December02,2022

Abstract Volume: 4 Issue: 6 ISSN:

Case of Metastatic Spinal Disease with Unusual Primary

Parmilan Gill 1, Farah Nasir 2, Aravind Ponnuswamy 3, Tom Healey 4

1. IMT trainee, Countess of Chester Hospital NHS FT, Countess of Chester Health Park, Liverpool Road, CH2 1UL.

2. Specialist Registrar, Countess of Chester Hospital NHS FT, Countess of Chester Health Park, Liverpool Road, CH2 1UL.

3. Consultant Respiratory Physician, Countess of Chester Hospital NHS FT, Countess of Chester Health Park, Liverpool Road, CH2 1UL.

4. Associate Specialist-Oral & Maxillofacial Surgery, Countess of Chester Hospital NHS FT, Countess of Chester Health Park, Liverpool Road, CH2 1UL.


Corresponding Author: Aravind Ponnuswamy, Consultant Respiratory Physician, Countess of Chester Hospital NHS FT, Countess of Chester Health Park, Liverpool Road, CH2 1UL.

Copy Right: © 2022 Aravind Ponnuswamy, This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Received Date: April 26, 2022

Published Date: May 01, 2022

 

Abstract

We present the case of a 60-year-old lady admitted with lower back pain subsequently diagnosed as metastatic spine disease. Although the patient had a background history of breast cancer and basal cell carcinoma of the skin in the past, a thorough search for primary revealed an unusual cause for her metastatic disease. A review of the patient's known primary sites was negative but interestingly had an oral ulcer that was histologically confirmed to be small cell carcinoma. It is easy to assume the metastasis was from the breast. However, a thorough history and focussed examination of the oral cavity helped us pick up the oral ulcer, which turned out to be small cell carcinoma, a rare cause for metastasis and spinal spread. The case highlights the importance of oral examination in a patient with metastatic disease and consideration of unusual primary.

Case of Metastatic Spinal Disease with Unusual Primary

Background

Studies have shown that the spine is the third most common metastatic site. In up to 15 to 20% of patients, a primary is not identified (1). The most common primary sites include the lungs, breast, prostate, kidneys and haematological system. Identifying a primary is essential to tailor treatment, which may influence the prognosis and survival. Current guidelines do not prescribe direct oral examination for metastasis of unknown primary disease 92).

Small cell carcinomas (SCC) are among the most aggressive forms of neuroendocrine carcinoma (3). They are most commonly seen in the lungs but can originate from the cervix, bladder, prostate and gastrointestinal tract. They are rare in the head and neck region, most commonly from the larynx, salivary glands and Sino-nasal region (4) . Database studies demonstrate that only 0.3% of head and neck cancers are histologically small cells carcinomas.


Case Presentation

A 65-year-old female was admitted with a history of sudden onset lower back pain of three weeks duration and constipation. There were no symptoms suggesting cauda equina syndrome. The patient complained of intra-oral pain from the right side of her maxilla, severe enough to prevent her from wearing her dentures. The patient denied dysgeusia, intra-oral bleeding, dysphagia, otalgia, weight loss or night sweats.

Past medical history included severe eosinophilic asthma. The patient was under the regional severe asthma team, having been commenced on mepolizumab one year previously with good results. The patient also had type 2 diabetes mellitus, CKD 3, hiatus hernia, colonic polyps, osteoporosis, and basal cell carcinoma. In 2018, the patient was diagnosed with screen-detected right breast cancer (grade 1 cribriform ductal carcinoma), subsequently managed with tamoxifen. The patient was a lifelong non-smoker, drank alcohol rarely with no family history of cancer. 

Neurological examination revealed loss of power in both legs. There was a slight facial swelling over the right side of the maxilla, and a right-sided neck lymph node was palpable at level III. Intra-orally, a raised erythematous lesion with rolled edges was noted, overlying the right maxillary alveolar ridge and extending to involve the buccal sulcus and palatal mucosa, overall measuring 4cm by 3cm. The patient was partially dentate, with only her upper and lower anterior teeth remaining.


