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Young patient with generalized lymphangiomatosis: Differentiating the differential

View Article: PubMed Central - PubMed

ABSTRACT

We present the case of a 19-year-old man who was extensively evaluated in multiple centres for long-standing cough, dyspnea, and hemoptysis without a definitive diagnosis. His chest radiograph at presentation showed mediastinal widening, bilateral pleural effusions, and Kerley B lines. Computed tomography of the thorax showed a confluent, fluid-density mediastinal lesion enveloping the mediastinal viscera without any mass effect. There were bilateral pleural effusions, prominent peribronchovascular interstitial thickening, interlobular septal thickening and lobular areas of ground glass density with relative sparing of apices. There were a few dilated retroperitoneal lymphatics and well-defined lytic lesions in the bones. In this case report, we aim to systematically discuss the relevant differentials and arrive at a diagnosis. We also briefly discuss the treatment options and prognosis along with our patient's course in the hospital and final outcome.

No MeSH data available.


Related in: MedlinePlus

Axial (A, B) and coronal (C) CT images in bone window, showing the lytic lesion in sternum (open arrow in A), mottled lucencies with coarse trabecular pattern in vertebrae and pelvic bones (solid arrows in B and C)
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Figure 4: Axial (A, B) and coronal (C) CT images in bone window, showing the lytic lesion in sternum (open arrow in A), mottled lucencies with coarse trabecular pattern in vertebrae and pelvic bones (solid arrows in B and C)

Mentions: A 19-year-old student presented with complaints of productive cough and progressive dyspnea for 18 months and hemoptysis for 6 months. He did not have palpitations, chest pain, or fever. He did not smoke and there was no history of tuberculosis or biomass exposure. He was extensively evaluated in multiple centres and was diagnosed with probable tuberculosis based on computed tomography (CT) findings of probable necrotic mediastinal lymphadenopathy and pericardial effusion. Histopathological examination of CT-guided and mediastinoscopy-guided nodal biopsies and thoracoscopy-guided pericardial and lung biopsies done elsewhere were inconclusive. Bronchoscopy showed inflamed tracheobronchial mucosa. He was treated with empirical anti-tubercular therapy (ATT) for 6 months following which there was no clinical improvement. He required repeated pericardial drainages and, therefore, underwent pleuropericardial shunt insertion for recurrent hemorrhagic pericardial effusions. Because there was no clinical improvement, he presented to our institution for further evaluation. Chest radiograph showed mediastinal widening, bilateral pleural effusions (left more than right), prominent interstitial lung markings, and Kerley B lines [Figure 1]. CT thorax and abdomen with intravenous contrast showed confluent, sheet-like, hypodense mediastinal lesion (HU-28) encasing the mediastinal viscera without mass effect [Figure 2]. No discrete mediastinal lymph nodes could be visualized. There were moderate pericardial and bilateral pleural effusions. Extensive interlobular septal thickening, peribronchovascular interstitial thickening, and patchy, lobular areas of ground glass density were seen in both the lungs with relative sparing of the lung apices [Figure 3]. A few well-defined lytic lesions with prominent primary trabeculae were seen in the sternum [Figures 2 and 4] and a few vertebrae. Mottled lucencies with a coarse trabecular pattern were seen in a few vertebral bodies, a few ribs, and pelvic bones [Figure 4]. Minimal, ill-defined, hypodense soft tissue was also seen in the lesser omentum along with a few dilated retroperitoneal lymphatics [Figure 5]. Lab investigations revealed Iron deficiency anemia, normal total and differential white blood cell counts, negative sputum acid-fast bacilli, and negative XPERT polymerase chain reaction for Mycobacterium tuberculosis. Pleural fluid was hemorrhagic and analysis showed multiple red blood cells, lymphocytosis, elevated lactate dehydrogenase, elevated protein, and low glucose. Pleural fluid culture was negative. Combination of the lung findings (smooth interlobular septal thickening, peribronchovascular interstitial thickening, and lobular areas of ground glass density) and hypodense soft tissue lesion insinuating around the mediastinal viscera made us consider the following differentials: Pulmonary lymphoma, pulmonary veno-occlusive disease (PVOD), Erdheim Chester disease, lymphangiomatosis, and histiocytosis. The fluid-density nature of the mediastinal lesion, lack of infiltration and absence of mass effect on the mediastinal structures made lymphoma less likely. PVOD and Erdheim Chester disease could show similar lung findings, but the mediastinal lesion and the lack of CT findings of pulmonary arterial hypertension excluded PVOD. Erdheim Chester disease typically shows a rind of soft tissue around the kidneys, adrenal fossa, and aorta which were not seen in our patient. Although Langerhans cell histiocytosis (LCH) can also present with multiple lytic lesions in the bones, the lesions in LCH tend to be ill-defined, with adjacent bone marrow edema, periosteal reaction, and enhancing soft tissue.[1] Skeletal lesions in lymphangiomatosis are typically well-defined lytic lesions similar to our case. Finally, generalized lymphangiomatosis was thought to be the most likely diagnosis because of the multi-system involvement (mediastinal, pulmonary and bone involvement, retroperitoneal dilated lymphatics, and pericardial effusion), typical lung findings, and the low-density, nonenhancing nature of the mediastinal lesion. These imaging findings are summarized in Table 1.


