 |
| About Bioline | All Journals | Testimonials | Membership | News |
|
||||||
|
||||||
Journal of Cancer Research and Therapeutics, Vol. 6, No. 3, July-September, 2010, pp. 391-393 Case Report Metastatic lung cancer presenting with jugular foramen syndrome in a case of von Recklinghausens disease Amit Agarwal1, Nitish Baisakhiya2, Anand Kakani1, Arvind Bhake3, Manda Nagrale4, Shivshankar Reddy5 1 Department of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi, Meghe, Wardha, Maharashtra, India Correspondence Address:Amit Agarwal, Department of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), Wardha- 442004, Maharashtra, India, dramitagrawal@gmail.com Code Number: cr10098 PMID: 21119288 DOI: 10.4103/0973-1482.73344 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 Introduction 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 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. Discussion 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
Copyright 2010 - Journal of Cancer Research and Therapeutics The following images related to this document are available:Photo images[cr10098f4.jpg] [cr10098f1.jpg] [cr10098f2.jpg] [cr10098f3.jpg] |
| |||||||||
![[Figure - 1]](/showimage?cr/photo/cr10098f1.jpg){kind=link}
![[Figure - 2]](/showimage?cr/photo/cr10098f2.jpg){kind=link}
![[Figure - 3]](/showimage?cr/photo/cr10098f3.jpg){kind=link}
![[Figure - 4]](/showimage?cr/photo/cr10098f4.jpg){kind=link}