of Physicians and Surgeons at Harlem Hospital, New York, NY 10037, USA.

ABSTRACT
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare clinical condition with only about 100 cases reported in the literature. It is characterized by primary hyperplasia of pulmonary neuroendocrine cells (PNECs) which are specialized epithelial cells located throughout the entire respiratory tract, from the trachea to the terminal airways. DIPNECH appears in various forms that include diffuse proliferation of scattered neuroendocrine cells, small nodules, or a linear proliferation. It is usually seen in middle-aged, nonsmoking women with symptoms of cough, dyspnea, and wheezing. We present a 45-year-old, nonsmoking woman who presented with symptoms of DIPNECH associated with bilateral pulmonary nodules and left hilar adenopathy. Of interest, DIPNECH in our patient was associated with metastatic pulmonary carcinoids, papillary carcinoma of the left breast, oncocytoma and angiomyolipoma of her left kidney, and cortical nodules suggestive of tuberous sclerosis. She had video assisted thoracoscopic surgery (VATS), modified radical mastectomy with reconstruction, and radical nephrectomy. She is currently symptom-free most of the time with over two years of follow-up.

No MeSH data available.


Related in: MedlinePlus