Young patient with generalized lymphangiomatosis: Differentiating the differential
Axial (A, B) and coronal (C) CT images in bone window, showing the lytic lesion in sternum (open arrow in A), mottled lucencies with coarse trabecular pattern in vertebrae and pelvic bones (solid arrows in B and C)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5036344&req=5

Figure 4: Axial (A, B) and coronal (C) CT images in bone window, showing the lytic lesion in sternum (open arrow in A), mottled lucencies with coarse trabecular pattern in vertebrae and pelvic bones (solid arrows in B and C)
Mentions: A 19-year-old student presented with complaints of productive cough and progressive dyspnea for 18 months and hemoptysis for 6 months. He did not have palpitations, chest pain, or fever. He did not smoke and there was no history of tuberculosis or biomass exposure. He was extensively evaluated in multiple centres and was diagnosed with probable tuberculosis based on computed tomography (CT) findings of probable necrotic mediastinal lymphadenopathy and pericardial effusion. Histopathological examination of CT-guided and mediastinoscopy-guided nodal biopsies and thoracoscopy-guided pericardial and lung biopsies done elsewhere were inconclusive. Bronchoscopy showed inflamed tracheobronchial mucosa. He was treated with empirical anti-tubercular therapy (ATT) for 6 months following which there was no clinical improvement. He required repeated pericardial drainages and, therefore, underwent pleuropericardial shunt insertion for recurrent hemorrhagic pericardial effusions. Because there was no clinical improvement, he presented to our institution for further evaluation. Chest radiograph showed mediastinal widening, bilateral pleural effusions (left more than right), prominent interstitial lung markings, and Kerley B lines [Figure 1]. CT thorax and abdomen with intravenous contrast showed confluent, sheet-like, hypodense mediastinal lesion (HU-28) encasing the mediastinal viscera without mass effect [Figure 2]. No discrete mediastinal lymph nodes could be visualized. There were moderate pericardial and bilateral pleural effusions. Extensive interlobular septal thickening, peribronchovascular interstitial thickening, and patchy, lobular areas of ground glass density were seen in both the lungs with relative sparing of the lung apices [Figure 3]. A few well-defined lytic lesions with prominent primary trabeculae were seen in the sternum [Figures 2 and 4] and a few vertebrae. Mottled lucencies with a coarse trabecular pattern were seen in a few vertebral bodies, a few ribs, and pelvic bones [Figure 4]. Minimal, ill-defined, hypodense soft tissue was also seen in the lesser omentum along with a few dilated retroperitoneal lymphatics [Figure 5]. Lab investigations revealed Iron deficiency anemia, normal total and differential white blood cell counts, negative sputum acid-fast bacilli, and negative XPERT polymerase chain reaction for Mycobacterium tuberculosis. Pleural fluid was hemorrhagic and analysis showed multiple red blood cells, lymphocytosis, elevated lactate dehydrogenase, elevated protein, and low glucose. Pleural fluid culture was negative. Combination of the lung findings (smooth interlobular septal thickening, peribronchovascular interstitial thickening, and lobular areas of ground glass density) and hypodense soft tissue lesion insinuating around the mediastinal viscera made us consider the following differentials: Pulmonary lymphoma, pulmonary veno-occlusive disease (PVOD), Erdheim Chester disease, lymphangiomatosis, and histiocytosis. The fluid-density nature of the mediastinal lesion, lack of infiltration and absence of mass effect on the mediastinal structures made lymphoma less likely. PVOD and Erdheim Chester disease could show similar lung findings, but the mediastinal lesion and the lack of CT findings of pulmonary arterial hypertension excluded PVOD. Erdheim Chester disease typically shows a rind of soft tissue around the kidneys, adrenal fossa, and aorta which were not seen in our patient. Although Langerhans cell histiocytosis (LCH) can also present with multiple lytic lesions in the bones, the lesions in LCH tend to be ill-defined, with adjacent bone marrow edema, periosteal reaction, and enhancing soft tissue.[1] Skeletal lesions in lymphangiomatosis are typically well-defined lytic lesions similar to our case. Finally, generalized lymphangiomatosis was thought to be the most likely diagnosis because of the multi-system involvement (mediastinal, pulmonary and bone involvement, retroperitoneal dilated lymphatics, and pericardial effusion), typical lung findings, and the low-density, nonenhancing nature of the mediastinal lesion. These imaging findings are summarized in Table 1.

View Article: PubMed Central - PubMed

ABSTRACT

We present the case of a 19-year-old man who was extensively evaluated in multiple centres for long-standing cough, dyspnea, and hemoptysis without a definitive diagnosis. His chest radiograph at presentation showed mediastinal widening, bilateral pleural effusions, and Kerley B lines. Computed tomography of the thorax showed a confluent, fluid-density mediastinal lesion enveloping the mediastinal viscera without any mass effect. There were bilateral pleural effusions, prominent peribronchovascular interstitial thickening, interlobular septal thickening and lobular areas of ground glass density with relative sparing of apices. There were a few dilated retroperitoneal lymphatics and well-defined lytic lesions in the bones. In this case report, we aim to systematically discuss the relevant differentials and arrive at a diagnosis. We also briefly discuss the treatment options and prognosis along with our patient's course in the hospital and final outcome.

No MeSH data available.


Related in: MedlinePlus