Journal de Mycologie Médicale 29 (2019) 80–83

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Case report

A case of pulmonary mucormycosis presented as Pancoast syndrome

and bone destruction in an immunocompetent adult mimicking lung
carcinoma
J. Yang a, J. Zhang a, Y. Feng a, F. Peng b, F. Fu a,*
a
Department of Radiology, Zhejiang Hospital, 12, Lingyin road, 310013 Hangzhou, China
b
Department of Pathology, Zhejiang Hospital, 12, Lingyin road, 310013 Hangzhou, China

A R T I C L E I N F O A B S T R A C T

Article history: Pulmonary mucormycosis is a rare opportunistic infection caused by Mucormycosis. This fungal
Received 31 December 2017 infection is uncommon in immunocompetent individuals. Because of its various clinical and imaging
Accepted 29 October 2018 manifestations, it is a diagnostic challenge to distinguish pulmonary mucormycosis from other
Available online 12 December 2018
pulmonary diseases, such as carcinoma. Herein, we report a case of pulmonary mucormycosis presenting
as Pancoast syndrome and bone destruction of ribs. A 46-year-old Chinese woman was admitted due to
Keywords: pain in chest, right neck and arm for four months and hoarseness for one week. The pre-admission
Pulmonary mucormycosis
diagnosis via chest CT was pulmonary carcinoma. The subsequent bronchoalveolar lavage fluid analysis
Immunocompetent
Pancoast syndrome
and bronchoscopic biopsy were negative for malignant cells, except chronic inflammation. Imaging-
Computed tomography guided percutaneous biopsies were carried out after admission and the final pathological diagnosis was
pulmonary mucormycosis. Although the patient was started on oral posaconazole of 400 mg bid, the
disease condition continued to deteriorate. She finally died of respiratory failure.
C 2018 The Authors. Published by Elsevier Masson SAS. This is an open access article under the CC BY-NC-

ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Pancoast syndrome and bone destruction of ribs in an immuno-

competent adult.
1. Introduction

Pulmonary mucormycosis is the common form of infection 2. Case report

caused by Mucorales speciesa, with a mortality rate of 50%–70%
[1]. Dysfunction in cellular immunity renders individuals more
susceptible to this fungal infection. However, immunocompetent
hosts with pulmonary mucormycosis have been increasingly
reported in recent years. The clinical and radiological features of
pulmonary mucormycosis were non-specific and similar to
alterations of other pulmonary diseases. Pancoast syndrome is
characterized by Horner syndrome, shoulder pain radiating down
the arm, compression of the brachial blood vessels, and atrophy of
the arm and hand muscles [2]. Pancoast syndrome or bone
destruction is more prevalent in primary lung cancer, and rare in
certain infections, including mucormycosis [2]. In the following
case, we present the pulmonary mucormycosis manifested as
Abbreviations: CT, Computer tomography; PAS, Periodic acid-Schiff.
* Corresponding author.
E-mail addresses: jhtc6668@163.com (J. Yang), zhangyp31113@163.com
(J. Zhang), fengyue1974@gmail.com (Y. Feng), pengfang999@139.com (F. Peng),
fufengli1984@163.com (F. Fu).
In February 2016, a 46-year-old Chinese woman was admitted
into Zhejiang Hospital. She complained of intermittent blunt pain
in chest, persistent distended pain in right neck and arm four
months ago with no reasons. One week prior to admission into our
hospital, the patient’s disease condition was deteriorative coupled
with hoarseness, dysphagia and anhelation after activities. She
denied diabetes and use of alcohol, tobacco, intravenous drugs and
steroid. On admission, the patient was ill appearing with obvious
swollen right chest wall and arm. She had a temperature of 37.5 8C,
a pulse rate of 91 beats/min, a respiratory rate of 25 breaths/min
and a blood pressure of 128/70 mmHg. The laboratory test results
on admission were shown in Table 1. Human immunodeficiency
virus antibody test was negative.
Chest CT showed a large soft tissue mass (9.5 cm  8.1 cm) in
right upper lobe, several nodules (diameter, 0.8 cm–1.3 cm) of left
lung (Fig. 1A), swollen right chest wall and bone destruction of
right 1st and 2nd ribs along with right pleural effusion (Fig. 1B).
Contrast-enhanced CT examination demonstrated that there were

https://doi.org/10.1016/j.mycmed.2018.10.005
1156-5233/ C 2018 The Authors. Published by Elsevier Masson SAS. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-

