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CASE REPORT
Year : 2010  |  Volume : 6  |  Issue : 3  |  Page : 391-393

Metastatic lung cancer presenting with jugular foramen syndrome in a case of von Recklinghausens disease


1 Department of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi, Meghe, Wardha, Maharashtra, India
2 Department of ENT, Datta Meghe Institute of Medical Sciences, Sawangi, Meghe, Wardha, Maharashtra, India
3 Department of Pathology, Datta Meghe Institute of Medical Sciences, Sawangi, Meghe, Wardha, Maharashtra, India
4 Department of Anesthesiology, Datta Meghe Institute of Medical Sciences, Sawangi, Meghe, Wardha, Maharashtra, India
5 Department of Surgery, Datta Meghe Institute of Medical Sciences, Sawangi, Meghe, Wardha, Maharashtra, India

Date of Web Publication29-Nov-2010

Correspondence Address:
Amit Agarwal
Department of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), Wardha- 442004, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.73344

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 > Abstract 

Metastatic carcinomas from a distant primary malignancy involving the temporal bone particularly the jugular foramen are rare tumors. A 57-year-old gentleman had multiple gradually increasing swellings over the body since many years. For last two years, he noticed enlargement of the swelling in left calf and rapidly enlarging painful new swelling over the left chest wall in back for last one year. He had recent involvement of left seventh, eighth and lower cranial nerves. CT scan showed an extensive lesion in left jugular foramen region with bone destruction. The patient underwent decompression of the left jugular foramen mass lesion. The tumor was extremely vascular and a partial decompression could only be performed. Although there was relief in the headache but the neurological deficits were persisting. Histopathology of the tumor showed features of metastatic small ling cancer. In the present case, there was presence of long standing multiple swelling all over the body and the patient had painful enlargement that he perceived as an ongoing process of the von Recklinghausen's disease and made a delay in seeking the medical advice resulting in a well advanced disease and with poor prognosis.

Keywords: Skull base tumors, metastasis, cranial nerve palsy, von Recklinghausen′s disease, small cell cancer, lung cancer, jugular foramen, glomus jugulare tumor


How to cite this article:
Agarwal A, Baisakhiya N, Kakani A, Bhake A, Nagrale M, Reddy S. Metastatic lung cancer presenting with jugular foramen syndrome in a case of von Recklinghausens disease. J Can Res Ther 2010;6:391-3

How to cite this URL:
Agarwal A, Baisakhiya N, Kakani A, Bhake A, Nagrale M, Reddy S. Metastatic lung cancer presenting with jugular foramen syndrome in a case of von Recklinghausens disease. J Can Res Ther [serial online] 2010 [cited 2019 Jun 20];6:391-3. Available from: http://www.cancerjournal.net/text.asp?2010/6/3/391/73344


 > Introduction Top


Metastatic carcinomas from a distant primary malignancy involving the temporal bone particularly the jugular foramen are rare tumors, usually arising from the prostate, breast, kidney or lung and can have clinical and radiologic features similar to those of glomus jugulare tumors. [1],[2] We present an unusual case of jugular foramen metastases from a small cell cancer of the lung in a case of von Recklinghausen's disease.