Investigations

Routine blood results were unremarkable. MRI of the spine revealed multiple diffuse bony metastases with no evidence of cord compression. An orthopantomogram revealed no dent alveolar pathology. The maxillofacial team carried out an incisional biopsy of the intra-oral lesion. Histological examination confirmed a small cell carcinoma with malignant cells showing strong positivity for synaptophysin and p63. They were negative for chromogranin, GATA3, CD3, CD5 and CD20. (Fig 1 and 2)

MRI of the head & neck revealed a malignant mass of the right maxilla, centred in the alveolar mucosa extending into the subcutaneous tissue of the right cheek and oral cavity and breaching the anterior and inferior bony wall of the maxillary sinus with right level III lymphadenopathy, staged at T3N1MX.

CT staging showed no pulmonary pathology, but extensive metastatic infiltration of the liver and left proximal humerus was identified.

Fig 1 section of the cheek biopsy Fig 2 HP section of the cheek biopsy


Outcome

The patient was discussed at the regional head and neck MDT, where it was felt that the tumour was extensively disseminated for treatment with curative intent. Since the patient had a WHO score of 4 and multiple comorbid conditions, MDT recommended the best supportive care. Unfortunately, the patient passed away three weeks later with palliative team support.

 

Discussion

Identifying primary in a case of metastatic spine disease can be tricky and requires a detailed history, systematic review and examination. Investigation for identifying a primary can be exhaustive. Our junior doctors' thorough examination and history taking picked up this lady's diagnosis. Ours is one of the first cases of metastatic spinal disease arising from an oral small cell carcinoma reported in Western literature to the best of our knowledge. Previous reports and series (5,6,7) do not feature any spinal or liver metastatic disease, and we believe this case is unique. Current literature quotes only two other small cell cancer of the cheek and tonsils in World literature (8, 9)

The patient was investigated for a primary lung or the breast tumour's re-occurrence. The lung, breast, head and neck MDTs all concluded that the metastases were likely derived from the small, oral cell cancer. Since the patient was not fit for active oncological therapy, a further biopsy of the metastatic disease was not merited to confirm the diagnosis. The lack of biopsy of the metastasis is the only limitation of our report. However, the concerted opinion of the MDTs and the absence of any evidence of the recurrence of breast tumour or primary lung cancer makes the metastasis likely originating from an oral small cell lesion.


Conclusion

This case adds to the literature that Oral small cell carcinoma can present as distant spinal metastases. The report highlights the importance of oral examination in metastatic spine disease.


References

1. Paholpak, P., Sirichativapee, W., Wisanuyotin, T., Kosuwon, W., & Jeeravipoolvarn, P. (2012). Prevalence of known and unknown primary tumour sites in spinal metastasis patients. The open orthopaedics journal, 6, 440–444. https://doi.org/10.2174/1874325001206010440

2. https://www.nice.org.uk/guidance/cg104/resources/metastatic-malignant-disease-of-unknown-primary-origin-in-adults-diagnosis-and-management-pdf-35109328970437

3. Thompson L. World Health Organisation Classification of Tumours: Pathology and Genetics of Head and Neck Tumours. Ear, Nose and Throat J. 2006;85:74

4. Jaiswal VR and Hoang MP. Primary Combined Squamous and Small Cell Carcinoma of the Larynx – Case Report and Review of Literature. Arch.Pathol. Lab.Med 2004 Nov;128(11):1279-82

 

5. Koss LG, Spiro RH, Hajdu S. Small cell (oat cell) carcinoma of minor salivary gland origin. Cancer. 1972 Sep; 30(3):737-41.

6. Bishop JA, Westra WH. Human papillomavirus-related small cell carcinoma of the oropharynx. The American journal of surgical pathology. 2011 Nov; 35(11):1679.

7.  Kraft S, Faquin WC, Krane JF. HPV-associated neuroendocrine carcinoma of the oropharynx: a rare new entity with potentially aggressive clinical behaviour. The American journal of surgical pathology. 2012 Mar 1; 36(3):321-30.

8. Terada T. Small cell carcinoma of the oral cavity (cheek mucosa): a case report with an immunohistochemical and molecular genetic analysis. International Journal of Clinical and Experimental Pathology. 2013; 6(4):780.

9. Shenoy N, Sholapurkar AA, Pai KM, Pal K. Small cell carcinoma of the oral cavity: a rare case report. Journal of the California Dental Association. 2009 Jun; 37(6):399.

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