nd/4.0/).
 J. Yang et al. / Journal de Mycologie Médicale 29 (2019) 80–83 81

Table 1 mucormycosis (Fig. 3). Mucormycosis frequently occurs in the

Laboratory results.
patients with hematological malignancies. She was given bone
Test Result Reference range marrow puncture to determine whether there were abnormalities
9
White blood cell count (10 /L) 12.6 4.00–10.00 in blood system. There was no evidence for hematological
Hemoglobin (g/L) 100 130–175 malignancies in bone marrow smears. The patient was not a
Platelet count (109/L) 530 125–350 candidate for surgical intervention due to both the unstable
C-reactive protein (mg/L) 185.25 <3 condition and multiple tissues involvement. She was started on
Albumin (g/L) 29.19 35.2–52.0
oral posaconazole of 400 mg bid. However, her disease condition
Globulin (g/L) 43.48 20–40
Alanine aminotransferase (U/L) 103.6 5–35 continued to progress after anti-fungal therapy and other
Aspartate aminotransferase (U/L) 99.1 13–35 supportive treatment. On day 14 of anti-fungal treatment, she
Glutamgtranspeptidaser (U/L) 585 7–32 experienced tachypnea, hypotension at 80/57 mmHg and systemic
Alkaline phosphatase (U/L) 410.6 35–100
cyanosis with oxygen saturation of 57%. Arterial blood gas analysis
Glucose (mmol/L) 5.14 3.90–6.10
Prothrombin time (S) 13.1 9.4–12.5
showed metabolic acidosis, pH 7.37, pCO2 59.0 mmHg, pO2
Fibrinogen (g/L) 7.15 2.38–4.98 69.1 mmHg. The first-aid measures and endotracheal intubation
International normalized ratio 1.18 0.85–1.15 were given accordingly. She was transferred into the ICU
Plasma levels of d-dimer (mg/)L 0.96 0.00–0.30 subsequently due to aggravated respiratory failure requiring
Alpha-fetal protein (ng/mL) 2.66 0.00–9.00
assisted ventilator treatment. The therapies in ICU included
Carcinoembryonic antigen (ng/mL) 1.27 0.00–5.00
Ferritin (ng/mL) 720.8 11.00–306.80 antibiotics, anti-fungal and circulation function and nutrition
Carbohydrate antigen 12–5 (U/mL) 306.1 0.00–35.00 support treatment. Unfortunately, the patient’s condition contin-
Carbohydrate antigen 15–3 (U/mL) 24.18 0.00–25.00 ued to deteriorate (Fig. 4). She and her husband denied the
Carbohydrate antigen 19–9 (U/mL) 4.52 0.00–39.00
continued treatment and requested discharge after 4 days in ICU.
She finally died of respiratory failure.

thickened arteries of chest wall (Fig. 2A), necrosis in the tumor and
multiple enlarged lymph nodes in mediastinum, right armpit and
clavicular region (Fig. 2B). The filling defects seen in right internal
jugular vein and right subclavicular vein (Fig. 2C) were identified as
thrombosis by ultrasound (Fig. 2D). Throat swab and sputum
cultures for bacteria and fungus were repeatedly obtained, but no
pathogens were found. Sputum smears were negative for acid-fast
bacilli and fungi. According to the imaging findings and abnormal
tumor marker, the initial diagnosis was lung cancer accompanied
with metastasis. Fiberoptic bronchoscopy revealed mucosal edema
of right upper lobe bronchus and luminal stenosis of apical
segmental bronchus, which was obstructed by necrotic material.
Bronchoalveolar lavage fluid analysis and bronchoscopic biopsy
were negative for malignant cells, except chronic inflammation.
The brain magnetic resonance imaging and abdominal CT imaging
were normal. During hospitalization at respiratory department,
she was treated with the anti-infective, anticoagulant, and liver-
protecting therapies.
Imaging-guided needle biopsies were performed for further
etiology. The first CT-guided percutaneous biopsy of mass in right
lung was conducted. Lung biopsy revealed extensive necrosis and
slight chronic inflammation of epithelial tissue. The CT-guided
pathological reexamination of the mass in both lung and
subsequent bilateral supraclavicular lymph nodes under ultra-
sound showed extensive necrosis and few scattered broad non-
septate hyphae with right angle branching, consistent with
3. Comments
Pulmonary mucormycosis begins with the inhalation of fungi
spores into the bronchioles and alveoli, which typically results in
the rapid progression of pneumonia or endobronchial disease
[3]. The first pulmonary mucormycosis was described by Furbinger
et al. in 1876 [4]. In recent years, pulmonary mucormycosis has
emerged as an important opportunistic mycosis in immunocom-
promised patients. Majority of patients are accompanied with
predisposing factors, such as diabetes mellitus (with or without
ketoacidosis), hematological malignancies, chemotherapy, chronic
renal failure, pharmacologic immunosuppression, post-transplan-
tation, HIV infection, iron overload, trauma or burns [1,3,5]. Only
few cases of pulmonary mucormycosis were reported in immuno-
competent hosts [5–8]. No more than 15 cases have been reported.
There is no evidence of immune dysfunction and extra-pulmonary
organ involvement in the case we reported here. Therefore, it is
considered as solely lung-involved mucormycosis in an immuno-
competent individual. The etiology of this rare disease condition is
not yet clear. In our case, the patient farmed in her life for more
than twenty years. Zhang et al. [5] suggested that farming might
predispose patients to mucormycosis. This finding possibly reflects
the fact that fungi in the order of Mucorales often exist in decaying
vegetation and in soil [5].
The clinical and imaging features of pulmonary mucormycosis
manifest as different forms. Pulmonary symptoms usually present

Fig. 1. Non-contrast computed tomography. (A) Lung and (B) mediastinal window showed a large mass (9.5 cm  8.1 cm) in right upper lobe, one nodule (diameter, 1.3 cm) of
left lung, swollen right chest wall and bone destruction in right first rib (arrowhead) along with right pleural effusion.
82 J. Yang et al. / Journal de Mycologie Médicale 29 (2019) 80–83

Fig. 2. Contrast-enhanced computed tomography. (A) Arterial phase CT imaging revealed collateral arteries in chest wall. (B) Large areas of necrosis in the tumor and many
enlarged lymph nodes in mediastinum and right armpit region were showed in venous phase imaging. (C) Right internal jugular vein and right subclavicular (arrow) vein
were filled with thrombosis, (D) which was identified by ultrasound.

