Small Cell Carcinoma of the Larynx - · PDF fileSmall Cell Carcinoma of the Larynx Satoshi...

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Small Cell Carcinoma of the Larynx Satoshi Ohno, MD, PhD 1) ; Shigeru Hirano, MD, PhD 2) ; Shinji Takebayashi, MD, PhD 1) ; Chiaki Suzuki, MD 1) ; Takushi Miyoshi, MD 1) ; Saki Yabuuchi, MD 1) ; Hiroki Ikeda, MD, PhD 1) ; Makoto Miura, MD, PhD 1) 1) Department of Otolaryngology, Japanese Red Cross Wakayama Medical Center 2) Department of Otolaryngology, Head and Neck Surgery, Graduate school of Medicine, Kyoto University INTRODUCTION DISCUSSION THERAPEUTIC COURSE Figure 1. Fiberoptic Laryngoscopy Figure 2. PET-CT ABSTRACT CONCLUSIONS CONTACT Educational Objective: At the conclusion of this presentation, the participants should be able to understand the clinical and pathologic features of small cell carcinoma of the larynx. Treatment options should be discussed in order to manage this poor- prognosis cancer. Objectives: Small cell carcinoma arises commonly in the lung and rarely in the head and neck, especially in the larynx. It tends to be very aggressive and metastasize widely, leading to very poor prognosis. We describe our experience with a systematic review and discuss treatment options of this rare tumor. Study Design: Case report and literature review. Methods: A case report is described with fiberscopic images, positron emission tomography - computed tomography (PET- CT) and histopathologic images. A literature review is presented and treatment options are discussed to achieve better prognosis. Results: A 70 year old man presented with throat discomfort. Fiberoptic laryngoscopy revealed ulcerative tumor in the right false vocal fold of the larynx. PET-CT demonstrated the tumor, invading the thyroid cartilage, and metastatic lymph nodes swelling in the right neck, however there was no evidence of metastasis in other organs. The patient underwent total laryngectomy and right modified radical neck dissection. Histopathologic analysis confirmed the diagnosis of small cell carcinoma of the larynx. Five cycles of chemotherapy (Cisplatin/ Etoposide) were delivered, according to the therapeutic regimen for pulmonary small cell carcinoma. Conclusions: Small cell carcinoma of the larynx is a very rare and aggressive tumor with high rate and early tendency for distant metastases. Systemic chemotherapy should be delivered as soon as possible to prevent distant metastases. Surgical resection of primary tumor for locally advanced tumor without distant metastasis might be one of therapeutic options to achieve quick local control and deliver systemic chemotherapy immediately, leading to better prognosis. Operation Total Laryngectomy Right Neck Dissection (Level I-V) Excised Specimen (Figure 3) The tumor (red arrow) was found on the right false vocal fold. Histology and Immunohistochemistry (Figure 4) High N/C ratio, naked nucleus like cells No keratinization Thyroid cartilage invasion (-) Definitive Diagnosis Small Cell Carcinoma of the Larynx (Supraglottis) pT3N2bM0, stage IVA Postoperative Course Five cycles of chemotherapy were delivered from three weeks after the operation, according to the therapeutic regimen for pulmonary small cell carcinoma (Cisplatin 80mg/m 2 + Etoposide 100mg/m 2 ). There is no evidence of recurrence and metastasis nine months after the operation. Small Cell Carcinoma of the Larynx Firstly reported in 1972 (Olofsson et al. 1972) 0.5% of laryngeal cancer Age Median age; 64 years old (rarely seen in patient younger than 40 years) Gender difference Male:Female = 3:1 Occurrence site Supraglottis:Glottis:Subglottis = 6:1:3 Metastases at the initial visit Neck lymph nodes: >50% Distant organs: 10-20% Overall survival (OS) 2-year OS: 16%, 5-year OS: 5% 2-year OS w/o chemotherapy: w/ chemotherapy = 10%: 52% Cause of death Distant metastases (70%) Therapy w/ distant metastasis : Concurrent chemoradiotherapy + adjuvant chemotherapy w/o distant metastasis : T1: Surgical resection or concurrent chemoradiotherapy + adjuvant chemotherapy T2: Concurrent chemoradiotherapy + adjuvant chemotherapy T3, T4: Not established because of few reports CASE PRESENTATION Clinical History A 70 year-old man was seen in a private clinic with many-year history of hoarseness and a month history of throat discomfort. Fiberoptic laryngoscopy revealed a tumor in the larynx. He was referred to Japanese Red Cross Wakayama Medical Center for further examinations. Previous History Chronic thyroiditis Smoking Habit 20 cigarettes/day x 40 years Clinical Examination Fiberoptic Laryngoscopy (Figure 1). A tumor extended from the right false vocal fold to the laryngeal side of the epiglottis. The mobility of the right vocal fold was slightly limited. Positron Emission Tomography - Computed Tomography (PET-CT) (Figure 2) The supraglottic tumor (red arrow) showed high uptake of FDG and invasion of the thyroid cartilage. Level III and IV lymph nodes swelling ( green arrow) in the right neck showed high uptake of FDG and suspicious invasion of the right internal jugular vein. There was no evidence of metastasis in other organs. Biopsy Suspicious of small cell carcinoma Clinical Diagnosis Laryngeal Cancer (Supraglottis), cT4aN2bM0 Small cell carcinoma of the larynx is a very rare and aggressive tumor with high rate and early tendency for distant metastases. Systemic chemotherapy should be delivered as soon as possible to prevent distant metastases. Surgical resection of primary tumor for locally advanced tumor without distant metastasis might be one of therapeutic options to achieve quick local control and deliver systemic chemotherapy immediately, leading to better prognosis. Satoshi Ohno, MD, PhD Department of Otolaryngology Japanese Red Cross Wakayama Medical Center Email: [email protected] Small cell carcinoma arises commonly in the lung and rarely in the head and neck, especially in the larynx. It tends to be very aggressive and metastasize widely, leading to very poor prognosis. We describe our experience with a systematic review and discuss treatment options of this rare tumor. CD56(+) chromogranin(+) NSE(+) synaptophysin(+) HE staining Figure 3. Excised Specimen Figure 4. Histology and Immunohistochemistry THERAPEUTIC STRATEGY Need for airway management because of airway narrowing by the tumor Locally advanced tumor (T4a) without distant metastasis (M0) Need for quick delivery of chemotherapy if pathology confirms small cell carcinoma Total Laryngectomy + Right Neck Dissection + Postoperative Chemotherapy one week after the pathological report R L R L R L L R