 > Case Report Top


A 57-year-old gentleman had multiple gradually increasing swellings over the body since many years, for last two years he noticed enlargement of the swelling in left calf and a rapidly enlarging painful new swelling over the left side of the back of chest wall for last one year [Figure 1]b. He also had headache for last 15 days but it got worsened over last two days and associated with inability to close the left and decreased hearing and tinnitus in the left ear. He also noticed that he is coughing while eating food. There was no history of fever, trauma or ear discharge. Apart from that, he had bilateral corneal opacities for last two years after an episode of febrile illness details of which were not available. In childhood he suffered from smallpox. He was a chronic smoker for last thirty years. His general and systemic examination was unremarkable except multiple swelling all over the body [Figure 1]a-d. Neurologically, his higher mental functions were normal; fundus could not be checked because of bilateral corneal opacities. Cranial nerve examination showed left sided facial droop with drooling of saliva, left sided sensorineural deafness, impaired gag reflex on left side and difficulty in swallowing (left seventh, eighth, ninth and tenth cranial nerve involvement). Motor and sensory examination was normal. Pure-tone audiometry revealed left sided senosrineural deafness. CT scan of brain revealed an irregular vascular mass (about 8 × 6 cm in size) in the left jugular foramen with the extensive destruction of a surrounding bony structure [Figure 2]. An X-ray of chest revealed left seventh rib mass lesion with destruction of the bone but apparently normal lung fields [Figure 3]. The patient underwent decompression of the left jugular foramen mass lesion. The tumor was extremely vascular and a partial decompression could be performed. Histopathology of the tumor showed features of metastatic small ling cancer [Figure 4]. Fine needle aspiration cytology from the left chest wall lesion showed features of small cell cancer of the lung. Although there was relief in the headache with high dose of opioid analgesics but the neurological deficits were persisting. Because of the non-availability and non-affordability, fractionated stereotactic radiotherapy (FSRT) or stereotactic radiosurgery (SRS) could not be offered as a noninvasive treatment alternatives to microsurgery either for the primary control of the disease or for control of post-operative pain.
Figure 1: Clinical photographs showing multiple swelling (A) suprasternal notch, (B) over left side chest wall on back, (C) right shin and (D) over anterior chest wall and abdominal wall

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Figure 2: CT scan of brain plain, contrast and bone window images showing a large enhancing tumor in the left jugular foramen region with extensive bone destruction

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Figure 3: X-ray of chest showing destruction of the left seventh rib (arrow)

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Figure 4: Microscopic appearance of the tumor from the skull base suggestive of metastatic deposits from the small cell cancer of the lung (H and E, ×100)

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 > Discussion Top


Paragangliomas, schwannomas and meningiomas are the common jugular foramen tumors and because of their deep location their diagnosis and management are challenging. [3] Apart from these common neoplasms, a large variety of unusual tumors including carcinoma, sarcoma, and metastatic lesions are encountered at the jugular foramen and [2],[4] as in present case when these malignant tumors present with the clinical features of jugular foramen syndrome and also the radiological features of jugular foramen lesion can very closely mimic glomus jugulare and other primary tumors of this area. [4] Although metastases from the lung cancer have been implicated in many series, [5],[6] but only in few cases it was documented histologically. [6] Von Recklinghausen's disease, an autosomal dominant hereditary disease, is known to be associated with malignant tumor and neurofibrosarcomas being the most common; [7] lung cancer is an uncommon tumor in patients with von Recklinghausen's disease with only few case reports in the literature. [8],[9] The presence of erosion of the jugular foramen, intraluminal involvement of the jugular vein on CT scan, a heavy tumor stain on angiography, multiple tubular undulating signal voids and marked enhancement on contrast enhanced MRI scans, irrespective of the pathognomonic criteria of glomus jugulare tumor, [10] can only tell the presence of a vascular tumor but cannot reveal the true histopatholgical nature of the lesion. [3] In cases of schwannomas, benign meningiomas, noninfiltrative paragangliomas, and other benign jugular foramen tumors, a total surgical excision is the treatment of choice and may be curative. [11] However, in cases of metastatic lesions particularly in radio-resistant metastases, good surgical decompression offers better palliation than radiotherapy and can be achieved with the skull base approaches either alone or in combinations. [12] Postoperative radiotherapy is used to control the residual disease, where there is subtotal removal either to preserve the cranial nerve functions, vital vascular structures, and the brainstem. [13],[14] Novalis stereotactic radiotherapy (SRT) has been used in high doses (30-50 Gy in 10-14 fractions) as a safe and effective treatment modality to treat skull base metastases from visceral carcinomas with sparing of normal structures and it was shown that in majority of the patients cranial nerve deficits were improved either completely or partially. [14] Because of the non-availability and non-affordability, fractionated stereotactic radiotherapy (FSRT) or stereotactic radiosurgery (SRS) could not be offered to the patient as noninvasive treatment alternatives to microsurgery either for the primary control of the disease or for control of post-operative pain. In the present case, there was presence of long standing multiple swelling all over the body and the patient had painful enlargement that he perceived as an ongoing process of the von Recklinghausen's disease and made a delay in seeking the medical advice resulting in a well advanced disease and with poor prognosis.

