Pediatr Blood Cancer 2005;44:280–282

BRIEF REPORTSuperior Mediastinal Syndrome With Rowland–Payne Syndrome: An Unusual Presentation of

Cervico-Mediastinal Neuroblastoma

Vishal Kapoor,1 Rakesh Lodha,1 and Sandeep Agarwala2*

Neuroblastomas are the most common extra-cranialsolid tumors in children accounting for about 8% of allchildhood cancers [1]. Neuroblastomas can arise in thecervical sympathetic chain or at times as a cervico-mediastinal mass [2]. We report a child having cervico-mediastinal neuroblastoma with unusual clinical features.

CASE REPORT

We illustrate a case of 5-month-old childwho presentedin the emergency department with the complaints of inter-mittent episodes of coughing, respiratory distress withabnormal posturing lasting for a few minutes for 1 month,intermittent cough, hoarseness, stridor, and fever for aweek which was progressively increasing.

On examination the child had heart rate of 200/min andmarked respiratory distress for which the child had to beintubated and mechanically ventilated. The child had a2� 2 cm, smooth and mobile left supraclavicular mass.Puffiness of the face especially the eyes and prominence ofthe veins of the neck increased over the next few days.Chest examination revealed bilateral equal air entry withcrepitations and rhonchi. There was no hepatosplenome-galy or abdominal mass.

Initial investigations revealed hemoglobin 8.6 g/dl andtotal leucocyte counts of 19,200 per mm3 with 80%neutrophils and 20% lymphocytes. The platelet count was5.2� 105 per mm3. The chest X-ray revealed bilateralinfiltrates, elevation of the left hemidiaphragm suggestiveof phrenic nerve palsy, a normal cardiac shadow, andwidened mediastinum (Fig. 1). A possibility of cervico-mediastinal tumor was considered causing superiormediastinal syndrome.

Subsequent examination revealed miosis, and mildptosis of the left eyelid suggestive of Horner syndrome.

The mass in the neck increased in size. The child also hadtachycardia, anxiousness, and raised blood pressure up to140/90 mmHg intermittently.

CT chest revealed a significantly enhancing mass6.5� 4� 5 cm size in the left carotid triangle extendingsuperiorly from the level of hyoid to the main pulmonaryartery inferiorly. The mass was displacing the carotidvessels, superior vena cava and encompassing the neuro-vascular bundle including subclavian vessels, phrenicnerve, and the surrounding structures. Contrast enhancedCT scans of the head and abdomen were normal. Bonemarrow examination did not reveal any tumor involve-ment. Twenty-four hours urinary collection revealedgrossly increased catecholamine levels.

The morphological and immunohistochemistry (MIC2negative, synaptophysin, and chromogranin positive) find-ings on fine needle aspiration cytology of the mass weresuggestive of neuroblastoma. CT angiography confirmedthat the mass was completely encompassing the vesselsand was unresectable (Fig. 2). Whole body nuclear bonescan was within normal limits. MIBG pickup was seenonly in themass described. On the basis of thework up, thetumor was staged as 2B by the International Neuroblas-toma Staging System.

A 5-month-old boy presented with superiormediastinal syndrome due to a large, unresect-able cervico-mediastinal neuroblastoma. Hehad an unusual constellation of findings notpreviously reported in neuroblastoma: Hornersyndrome, phrenic nerve palsy, and palsy of theipsilateral recurrent laryngeal nerve. This con-

stellation of findings in adult malignancies istermed the Rowland–Payne syndrome. Thechild required prolonged mechanical ventila-tion but responded to chemotherapy and is nowfree of tumor and completely recovered. PediatrBlood Cancer 2005;44:280–282.� 2004 Wiley-Liss, Inc.

Key words: intensive care; neuroblastoma; supportive care

——————1Department of Pediatrics, All India Institute of Medical Sciences,

Ansari Nagar, New Delhi, India

2Department of Pediatric Surgery, All India Institute of Medical

Sciences, Ansari Nagar, New Delhi, India

*Correspondence to: Sandeep Agarwala, Department of Pediatric

Surgery, All India Institute of Medical Sciences, Ansari Nagar,

New Delhi 110029, India. E-mail: sandpagr@hotmail.com

Received 6 May 2004; Accepted 9 June 2004

� 2004 Wiley-Liss, Inc.DOI 10.1002/pbc.20198

The child was started on chemotherapy protocol con-sisting of cyclophosphamide—150 mg/m2/day; days 1–7on weeks 1, 3, 6, 13, 17, 21, 29—given intravenously inweek 1 and thereafter orally; cisplatin—90 mg/m2/day;day 1 on weeks 2, 7, 10, 14, 25, 33; doxorubicin—35 mg/m2/day; day 1 on weeks 2, 7, 14, 22, 30; and etoposide—100 mg/m2/day; days 1, 2 on weeks 4, 10, 18, 25, 33. Thechild required mechanical ventilation for 26 days becauseof the pressure effects on the airway from a large cervico-mediastinal mass. Second CT chest done at 1 monthrevealed disappearance of the cervical mass and signifi-cant regression of the mediastinal mass. There was noevidence of Horner syndrome by the second chemother-apy cycle and therewas no stridor or evidence of recurrent

