A case report on rapidly worsening anterior mediastinal mass with metastasis

Deepa. S¹, Subathra Devi. M², Maha Lakshmi³

¹Nursing Supervisor, 1win, Cantonment, Trichy

²Nurse Educator, 1win, Cantonment, Trichy

³Nursing Superintendent, 1win, Cantonment, Trichy

Abstract

T-cell lymphoblastic lymphoma (T-LBL) is an aggressive, high-grade non-Hodgkin lymphoma primarily affecting adolescents and young adults, although it can occur at any age. It arises from precursor T lymphocytes and is closely related to T-cell acute lymphoblastic leukemia (T-ALL), differing mainly in its presentation as a solid mass rather than widespread hematologic involvement. Clinically, T-LBL often presents with rapidly enlarging lymphadenopathy, mediastinal masses, or extra nodal involvement, including the skin, gastrointestinal tract, and central nervous system. Diagnosis is confirmed through a combination of histopathological examination, immunophenotyping, and molecular studies, with tumor cells typically expressing T-cell markers (e.g., CD3, CD7) and markers of immaturity such as TdT (terminal deoxynucleotidyl transferase). Treatment usually involves aggressive chemotherapy regimens, often adapted from protocols used for T-ALL, and may include radiation therapy or stem cell transplantation for high-risk cases. Despite intensive treatment, the prognosis remains poor for some patients, particularly those with advanced-stage disease or inadequate response to initial therapy. Ongoing research aims to improve understanding of the molecular mechanisms underlying T-LBL and develop novel therapeutic strategies, including targeted therapies and immunotherapy, to improve outcomes in affected patients.

Background. T-LBL usually presents with rapidly enlarging masses, particularly in the mediastinum, which is the most common site of involvement. Lymphadenopathy, pleural effusions, and extra nodal involvement (including skin, gastrointestinal tract, and central nervous system) are also frequently observed. The disease is aggressive, and patients often present with advanced-stage disease at the time of diagnosis. As T-LBL is part of the group of lymphoblastic lymphomas, it is associated with a high proliferative index, which contributes to its aggressive nature.

Histologically, T-LBL is characterized by a diffuse infiltration of immature, large lymphoid cells that resemble early T-lymphocyte precursors. Immunophenotypically, these tumor cells typically express T-cell markers such as CD3, CD7, and CD5, and are positive for terminal deoxynucleotidyl transferase (TdT), a marker of lymphoid progenitor cells. Genetic studies may reveal chromosomal translocations, most notably involving the TCR (T-cell receptor) loci or mutations in the NOTCH1 and FBXW7 genes, which are often associated with poor prognosis.

The clinical presentation and diagnostic approach to T-LBL are similar to those of T-ALL, though the latter usually involves the bone marrow and peripheral blood, whereas T-LBL typically manifests as a localized solid tumor. Given its clinical and molecular similarities with T-ALL, treatment regimens for T-LBL often follow those used in T-ALL, involving intensive chemotherapy, often in combination with radiation therapy. Stem cell transplantation may be considered for patients with high-risk disease or those with relapsed disease.

Despite intensive treatment strategies, the prognosis for T-LBL remains guarded, with long-term survival rates varying significantly based on factors such as age, stage at diagnosis, response to therapy, and the presence of high-risk molecular features. Advances in the understanding of the molecular biology of T-LBL are opening up potential avenues for more targeted therapies, including immunotherapy and gene therapy, which may improve treatment outcomes in the future.

Case Presentation

A 15 years male adult came with a history of fever with myalgia cough extreme fatigueness, abdominal pain c/o Bilateral lower limb pain and numbness, initially treated several times in outside. Left Eye Ptosis from the past 2 days on admission and also right eye defective of vision. On evaluation chest X-ray showed mediastinum widening subsequently CT chest with contrast was done which showed Anterior Mediastinal Mass. CT-guided Biopsy was done which showed Type B Thymoma, hence referred here from another unit.

Social History

He does not have any social history of cigarette smoking, alcohol addiction.

Allergies

No known medicine or environmental allergies.

Past Medical History

No significant medical history

Past Surgical history

No known surgical history.

Physical Examinations

Vital signs Temp: 100°F, HR – 132/min, RR – 24/min BP – 120/70mmHg, SpO₂ – 98%

A: Patient vocalizing, NO obstruction in airways
B: Spontaneous. Bilateral depth adequate, RR:16/min
C: All peripheral pulse present.HR 132/min BP 120/70 mm Hg
D: GCS E4V5 M6
E: No pressure injury and no other external injury noted

No pale, No icterus, No cyanosis,

Left Eye: Partial Ptosis

Power: Bilateral hand grip good and no sensory deficit.

Shoulder Right	4
Shoulder Left	4
Elbow Right	4
Elbow Left	4

Lower limb

Right Hip	3
Left Hip	3
Right Leg	3
Left leg	3

Initial Evaluation

POCUS

Good LV contractility.IVC collapsing on breathing. Bilateral lung Bilateral Basal B lines

Abdomen -No free fluid, Bladder Partially full.

