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Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman.

Jahandideh H, Nasoori Y, Rostami S, Safdarian M - Case Rep Otolaryngol (2016)

Bottom Line: There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients.Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago.The clinical and histopathological findings are reported with a brief discussion about the disease.

View Article: PubMed Central - PubMed

Affiliation: ENT and Head & Neck Surgery Research Center, Department of Otolaryngoloy, Head and Neck Surgery, Hazrat Rasool Hospital, Iran University of Medical Sciences, Tehran 11365-3876, Iran.

ABSTRACT
Langerhans cell histiocytosis (LCH) is a very rare condition that commonly affects the head and neck region. There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients. Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago. The clinical and histopathological findings are reported with a brief discussion about the disease.

No MeSH data available.


Related in: MedlinePlus

Histological view of the laryngeal lesion (magnification: (a) ×10, (b) ×100, and (c) ×1000).
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fig3: Histological view of the laryngeal lesion (magnification: (a) ×10, (b) ×100, and (c) ×1000).

Mentions: Physical examination showed a 5∗5 cm supraglottic mass with extension to the pharynx. Direct laryngoscopy revealed a hypopharyngeal mass with normal epithelium, while the movement of true vocal cords could not be assessed. The physical examination of other organs was otherwise normal. The fine needle aspiration (FNA) cytology from the right neck laryngeal mass showed hypercellular smears composed of some dissociated atypical cells with large pleomorphic nuclei and high nucleus-to-cytoplasm (N/C) ratio mixed with acute inflammatory cells in a necrotic background. Reactive lymphoid tissue lined by partially atrophied squamous epithelium was reported in addition to some atypical large lymphocytes invading epithelium, suggesting lymphoepithelial lesion with extensive necrosis. Figure 1 shows the stroboscopic view of the lesion and cervical CT-scan of the laryngeal mass is shown in Figures 2(a)–2(c). The incisional biopsy of the right cervical mass was done with a macroscopic feature of a creamy soft tissue. Sections showed neoplastic tissue composed of large mononuclear and few multinuclear cells sheets admixed with eosinophils, which infiltrated muscle bundles on microscopic evaluations. Tumor cells had irregular nuclei with prominent grooves, folds, and inconspicuous nucleoli. Figure 3 shows the histological view of the laryngeal lesion. The diagnosis was suggested to be LCH (histiocytosis X). The tumor markers CD1a and S100 were positive in the immunohistochemical (IHC) staining. Based on the clinical and cytomorphological findings, a diagnosis of laryngeal LCH was made for the patient. In order to rule out the other potentially involved sites, a whole body bone scan and a chest CT-scan were done for the patient, which were both negative for an extra involvement site. Although surgical excision is considered as the standard treatment, anticipated morbidity, due to extensive surgical procedure of laryngopharyngectomy, convinced us to choose low-dose radiation. The patient had 10 sessions of cervical radiotherapy with a dose of 2000 cGy at each session. This protocol was well tolerated with a good response to treatment (Figures 2(d)–2(f)).


Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman.

Jahandideh H, Nasoori Y, Rostami S, Safdarian M - Case Rep Otolaryngol (2016)

Histological view of the laryngeal lesion (magnification: (a) ×10, (b) ×100, and (c) ×1000).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4835642&req=5

fig3: Histological view of the laryngeal lesion (magnification: (a) ×10, (b) ×100, and (c) ×1000).
Mentions: Physical examination showed a 5∗5 cm supraglottic mass with extension to the pharynx. Direct laryngoscopy revealed a hypopharyngeal mass with normal epithelium, while the movement of true vocal cords could not be assessed. The physical examination of other organs was otherwise normal. The fine needle aspiration (FNA) cytology from the right neck laryngeal mass showed hypercellular smears composed of some dissociated atypical cells with large pleomorphic nuclei and high nucleus-to-cytoplasm (N/C) ratio mixed with acute inflammatory cells in a necrotic background. Reactive lymphoid tissue lined by partially atrophied squamous epithelium was reported in addition to some atypical large lymphocytes invading epithelium, suggesting lymphoepithelial lesion with extensive necrosis. Figure 1 shows the stroboscopic view of the lesion and cervical CT-scan of the laryngeal mass is shown in Figures 2(a)–2(c). The incisional biopsy of the right cervical mass was done with a macroscopic feature of a creamy soft tissue. Sections showed neoplastic tissue composed of large mononuclear and few multinuclear cells sheets admixed with eosinophils, which infiltrated muscle bundles on microscopic evaluations. Tumor cells had irregular nuclei with prominent grooves, folds, and inconspicuous nucleoli. Figure 3 shows the histological view of the laryngeal lesion. The diagnosis was suggested to be LCH (histiocytosis X). The tumor markers CD1a and S100 were positive in the immunohistochemical (IHC) staining. Based on the clinical and cytomorphological findings, a diagnosis of laryngeal LCH was made for the patient. In order to rule out the other potentially involved sites, a whole body bone scan and a chest CT-scan were done for the patient, which were both negative for an extra involvement site. Although surgical excision is considered as the standard treatment, anticipated morbidity, due to extensive surgical procedure of laryngopharyngectomy, convinced us to choose low-dose radiation. The patient had 10 sessions of cervical radiotherapy with a dose of 2000 cGy at each session. This protocol was well tolerated with a good response to treatment (Figures 2(d)–2(f)).

Bottom Line: There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients.Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago.The clinical and histopathological findings are reported with a brief discussion about the disease.

View Article: PubMed Central - PubMed

Affiliation: ENT and Head & Neck Surgery Research Center, Department of Otolaryngoloy, Head and Neck Surgery, Hazrat Rasool Hospital, Iran University of Medical Sciences, Tehran 11365-3876, Iran.

ABSTRACT
Langerhans cell histiocytosis (LCH) is a very rare condition that commonly affects the head and neck region. There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients. Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago. The clinical and histopathological findings are reported with a brief discussion about the disease.

No MeSH data available.


Related in: MedlinePlus