laryngeal nerve palsy at discharge. The child had persis-tence of swallowing difficulty and required nasogastrictube feeding for 3 months. The child responded well withcomplete regression of the tumor mass within 6 months.RepeatMIBGwas done at the end of therapy did not revealany pickup. The patient is presently 23 months of age(18 months since diagnosis) and doing well.

DISCUSSION

Cervical neuroblastomas account for 5% of all neuro-blastomas and generally have a good prognosis [3]. Inone report, of 16 patients with superior mediastinal syn-drome only one had neuroblastoma; the others beinghematological malignancies [4]. In addition the patientscan also have subcutaneous nodules, proptosis, and rarelyopsomyoclonus. These tumors can secrete various neuro-hormones causing intermittent diarrhea, episodic tachy-cardia, and hypertension.

Cervical or cervico-mediastinal masses are known toproduceHorner syndrome either due to a pre-ganglionic ora post-ganglionic sympathetic chain involvement [5–8].In 1981, Payne described a syndrome of association ofHorner syndrome, phrenic nerve palsy, and ipsilateralrecurrent laryngeal nerve palsy in adult patients withmetastatic breast cancer and lung cancer. Similar associa-tion has not been described till now in children with acervico-mediastinal mass [9,10]. It is possible that acervico-mediastinal tumor as a neuroblastoma may pro-duce Rowland–Payne syndrome [11]. It is known thatfeatures ofHorner syndrome can bemasked by high serumepinephrine levels [11]. The Horner syndrome and miosismight have been missed in the initial examination espe-cially as neuroblastoma is often associated with highcirculating catecholamine levels, or simply because itevolved later.

In a patient with stridor and respiratory distress apossibility of mediastinal mass like neuroblastoma shouldbe considered and a cervical extension should be lookedfor. Presence of Horner syndrome and raised hemidiaph-ragm with stridor are unusual findings. Since most ofthe cervico-mediastinal neuroblastomas have a favorableoutcome, it is worthwhile to aggressively manage thesechildren with supportive measures like mechanical ven-tilation, if required, till the tumor regresses withchemotherapy.

REFERENCES

1. Ater JL. Neuroblastoma. In: Behrman RE, Kliegman RM, Jenson

HB, editors. Nelson textbook of pediatrics, 17th edn. Philadelphia:

Saunders; 2004. pp 1709–1710.

2. Smith MC, Smith RJ, Bailey CM. Primary cervical neuroblastoma

in infants. J Laryngol Otol 1985;99:209–214.

3. Moukheiber AK, Nicollas R, Roman S, et al. Primary pediatric

neuroblastic tumors of the neck. Int J Pediatr Otorhinolaryngol

2001;60:155–161.

Fig. 1. Chest radiograph showing elevated left hemidiaphragm.

Fig. 2. CT angiography showing the mass completely encompassing

the major vessels.

Neuroblastoma and Rowland–Payne Syndrome 281

4. IngramL, RiveraGK, Shapiro DN. Superior mediastinal syndrome

associated with childhood malignancy. Med Pediatr Oncol 1990;

18:476–481.

5. Woodruff G, Buncic JR, Morin JD. Horner syndrome in children.

J Pediatr Ophthalmol Strabismus 1988;25:40–44.

6. George ND, Gonzalez G, Hoyt CS. Does Horner syndrome in

infancy require investigation? Br J Opthalmol 1988;82:1097.

7. Gilboa N, Campbell JR. Neuroblastoma in a newborn with

Horner syndrome and respiratory distress. Am J Dis Child 1976;

130:218.

8. Jaffe N, Cassady R, Petersen R. Heterochromia and Horner

syndrome associated with cervical andmediastinal neuroblastoma.

J Pediatr 1975;87:75–77.

9. Payne CM. Newly recognised syndrome in the neck: Horner’s

syndrome with ipsilateral vocal cord paralysis and phrenic nerve

paralysis. JR Soc Med 1981;74:814–818.

10. Amin R. Horner syndromewith ipsilateral phrenic nerve and vocal

cord palsies. Postgrad Med J 1984;60:140–142.

11. Brazis P, Masdeu JC, Biller J. Localization in clinical neurology,

3rd edn. Boston: Little, Brown & Co.; 1996. pp 181–185.

282 Kapoor et al.