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Clinical case: Horner's syndrome: want to learn more about it?

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Clinical case: Horner's syndrome

This is a clinical case of a Horner's Syndrome. 

After reviewing this case a student should be able to describe:

  • The arrangement of the lymph nodes and the drainage points of the lymphatic vessels of the neck.  What might cause cervical lymphadenopathy? 
  • The condition that the child likely would have had if the tumor had invaded the sternocleidomastoid muscle and caused the muscle to become fibrotic.
  • What is Horner syndrome and why it developed in this patient?  Also, in addition to the sympathetic trunk, what nerves might be at risk in the surgical extirpation done in this case and the consequences of injury to those nerves?
  • The origin of the internal carotid artery, including the vertebral level of the cervical segment. The relationship of this artery to the sympathetic innervation of the head.  

This article is based on a case report published in the Journal "Case Reports in Otolaryngology" in 2016, by Oz. Erol, Al. Koycu, and Er. Aydin.

It has been modified and reviewed by Joel A. Vilensky PhD, Carlos A. Suárez-Quian PhD, Aykut Üren, MD.

Case description

History

The patient was a five-year old boy complaining of a non-painful swelling in the left side of the neck that had been present for the past four months. The boy had not had any other complaints such as loss of weight, fever, night sweating, respiratory distress, or difficulty swallowing.

Physical examination

The physical examination revealed a hard, mobile mass with an approximate size of 2 X 3 cm on the anterior surface of the sternocleidomastoid muscle in the patient’s neck (Figure 1).

Figure 1: Cadaver image, location of the tumor

Imaging

A prior neck ultrasonography had suggested a diagnosis of lymphadenopathy. MRI revealed a limited 2.8 X 2.3 cm lesion on the sternocleidomastoid muscle and internal carotid artery. The lesion had a mass effect in that the parotid gland was displaced anteriorly (Figure 2B).

Figure 2: A - coronal MRI showing the tumor. B - axial MRI showing the tutor

Diagnosis

A fine-needle aspiration biopsy was refused by the family. Examination of the patient one month later revealed additional growth of the tumor; the tumor was now approximately 4 X 4 cm. Surgery was recommended to the patient’s family with the caveat that:

  • the tumor was deep to the large arteries in the neck,
  • that it involves nerves in addition to the presumed enlarged lymph node, and accordingly that there were surgical risks of a droopy left eyelid, respiratory changes and some loss of vocal control.

The family gave written consent for the surgery. The patient underwent the surgery with provisional diagnosis of lymphadenopathy with neural involvement

Management

The encapsulated tumor on the sternocleidomastoid was found to be displacing the internal carotid artery and internal jugular vein laterally (Figure 2A); it was separated from the surrounding tissue by blunt dissection. The tumor was found to have continuity superiorly and inferiorly with nerve elements, and that it was entirely of neural origin (not an enlarged lymph node). The total tumor was extracted (Figure 3B).

Figure 3: A - neurogenic tumor in situ. B - excised tumor. C - superior part of the sympathetic trunk in situ

Histopathology

Subsequent, pathological evaluation revealed the nerve to be an intermixed type of ganglioneuroblastoma (Figure 4), consisting of:

  • Schwannian stroma
  • Neuropil-like islands
  • Differentiating neuroblasts
  • Immature and dysplastic ganglion cells
Figure 4: Histological appearance of the tumor. H&E Stain. A. 10X B. 40X

Evolution

After the surgery the patient developed an ipsilateral Horner syndrome (miosis, ptosis, and anhidrosis). However, these signs were not apparent at follow-up three months post surgery.

After a I-131 Metaiodobenzylguanidine (I-131 MIBG) scan in the 6th- month postoperative examination, an involvement was observed in the contralateral cervical region; the patient was directed to undergo adjuvant chemotherapy to treat this contralateral tumor

Metaiodobenzylguanidine [MIBG] is a catecholamine analog similar to noradrenaline, which can be up taken by hyperactive endocrine tissues and certain types of tumors including medullary carcinoma of thyroid, pheochromocytoma, neuroblastoma, paraganglioma and other neuroendocrine tumors. Combining a radioactive tracer [131I-MIBG], provides both a diagnostic tool and a therapeutic agent. 131I-MIBG is administered intravenously, and the whole body imaging of the patient is acquired 24 and 48 hour later. The patient in this case thus had a diagnostic 131I-MIBG examination

At 12 months postoperative there was no indication of any recurrence presumably as a result of the adjuvant chemotherapy.

Anatomical and medical considerations

Neuroblastomas

Neuroblastomas are malignant solid tumors that develop from abnormal replication of immature sympathetic neurons. Neuroblastomas can develop anywhere sympathetic nervous tissues are present. They are most commonly seen in the suprarenal gland and within the sympathetic trunks.

Ganglioneuroma

Different levels of microscopic differentiations are observed in the majority of neuroblastomas. These tumors may transform into ganglioneuroma on the basis of fibrous stroma. Various levels of differentiation may be observed between pure neuroblastoma and pure ganglioneuroma, and these are called ganglioneuroblastoma. Cervical tumors are common pathologies in children. Whereas most are benign, nevertheless the suspicion of malignancy must come into play in cases of unusual tumors.

Ganglioneuroblastomas

Ganglioneuroblastomas represent a histological subgroup of rare neuroblastic tumors with moderate malignancy potential. They are very difficult to diagnose based solely on only physical examination and imaging modalities.  Typically, final diagnosis is possible only after cytological and immuno-histochemical analysis following surgical excision.

In cases in which surgical excision is recommended, the parents consent should be obtained and they should be told of possible complications, such as:

  • Horner syndrome due to sympathetic nerve chain incision
  • respiratory, vocal  and nutrition problems due to vagal nerve incision, etc.

Establishing Prognosis

Most neurogenic tumors secrete catecholamines. High levels of catecholamines may indicate the likelihood of recurrence. Genetic characteristics also may be used to predict tumor behavior. N-myc is a protooncogen, in which its chromosome arm resides at the far end of 2p. Detection of multiple N-myc copies indicates a fast tumor growth and a negative prognosis in patients who show these histological characteristics and are over 1 year of age. Also, a scintigraphic scan with MIBG should be conducted and invasion should be determined. In our patient, vanilmandelic acid (VMA) and N-myc protooncogene were found to be negative during the postoperative period.

Clinical case: Horner's syndrome: want to learn more about it?

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