Transcript of Small Cell Carcinoma of the Larynx - · PDF fileSmall Cell Carcinoma of the Larynx Satoshi...

Page 1: Small Cell Carcinoma of the Larynx - · PDF fileSmall Cell Carcinoma of the Larynx Satoshi Ohno, MD, PhD 1); Shigeru Hirano, MD, PhD 2); Shinji Takebayashi, MD, PhD 1); Chiaki Suzuki,

Small Cell Carcinoma of the Larynx Satoshi Ohno, MD, PhD 1); Shigeru Hirano, MD, PhD 2); Shinji Takebayashi, MD, PhD 1); Chiaki Suzuki, MD 1);

Takushi Miyoshi, MD 1); Saki Yabuuchi, MD 1); Hiroki Ikeda, MD, PhD 1); Makoto Miura, MD, PhD 1) 1) Department of Otolaryngology, Japanese Red Cross Wakayama Medical Center

2) Department of Otolaryngology, Head and Neck Surgery, Graduate school of Medicine, Kyoto University

INTRODUCTION

DISCUSSION

THERAPEUTIC COURSE Figure 1. Fiberoptic Laryngoscopy

Figure 2. PET-CT

ABSTRACT

CONCLUSIONS

CONTACT

Educational Objective: At the conclusion of this presentation, the participants should be able to understand the clinical and pathologic features of small cell carcinoma of the larynx. Treatment options should be discussed in order to manage this poor-prognosis cancer. Objectives: Small cell carcinoma arises commonly in the lung and rarely in the head and neck, especially in the larynx. It tends to be very aggressive and metastasize widely, leading to very poor prognosis. We describe our experience with a systematic review and discuss treatment options of this rare tumor. Study Design: Case report and literature review. Methods: A case report is described with fiberscopic images, positron emission tomography - computed tomography (PET-CT) and histopathologic images. A literature review is presented and treatment options are discussed to achieve better prognosis. Results: A 70 year old man presented with throat discomfort. Fiberoptic laryngoscopy revealed ulcerative tumor in the right false vocal fold of the larynx. PET-CT demonstrated the tumor, invading the thyroid cartilage, and metastatic lymph nodes swelling in the right neck, however there was no evidence of metastasis in other organs. The patient underwent total laryngectomy and right modified radical neck dissection. Histopathologic analysis confirmed the diagnosis of small cell carcinoma of the larynx. Five cycles of chemotherapy (Cisplatin/ Etoposide) were delivered, according to the therapeutic regimen for pulmonary small cell carcinoma. Conclusions: Small cell carcinoma of the larynx is a very rare and aggressive tumor with high rate and early tendency for distant metastases. Systemic chemotherapy should be delivered as soon as possible to prevent distant metastases. Surgical resection of primary tumor for locally advanced tumor without distant metastasis might be one of therapeutic options to achieve quick local control and deliver systemic chemotherapy immediately, leading to better prognosis.

