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    International Journal of Innovative Science and Research Technology
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    Small cell neuroendocrine carcinomas of tonsil are extremely rare cancers and only a few cases have been reported till date. They carry a poor prognosis. Paraneoplastic syndromes like SIADH, Cushing’s syndrome and Eaton-Lambert myasthenic syndrome can be found associated with these carcinomas. The tumor can metastasize to liver, lungs, bone, brain and skin.

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    Small Cell Neuroendocrine Carcinoma of Tonsil – a Rare Occurrence

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    Small cell neuroendocrine carcinomas of tonsil are extremely rare cancers and only a few cases have been reported till date. They carry a poor prognosis. Paraneoplastic syndromes like SIADH, Cushing’s syndrome and Eaton-Lambert myasthenic syndrome can be found associated with these carcinomas. The tumor can metastasize to liver, lungs, bone, brain and skin.

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    33 views2 pages

    Small Cell Neuroendocrine Carcinoma of Tonsil - A Rare Occurrence

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    International Journal of Innovative Science and Research Technology
    Small cell neuroendocrine carcinomas of tonsil are extremely rare cancers and only a few cases have been reported till date. They carry a poor prognosis. Paraneoplastic syndromes like SIADH, Cushing’s syndrome and Eaton-Lambert myasthenic syndrome can be found associated with these carcinomas. The tumor can metastasize to liver, lungs, bone, brain and skin.

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    of 2

    Volume 5, Issue 7, July – 2020 International Journal of Innovative Science and Research Technology

    ISSN No:-2456-2165

    Small Cell Neuroendocrine Carcinoma of


    Tonsil – A Rare Occurrence
    1.
    Peoli Mukutawat, 2. Anil Kumar Dhull, 3. Vivek Kaushal, 4. Rakesh Dhankhar, 5. Rajeev Atri, 6. Kunwar Prativyom
    Dr. Anita’s Diagnostic Realm, Sigra, Varanasi

    Abstract originates in the tonsil is extremely rare and only a few


    cases have been reported since it was first seen by Koss et
     Introduction: al. in 1972. Small cell neuroendocrine carcinoma of tonsil
    Small cell neuroendocrine carcinomas of tonsil are carries a poor prognosis.3
    extremely rare cancers and only a few cases have been
    reported till date. They carry a poor prognosis. Neuroendocrine carcinoma is positive for
    Paraneoplastic syndromes like SIADH, Cushing’s choromogranin A, synaptophysin and CD56 endocrine
    syndrome and Eaton-Lambert myasthenic syndrome markers. A Ki67 or mitotic index of 20% or more is also
    can be found associated with these carcinomas. The necessary for diagnosing NEC. Tumors with less than 20%
    tumor can metastasize to liver, lungs, bone, brain and Ki67 positivity are diagnosed as neuroendocrine tumors.4
    skin.
    As of now, the treatment strategy for small cell
     Case Presentation: neuroendocrine carcinoma has not been properly
    A 70-year-old male presented with a 2-month formulated due to the sparsity of data. Here we present a
    history of pain in throat which was moderate to severe rare case of primary small cell neuroendocrine carcinoma
    in intensity and intermittent.Local examination of arising from the tonsil.
    oropharynx revealed a 5×5 cm ulcero-proliferative
    growth over the right tonsil extending to right side of II. CASE PRESENTATION
    base of tongue, vallecula, and right retromolar trigone.
    Multiple matted lymph nodes were palpable in the right A 70-year-old male presented with a 2-month history
    cervical region. Histopathological examination of tissue of pain in throat which was gradual in onset, progressive,
    sample from the growth over the right tonsil revealed moderate to severe in intensity, intermittent, and with no
    small cell neuroendocrine carcinoma. On aggravating or relieving factors. The patient was a chronic
    immunohistochemistry, chromogranin and CD56 were smoker since 40-years. There was no history of any chronic
    positive. Patient received 2-courses of neoadjuvant illness or history of cancer in the family and the patient was
    chemotherapy with cisplatin and etoposide. Then the not receiving any medication.
    patient was planned for external beam radiation
    therapy with dose of 60 Gy in 30 fractions in 6 weeks. General physical examination and systemic
    examination were normal. Local examination of
     Discussion: oropharynx revealed a 5×5 cm ulcero-proliferative growth
    Small cell neuroendocrine carcinoma of tonsil are over the right tonsil extending to right side of base of
    likely to be very aggressive with a tendency of tongue, vallecula, and right retromolar trigone. Multiple
    developing early regional lymphatic and systemic matted lymph nodes were palpable in the right cervical
    metastases. More research and clinical trials shall be region involving levels IB, II, III, and IV; largest lymph
    explored to obtain a standard treatment strategy for node measuring 7×5 cm. Hematological and biochemical
    these patients. profile were within normal limits. Chest X-ray was also
    normal.
    I. INTRODUCTION
    CECT scan of face and neck revealed 5×4.5 cm
    Neuroendocrine carcinoma (NEC) is a malignant heterogeneously enhancing lobulated soft tissue mass
    epithelial cancer with neuroendocrinal morphology. Small lesion over the right tonsil with obliteration of right
    cell neuroendocrine carcinomas are poorly differentiated tonsillo-lingual sulcus and extending to right side of base of
    neuroendocrine tumors. They commonly originate in tongue and thickening of right lateral pharyngeal wall
    pulmonary, gastrointestinal tract and genitourinary system. superiorly upto soft palate. Multiple enlarged lymph nodes
    They have also been reported to occur in many other organs were noted in the right side of neck, largest being 6.5×5
    throughout the body.1The incidence of small cell NEC is cm.
    low in head and neck region. In head and neck region,
    larynx is relatively most commonly involved site, followed A biopsy was taken from the growth over the right
    by salivary glands, nasal cavity and paranasal tonsil and the histopathological examination revealed small
    sinuses.2Small cell neuroendocrine carcinoma (NEC) that