Fig. 3. Histopathology. Histopathology showed broad, nonseptate and ribbon-like hyphae with right angle branching (arrowhead), (A, HE  100), (B, HE  400) and
(C, PAS  100).

as common respiratory symptoms such as fevers, cough, chest pain
and dyspnoea. Based on the retrospective analysis of English
literature, the multiple radiologic changes of chest include
consolidation of lung, cavity resembling tuberculosis, solitary or
multiple pulmonary nodules of fungal ball, bronchopleural
fistulae, abscess, pneumonia and pleural effusion [3,5–8]. However,
above clinical and imaging manifestations are non-specific even at
late stages of infection [3]. In our case, the patient had no
symptoms of fever, dyspnea and coughing until her condition
deteriorated after several months. Pancoast syndrome is most
commonly caused by a bronchogenic carcinoma in the superior
sulcus of the lung with destructive lesions of the thoracic inlet and
invasion of the brachial plexus and cervicothoracic sympathetic
chain [2]. Bone destruction often occurs in the advanced stage of
neoplastic diseases. To our knowledge, pulmonary mucormycosis
associated with Pancoast syndrome and bone destruction has not
been reported so far, which may lead to the misdiagnosis.
The definitive diagnosis of pulmonary mucormycosis relies on
the identification of mucoraceus hyphae in affected tissues [3],
including body fluid culture, needle biopsy and resected lung
tissue biopsy. The fungal culture test of specimen is not often done
in the laboratory because of time-consuming and a low rate of
positive cultures [9]. Histopathological evaluation with affected
tissues remains the primary diagnostic criterion. The pathological
characteristic is broad (diameter, 6–16 mm), aseptate and ribbon-
like hyphae with right angles branching. Mucormycosis infections
can lead to extensive angioinvasion, which results in vessel
thrombosis and subsequent tissue necrosis [9]. This trait may
 J. Yang et al. / Journal de Mycologie Médicale 29 (2019) 80–83
Fig. 4. Chest X-ray. A. Before ICU admission, i.e., on day 14 after antifungal treatment. B. Two days later after admission to ICU.
hamper the acquisition of tissue specimen and establishment of a
definite diagnosis. Special tissue stains, such as Grocott methena-
mine silver and Periodic acid-Schiff (PAS) stain, can contribute to
identify the hypha from tissue debris.
The prognosis and outcome of pulmonary mucormycosis
depend on the positive activity against Mucorales aside from
early diagnosis. The primary therapies include timely antifungal
therapy (amphotericin B and posaconazole), surgical debridement
of necrotic tissue and reversal of underlying conditions. Delays in
the administration of effective systemic antifungal therapy within
the first few days of diagnosis increase the probability of patient
death due to disseminated infection [10]. In our case, the absence
of special symptoms led to a delay in seeking health service
(patient delay). Another possible reason of mortality may be
caused by the lack of effective treatment. Ischemic necrosis of
infected tissues can prevent delivery of leukocytes and antifungal
agents to the foci of infection [9]. The combination of surgical and
antifungal therapy may provide a better survival outcome than
medical therapy alone [2].
The infection caused by mucormycosis is uncommon in an
immunocompetent adult. Pulmonary mucormycosis presented as
Pancoast syndrome and bone destruction of ribs is not only
extremely rare but also easily misdiagnosed as lung cancer. It is
important that the clinician maintain vigilance against the rare
presentations of pulmonary mucormycosis in future.
Consent
Written informed consent was obtained from the patient’s next
of kin for publication of this case report and accompanying images.
A copy of the written consent is available for review by the editor-
in-chief of this journal.
Funding
This work was supported by Zhejiang Provincial administration
of Traditional Chinese Medicine (2018ZA001); Zhejiang Provincial
Medical and Health Research Fund Project under Grant
(2017KY183); and Zhejiang Provincial Natural Science Foundation
under Grant (LY15H180009).
83
Contributions of authors
J.H.Y. and Y.F. collected and analyzed the imaging data, drafted
the manuscript. J.J.Z. carried out needle biopsy. F.P. performed
histological analysis. F.L.F. participated in the design and
coordination of the manuscript, and conducted final edits of the
manuscript. All authors read and approved the final form
manuscript.
Disclosure of interest
The authors declare that they have no competing interest.
Acknowledgements
The authors appreciate all participants who were involved in
the management of the patient and the preparation of the
manuscript.
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