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Pancoast syndrome: A rare presentation of non-Hodgkin's lymphoma

Article  in  Lung India · July 2013

DOI: 10.4103/0970-2113.116266 · Source: PubMed

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Case Report

Pancoast syndrome: A rare presentation of non‑Hodgkin’s

lymphoma
Anirban Sarkar, Anirban Das1, Sumitra Basuthakur1, Sudipta Pandit1, Sibes K. Das1, Sabyasachi Choudhury1
Department of Pulmonary Medicine, Midnapore Medical College, Paschim Midnapore, 1Department of Pulmonary Medicine,
Medical College, Kolkata, West Bengal, India

ABSTRACT

Pancoast syndrome is a common presentation of bronchogenic carcinoma, but other malignancies are rarely cited as
its cause. Pancoast syndrome due to non‑Hodgkin’s lymphoma is rarely described in the literature. Here, we report
a case of Pancoast syndrome due to non‑Hodgkin’s lymphoma to increase the awareness of the clinicians regarding
essentiality of tissue diagnosis of Pancoast tumor before starting the treatment.

KEY WORDS: Computed tomography, non‑Hodgkin’s lymphoma, Pancoast syndrome, tru‑cut biopsy

Address for correspondence: Dr. Anirban Das, C/O Mr. Pradip Das, Peon Para, Bhatchala, Near Vidyarthi Girls’ High School, P.O.: Sripalili, P.S.: Burdwan
Sadar, Burdwan, West Bengal, India. E‑mail: dranirbandas_chest@rediffmail.com

INTRODUCTION CASE REPORT

Pancoast syndrome is characterized by the shoulder and
arm pain radiating along the distribution of eighth cervical,
first and second thoracic nerve trunks, weakness and
atrophy of the intrinsic hand musculature, and ipsilateral
Horner’s syndrome. It is most commonly caused by local
extension of an apical lung tumor at the superior thoracic
inlet, most common histopathological type being squamous
cell carcinoma.[1] These tumors are called Pancoast tumor
or Superior sulcus tumor. In 1932, Henry Pancoast
first described the clinic‑radiological features of the
thoracic inlet tumors, but he mistakenly thought that it
originates from embryonal epithelial rests of fifth brachial
cleft.[2] Among malignant etiologies other than lung cancer,
lymphoma, especially non‑Hodgkin lymphoma (NHL) is
reported occasionally to cause Pancoast syndrome. Here, we
report such a rare case of anterior mediastinal NHL, which
extended to the neck to produce the Pancoast syndrome
due to involvement of the lower trunk of brachial plexus
and inferior cervical ganglion.
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DOI:
10.4103/0970-2113.116266
A 16‑year‑old female presented with a painful swelling
on the left side of the neck for 2 months, and gradually
increasing weakness and numbness of left upper limb with
drooping of the left an upper eye lid for 1 month. She had
sharp, shooting pain in the neck and left shoulder, radiating
along the inner aspect of the left arm, associated with tingling
and numbness of her left forearm extending to the tips of her
fourth and fifth fingers, ulnar aspect of the forearm and hand.
The pain was not relieved by simple analgesic and her sleep
was disturbed because of intense pain. Neck swelling was
irregular in shape and progressively increasing in size; initial
size was 2 cm × 3 cm. There was no history of fever, weight
loss, and anorexia. She was normotensive and non‑diabetic.
There was no past history of the tuberculosis.
On general examination, there was a firm, tender, irregular
swelling of 4 cm × 4 cm size in the left side of the neck
extending to left supraclavicular area. The swelling was
fixed to underlying structures and lower margin was not
delineated. Temperature was not raised and overlying skin
was normal. Fluctuation was absent and transillumination
test was negative. There was no other peripheral swelling
in the body. Clubbing and anemia were absent and her
axillary temperature was 97°F, pulse rate 100 beats/min,
respiratory rate 20 breaths/min, and the blood pressure
was 110/70 mmHg.
On examination of the respiratory system, the movement
of the chest wall was restricted over left supraclavicular

Lung India • Vol 30 • Issue 3 • Jul - Sep 2013 209

 Sarkar, et al.: Non‑Hodgkin lymphoma presenting as Pancoast syndrome

and infracalvicular areas along with fullness in the same

areas. There was no venous engorgement over the chest
wall. Trachea was shifted to the right without shifting of
cardiac apex. Percussion note was dull over left second and
third intercostal spaces and sternal percussion was also
dull. Diminished vesicular breath sound and decreased
vocal resonance were noted over left supraclavicular and
infraclavicular areas.
Figure 1: Contrast enhanced computed tomography thorax showing
Eye examination revealed the partial ptosis of left upper a huge, heterogeneous anterior mediastinal mass extending to neck
eye lid with ipsilateral miosis, but the vision was preserved through the thoracic inlet
with normal light and accommodation reflexes. Ocular
movement was normal in all directions.