    IJISRT20JUL098 www.ijisrt.com 1
    Volume 5, Issue 7, July – 2020 International Journal of Innovative Science and Research Technology
    ISSN No:-2456-2165
    cell neuroendocrine carcinoma. On immunohistochemistry,
    chromogranin and CD56 were positive.

    Patient received 2-courses of neoadjuvant


    chemotherapy with injection cisplatin 150 mg and injection
    etoposide 150 mg, intravenously. Thereafter the disease
    had progressed in size and there was no response. Then the
    patient was planned for external beam radiation therapy
    with dose of 60 Gy in 30 fractions in 6 weeks.

    III. DISCUSSION

    Small cell neuroendocrine carcinomas of tonsil are


    extremely rare cancers and only a few cases have been
    reported since it was first seen by Koss et al. in 1972. They
    carry a poor prognosis.3 They most commonly occur during
    5th to 7th decade of life, and are more common in males
    than in females (male : female ratio = 2:1). The patient
    usually presents with a painless neck mass. Paraneoplastic
    syndromes like SIADH, Cushing’s syndrome and Eaton-
    Lambert myasthenic syndrome can be found associated
    with these carcinomas.2 The tumor can metastasize to liver,
    lungs, bone, brain and skin.5

    The treatment options for small cell neuroendocrine


    carcinoma of tonsil are chemotherapy, radiotherapy,
    surgical resection or a combinations of these modalities.
    Though, as of now, the treatment strategy for small cell
    neuroendocrine carcinoma has not been properly
    formulated due to the sparsity of data.

    Small cell neuroendocrine carcinoma of tonsil are


    likely to be very aggressive with a tendency of developing
    early regional lymphatic and systemic metastases. Due to
    this reason, it may be considered as a systemic disease.2
    More research and clinical trials shall be explored to obtain
    a standard treatment strategy for these patients.

    REFERENCES

    [1]. Jang H, Yuk SM, Kim JO, Han DS. A rare case of
    primary malignant small cell carcinoma combined
    with urothelial cell carcinoma in the ureter. World J
    SurgOncol. 2013;11:181.
    [2]. Renner G. Small cell carcinoma of the head and neck:
    a review. SeminOncol. 2007;34:3–14.
    [3]. Koss LG, Spiro RH, Hajdu S. Small cell (oat cell)
    carcinoma of minor salivary gland
    origin. Cancer. 1972;30:737–741.
    [4]. Hou X, Wei JC, Wu JX, Wang X, Fu JH, Lin P, et al.
    Multidisciplinary modalities achieve encouraging
    long-term survival in resectable limited-disease
    esophageal small cell carcinoma. PLoS One.
    2013;8(7):e69259 Epub 2013/07/23.
    [5]. Abedi E, Sismanis A: Extrapulmonary oat-cell
    carcinoma of the tonsil. Ear Nose Throat J
    1987;66:112– 115.

    IJISRT20JUL098 www.ijisrt.com 2

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