 
 > References Top

1.Ferreira MA, Feiz-Erfan I, Zabramski JM, Spetzler RF, Coons SW, Preul MC. Endolymphatic sac tumor: unique features of two cases and review of the literature. Acta Neurochir (Wien) 2002;144:1047-53.  Back to cited text no. 1
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2.Chao CK, Sheen TS, Lien HC, Hsu MM. Metastatic carcinoma to the jugular foramen. Otolaryngol Head Neck Surg 2000;122:922-3.  Back to cited text no. 2
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3.Ramina R, Maniglia JJ, Fernandes YB, Paschoal JR, Pfeilsticker LN, Neto MC, et al. Jugular foramen tumors: diagnosis and treatment. Neurosurg Focus 2004;17:5.  Back to cited text no. 3
    
4.Lowenheim H, Koerbel A, Ebner FH, Kumagami H, Ernemann U, Tatagiba M. Differentiating imaging findings in primary and secondary tumors of the jugular foramen. Neurosurg Rev 2006;29:1-11.  Back to cited text no. 4
    
5.Hellier WP, Crockard HA, Cheesman AD. Metastatic carcinoma of the temporal bone presenting as glomus jugulare and glomus tympanicum tumours: a description of two cases. J Laryngol Otol 1997;111:963-6.  Back to cited text no. 5
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6.Greenberg HS, Deck MD, Vikram B, Chu FC, Posner JB. Metastasis to the base of the skull: clinical findings in 43 patients. Neurology 1981;31:530-7.  Back to cited text no. 6
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7.Shimizu Y, Tsuchiya S, Watanabe S, Saitoh R. von Recklinghausen's disease with lung cancer derived from the wall of emphysematous bullae. Intern Med 1994;33:167-71.  Back to cited text no. 7
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8.Satoh M, Wakabayashi O, Araya Y, Jinushi E, Yoshida F. Autopsy case of von Recklinghausen's disease associated with lung cancer, gastrointestinal stromal tumor of the stomach, and duodenal carcinoid tumor. Nihon Kokyuki Gakkai Zasshi 2009;47:798-804.  Back to cited text no. 8
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9.Walter J, Kuhn SA, Brodhun M, Reichart R, Kalff R. Pulmonary meningioma and neurinoma associated with multiple CNS tumours in a patient with neurofibromatosis type 2. Clin Neurol Neurosurg 2009;111:454-9.  Back to cited text no. 9
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10.Graham MD, Larouere MJ, Kartush JM. Jugular foramen schwannomas: diagnosis and suggestions for surgical management. Skull Base Surg 1991;1:34-8.  Back to cited text no. 10
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11.Ramina R, Maniglia JJ, Fernandes YB, Paschoal JR, Pfeilsticker LN, Coelho Neto M. Tumors of the jugular foramen: diagnosis and management. Neurosurgery 2005;57:59-68.  Back to cited text no. 11
    
12.Kumar R, Wani AA. Unusual tumors of the posterior fossa skull base. Skull Base 2006;16:75-84.  Back to cited text no. 12
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13.Fayad JN, Keles B, Brackmann DE. Jugular foramen tumors: clinical characteristics and treatment outcomes. Otol Neurotol 2010;31:299-305.  Back to cited text no. 13
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14.Mori Y, Hashizume C, Kobayashi T, Shibamoto Y, Kosaki K, Nagai A. Stereotactic radiotherapy using Novalis for skull base metastases developing with cranial nerve symptoms. J Neurooncol 2010;98:213-9.  Back to cited text no. 14
[PUBMED]  [FULLTEXT]  


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