On neurological examination of upper extremity, the

hypothenar muscles of the left hand were wasted. There
was a decrease in power of all muscle groups in this hand
(grade 2/5). Fine touch and pain sensation were decreased
in the C8 and T1 segmental distribution in his left hand
and arm. Triceps jerk was diminished.

Complete hemogram and blood biochemistry were

normal, except elevated serum lactate dehydrogenase level
(340 U/L). Chest X‑ray (P.A. view) showed a huge anterior
mediastinal mass, compressing left lung and extending to
the thoracic inlet, but no rib erosion. Sputum smear for acid
fast bacilli was negative. Incisional biopsy from the neck
swelling was inconclusive. Contrast enhanced computed
tomography (CT) thorax showed heterogeneous huge Figure 2: Photomicrograph of computed tomography‑guided tru‑cut
anterior mediastinal necrotizing mass extending through biopsy specimen taken from anterior mediastinal mass showing
thoracic inlet to the neck, but no rib erosion [Figure 1]. effacement of normal architecture of lymph node with sheets of atypical
CT guided fine needle aspiration cytology (FNAC) was large lymphoid cells (H and E, ×40)
inconclusive. Excision biopsy from the left supraclavicular
swelling revealed malignant round cell tumor involving
lymph nodes. Histopathological examination of CT guided
tru‑cut biopsy of mediastinal mass showed effacement of
normal architecture of lymph gland, which was replaced
by sheets of atypical large lymphoid cells admixed
with the histiocytes and plasmacytoid cells. Scattered
immunoblasts were present. No Reed Sternberg cell or
its variant was found [Figure 2]. Hence, histopathology
ultimately confirmed the tissue diagnosis of NHL.
Immunohistochemistry further substantiated the diagnosis
of diffuse large B‑cell phenotype of NHL, as the tumor cells
expressed CD20 and CD10, but were immunonegative
for CD3, CD5, CD23, and CD30 [Figure 3]. Bone marrow
biopsy was within normal limit. Ultrasound of whole
abdomen was normal. Hence, he was finally diagnosed as
a case of diffuse large B‑cell phenotype of NHL. The size Figure 3: Immunohistochemistry showing CD20 positive tumor cells
of the lesion regressed following six cycles of combination
chemotherapy, comprising of cyclophosphamide, invade the second and third ribs, the brachial plexus, the
doxorubicin, vincristine, and prednisolone. subclavian vessels, the stellate ganglion, and adjacent
vertebral bodies, and therefore prognosis is definitely poor.
DISCUSSION Multiple myeloma, metastatic uterine cervical carcinoma,
metastatic hepatocellular carcinoma, and lymphomatoid
Pancoast syndrome is rare and primary bronchogenic granulomatosis are other malignant causes of may cause
carcinoma, located at pulmonary apex is the most common Pancoast’s syndrome.[3‑7] Lymphoma, especially NHL is
etiology, which is a rare location accounting for less than rarely reported as being a cause of Pancoast syndrome.[8]
5% of all bronchogenic carcinomas.[3] These tumors may Benign causes of Pancoast syndrome include pulmonary

210 Lung India • Vol 30 • Issue 3 • Jul - Sep 2013

 Sarkar, et al.: Non‑Hodgkin lymphoma presenting as Pancoast syndrome

tuberculosis, hydatid cyst, histoplasmosis, aspergillosis, REFERENCES

destructive sclerosing fibrosis following staphylococcal
infection at lung apex.[9‑12] Pancoast syndrome is due to
involvement of the lower trunk of brachial plexus and
inferior cervical ganglion by locally progressive pulmonary
apical tumors, which often cause erosion of upper ribs and
chest wall destruction before neural involvement.
Brachial neuritis has also been described as a paraneoplastic
syndrome of lymphoma, especially of Hodgkin’s variety.[13]
However, our case is an extremely rare case of diffuse large
B‑cell NHL, presenting as an anterior mediastinal mass
and causing Pancoast syndrome.
Transthoracic lung biopsy may be performed using
the fine needle aspiration (FNA) technique or using
cutting needle for core biopsy. FNA provides material
for cytological analysis and is sometimes sufficient for
diagnosis of primary or metastatic lung cancer, but core
biopsy is often required to provide the material for the
histopathological examination and is often useful for a
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lung lesions, lymphomas and to distinguish mesothelioma
from adenocarcinoma. It also provides sufficient sample for
microbiological culture and immunohistochemistry. Most
diagnosis of malignancy requires more tissue than FNAC
can provide and it often delays a definitive diagnosis.
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This case report not only illustrates a rare cause of Pancoast
syndrome, but also reemphasizes the importance of
the tissue diagnosis in all cases of Pancoast tumor as it
significantly alters the treatment options and prognosis.
How to cite this article: Sarkar A, Das A, Basuthakur S, Pandit S,
Das SK, Choudhury S. Pancoast syndrome: A rare presentation of
non-Hodgkin’s lymphoma. Lung India 2013;30:209-11.
Source of Support: Nil, Conflict of Interest: None declared.

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