Operation Total Laryngectomy Right Neck Dissection (Level I-V) Excised Specimen (Figure 3) The tumor (red arrow) was found on the right false vocal fold. Histology and Immunohistochemistry (Figure 4) High N/C ratio, naked nucleus like cells No keratinization Thyroid cartilage invasion (-) Definitive Diagnosis Small Cell Carcinoma of the Larynx (Supraglottis) pT3N2bM0, stage IVA Postoperative Course Five cycles of chemotherapy were delivered from three weeks after the operation, according to the therapeutic regimen for pulmonary small cell carcinoma (Cisplatin 80mg/m2 + Etoposide 100mg/m2). There is no evidence of recurrence and metastasis nine months after the operation.

Small Cell Carcinoma of the Larynx Firstly reported in 1972 (Olofsson et al. 1972) 0.5% of laryngeal cancer Age Median age; 64 years old (rarely seen in patient younger than 40 years) Gender difference Male:Female = 3:1 Occurrence site Supraglottis:Glottis:Subglottis = 6:1:3 Metastases at the initial visit Neck lymph nodes: >50% Distant organs: 10-20% Overall survival (OS) 2-year OS: 16%, 5-year OS: 5% 2-year OS w/o chemotherapy: w/ chemotherapy = 10%: 52% Cause of death Distant metastases (70%) Therapy w/ distant metastasis: Concurrent chemoradiotherapy + adjuvant chemotherapy w/o distant metastasis: T1: Surgical resection or concurrent chemoradiotherapy + adjuvant chemotherapy T2: Concurrent chemoradiotherapy + adjuvant chemotherapy T3, T4: Not established because of few reports

CASE PRESENTATION Clinical History A 70 year-old man was seen in a private clinic with many-year history of hoarseness and a month history of throat discomfort. Fiberoptic laryngoscopy revealed a tumor in the larynx. He was referred to Japanese Red Cross Wakayama Medical Center for further examinations. Previous History Chronic thyroiditis Smoking Habit 20 cigarettes/day x 40 years Clinical Examination Fiberoptic Laryngoscopy (Figure 1). A tumor extended from the right false vocal fold to the laryngeal side of the epiglottis. The mobility of the right vocal fold was slightly limited. Positron Emission Tomography - Computed Tomography (PET-CT) (Figure 2) The supraglottic tumor (red arrow) showed high uptake of FDG and invasion of the thyroid cartilage. Level III and IV lymph nodes swelling (green arrow) in the right neck showed high uptake of FDG and suspicious invasion of the right internal jugular vein. There was no evidence of metastasis in other organs. Biopsy Suspicious of small cell carcinoma Clinical Diagnosis Laryngeal Cancer (Supraglottis), cT4aN2bM0

Small cell carcinoma of the larynx is a very rare and aggressive tumor with high rate and early tendency for distant metastases. Systemic chemotherapy should be delivered as soon as possible to prevent distant metastases. Surgical resection of primary tumor for locally advanced tumor without distant metastasis might be one of therapeutic options to achieve quick local control and deliver systemic chemotherapy immediately, leading to better prognosis.

Satoshi Ohno, MD, PhD Department of Otolaryngology Japanese Red Cross Wakayama Medical Center Email: [email protected]

Small cell carcinoma arises commonly in the lung and rarely in the head and neck, especially in the larynx. It tends to be very aggressive and metastasize widely, leading to very poor prognosis. We describe our experience with a systematic review and discuss treatment options of this rare tumor.

CD56(+) chromogranin(+)

NSE(+) synaptophysin(+)

HE staining

Figure 3. Excised Specimen

Figure 4. Histology and Immunohistochemistry THERAPEUTIC STRATEGY

Need for airway management because of airway narrowing by the tumor

Locally advanced tumor (T4a) without distant

metastasis (M0)

Need for quick delivery of chemotherapy if pathology confirms small cell carcinoma

Total Laryngectomy + Right Neck Dissection

+ Postoperative Chemotherapy

one week after the pathological report

R L

R L R L

L R

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