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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

Brain MRI, showing subcortical masses.
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fig3: Brain MRI, showing subcortical masses.

Mentions: A brain MRI was later done because of the association of DIPNECH with MEN1 and tuberous sclerosis with angiomyolipoma of the kidney. MRI of the brain (Figure 3) showed flair hyperintense lesions in subcortical white matter of the right parietal lobe, which could represent subcortical tubers in the setting of Tuberous Sclerosis Complex (TSC). The patient then had a whole body octreotide scan, which, in addition to the masses discussed thus far, showed bilateral anterior neck masses. Fine needle aspiration biopsy (FNAB) was reported as benign. The patient refused further invasive testing to get a tissue diagnosis.


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)

Brain MRI, showing subcortical masses.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig3: Brain MRI, showing subcortical masses.
Mentions: A brain MRI was later done because of the association of DIPNECH with MEN1 and tuberous sclerosis with angiomyolipoma of the kidney. MRI of the brain (Figure 3) showed flair hyperintense lesions in subcortical white matter of the right parietal lobe, which could represent subcortical tubers in the setting of Tuberous Sclerosis Complex (TSC). The patient then had a whole body octreotide scan, which, in addition to the masses discussed thus far, showed bilateral anterior neck masses. Fine needle aspiration biopsy (FNAB) was reported as benign. The patient refused further invasive testing to get a tissue diagnosis.

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