Bronchial tumors, multi-organ invasions! But not malignant, not benign...

*For medical professionals only

What exactly is this disease?

New bronchial organisms, accompanied by tumors of the same type in other organs, are generally malignant tumor invasions
.
However, today I share with you a miraculous case
.
This tumor will invade many organs, but it is not a malignant tumor, and it is difficult to say that it is a benign tumor
.

What is the magic disease?

Case introduction 39-year-old female with dry cough for 2 years
.
Current medical history: dry cough for 2 years, occasional wheezing, denial of symptoms of runny nose, conjunctivitis, postnasal drip or reflux, denial of recent weight loss, hemoptysis, fever, chills, night sweats, denial of fatigue, easy bruising, muscle weakness or thinning of
the skin 。 Previous history: systemic lupus erythematosus with end-stage renal disease, 16-year prior kidney transplantation, immunosuppressive regimen: prednisone 5 mg once daily, tacrolimus 3 mg once daily, mycophenolate mofetil 500 mg once daily; Prevention of infection: valacyclovir 500 mg 2 times a day; Treatment of hypertension: clonidine 0.
1 mg once a day, metoprolol 100 mg 2 times
a day.
Never smoke.

No history of
international travel.
Physical examination: normal vital signs, oxygen saturation in the breathing chamber air 97%, clear consciousness, no rash, no jugular venous distension, no obvious abnormalities on cardiac examination, lung auscultation shows a slight inspiratory wheezing sound in the left dorsal lobe, no lower extremity edema, no cyanosis
.
Adjunctive examination: CT of the chest shows a left lower lobe bronchial nodule
.
Figure 1
CT of the abdomen shows an adrenal nodule
.
Figure 2
Bronchoscopy shows a new organism with a bronchial opening in the lower left lobe
.
Figure 3
Bronchial neoorganism + adrenal nodules, in general, highly suggestive of malignant metastasis
.
Will patients with long-term oral immunosuppressants be a special infection?


Diagnosis and treatment process

A hard bronchoscopy is performed, and the electric snare device completely excises the new organism
.

Figure 4

Left-sided adrenalectomy
.

In situ hybridization of bronchial neoorganisms detected small RNAs encoded by Epstein-Barr virus, and bronchial neoorganisms + adrenal specimens pathology showed leiomyoma
.

Final diagnosis: Epstein-Barr virus-associated leiomyomas involve both the bronchial lining and the adrenal glands
.

Epstein-Barr virus-associated leiomyoma, benign? Malignant?

Epstein-Barr virus-associated leiomyomas (EBV SMTs) are rare mesenchymal tumors found only in immunocompromised hosts and are divided into three separate clinicopathological subtypes depending on the cause of immunodeficiency: HIV-related, post-transplant, and congenital immunodeficiency-associated leiomyomas
.

Angstrovirus-associated leiomyomas after organ transplantation, which usually appear later, have a median time of 4 years after transplantation, about 50% are multi-organ invasions, and are more likely to develop tumors in organs with less smooth muscle, with kidney transplantation most likely to appear
.
Serologic testing is useless, and the most reliable is that in situ hybridization detects small RNAs encoded by EBV (not simply EBV is detected
).

Berke-associated leiomyoma after organ transplantation, it is unclear whether it is benign or malignant
.
There is no correlation
between the histological presentation of the tumor and the clinical outcome.
Instead, studies have shown that the biological behavior of a tumor depends on the patient's immune status, not on the histological characteristics
of the tumor.

Because the disease is so rare, there is no recognized optimal treatment
.
It is recommended to first improve the patient's immune function
.
When the tumor impairs organ function, surgery or endoscopic resection should be performed
.
In some cases, chemoradiation has been shown to be effective
.
Overall, the prognosis is good, and few patients die from the direct effects
of the tumor.
Poor prognosis is mainly associated
with intracranial symptoms and unresectable tumors.

Follow

After resection, the patient's immunosuppressive regimen
is adjusted.
The patient's chronic cough disappears
.
CT follow-up of 3 months after surgery showed no signs of
tumor recurrence.
If renal function deteriorates again, another kidney transplant
is necessary.

More practical knowledge of respiratory practice? Come to the "Doctor Station APP" to take a look 👇

References:

[1] Harpreet,et al.
A 39-Year-Old Woman With Synchronous Endobronchial and Adrenal Tumors.
[J].
Chest,2021,160(6):e629-e632.
DOI:10.
1016/j.
chest.
2021.
07.
028.
Source of this article: Medical Respiratory Channel This article author: Sun Danxiong Responsible editor: Peng Jianping Dai Dai
copyright declaration
original This article reprint, please contact the authorization- End - Call for "Medical Respiratory Channel" Long-term recruitment online author, 1.
Respiratory Medicine Clinical Practical and interesting content; 2.
Interesting diagnosis and treatment stories of respiratory physicians; 3.
Heavy progress in the respiratory medicine industry; 4.
Others (content of interest to the respiratory physician is sufficient).



Welcome to share the manuscript! We will provide you with competitive fees and a platform
to showcase your talents.
Submission Email: daijiaxing@yxj.
org.
cn (Submissions must be replied to)
* The medical community strives to be accurate and reliable in the review of the published content, but does not make any commitment and guarantee as to the timeliness of the published content, as well as the accuracy and completeness of the cited materials (if any), nor does it assume any responsibility
arising from the fact that such content is outdated, the cited materials may be inaccurate or incomplete, etc.
Relevant parties are invited to verify separately when adopting or using this as a basis for decision-making
.

↓↓↓ Click "Read the original article" to learn more clinical skills