Markable investigations

WBC	11010 to 2660 cells/cumm
ANC	7630
AMC	1160
AEC	480
Urea	51.36 mg/dl 121.98(9/12)
ABG	7.1(4/12), -20.8(5/12),
LDH	17168
Uric acid	11.31(5/12).12.96 (8/12)
Magnesium	2.65(5/12)
Phosphorus	7.0(5/12)
Creatinine	1.25 mg/dl
Na	134mg/dl
Platelet dropped from	145000 to33000

Imaging Examination

Large mass lesion in the superior and anterior mediastinum with heterogeneous contrast enhancement with encasement of the major vessels and their branches. Subcutaneous edema in the lower neck. Moderate right pleural effusion with the collapse of right lower lobe with diffuse nodular pleural thickening.

To consider invasive thymoma with pleural deposit. Mild hepatosplenomegaly.

Initially Patient was brought to cardiac specialty, Neurophysician and ophthalmology opinion was sought. On evaluation patient was thought to have Myasthenia Gravis and hence was advised to send antibiotics for the same. He had only perception of light in the right eye. His Ptosis improved with ICE pack test. He was advised to do MRI brain with orbital cuts. MRI brain was attempted twice but the same could not be completed as patient had distress on lying down. He was suspected to have crisis hence was discharged and was shifted to kauvery specialty hospital for further management and was admitted under critical care team for further management.

Initially when he was admitted in ER he was tachypnea and was hemodynamically stable.ABG showed lactic acidosis hence NIV was initiated. Patient was admitted in ICU for further care. Screening USG showed normal IV contractility and right moderate pleural effusion (Exudate in nature) and left minimal effusion. Also minimal pericardial diffusion. With no ℅ tamponade noted. On physical examination he had right upper limb swelling with neck, chest and facial swelling. He also had right IJV slow and swirling flow. He had features od SVC syndrome. RNS was done which was inconclusive. CTVS, Neurologist, Cardiologist, Ophthalmologist were involved. Gradually patient had worsening dyspnea and hence was electively intubated. MRI brain with orbital cuts showed multiple soft tissue intensity lesion also in extraconal aspect of right orbit. Multiple focal lesion in the extradural aspect of the spinal canal upper dorsal and upper sacral level, multiple secondary deposits. Contrast CT of chest showed large mass lesion in the superior and anterior mediastinum with heterogeneous contrast enhancement with encasement of the major vessels and its branches, subcutaneous edema in the lower neck, moderate right pleural effusion with collapse of right lower lobe with diffuse nodular pleural thickening, mild hepatosplenomegaly. Patient underwent once cycle of plasma exchange. Surgical and radiation oncologist were involved. As there was rapid growth in tumor with brain metastasis which was unusual in Thymoma, the tumor biopsy that was done elsewhere was sent for IHC in Bangalore to rule out Lymphocytes. Anand labs reported as T cell lymphoblastic Lymphoma. Hence Hematologist was involved. Care takers were explained about the condition and the diagnostic dilemma and steroid use in view of possible lymphoma as benefit of doubt were discussed and steroids were given. Bone marrow biopsy was done which has features of bone marrow necrosis with trephine biopsy imprint consistent with ALL.

However, due to logistics issues care takers want to continue treatment elsewhere and hence he is being shifted to another hospital for further management.

Follow up treatment.

During discharge time he was on
IV fluid NS 200ml per hour
Fentanyl Infusion
Theopentathol infusion
Vecuronium Infusion
Augmentin 1.2 gm IV TDS Day 4
Pantoptrazole 40mg IV OD
Paracetamol 1gm IV SOS
Nodosis 500mg TDS
Febugest 40mg BD

Skilled Nursing Care

1. Assessment and monitoring: including heart rate, respiratory rate, blood pressure, oxygen saturation, and temperature.

Assess oxygen levels regularly, as compression of the trachea or bronchi from the mediastinal mass can impair airflow.
Signs of airway compromise: Watch for signs like increased respiratory effort, labored breathing, or stridor.
Positioning: Encourage the patient to sit upright or lean forward in a position of comfort to help ease breathing and reduce the pressure on the chest.
Supplemental Oxygen: Administer oxygen as prescribed to maintain appropriate oxygen saturation levels.

Monitor for changes in mental status that may indicate brain metastasis or increased intracranial pressure. Symptoms could include confusion, agitation, headache, or seizures.

Perform regular neurological assessments to detect signs of worsening disease, especially if metastasis is suspected in the central nervous system (CNS).
Use pain scales to assess pain levels regularly (e.g., numeric rating scale for verbal patients, FLACC scale for non-verbal patients). CPOT as well.
Pain Management: Administer analgesics as ordered, such as opioids (morphine or hydromorphone), non-steroidal anti-inflammatory drugs (NSAIDs), and adjuncts like gabapentin for neuropathic pain.
Distended neck veins: A sign that venous blood return is obstructed by the mass pressing on the superior vena cava.

2. Symptom Management: Administer pain medications according to the patient’s needs and the pain level. This may include:

Opioids (morphine, fentanyl patches)
NSAIDs for mild to moderate pain.
Adjuvant analgesics like corticosteroids to reduce inflammation around the tumor mass.
Regular pain assessments and re-evaluation of the effectiveness of the prescribed pain management plan.

Positioning: Elevate the head of the bed or position the patient in a way that reduces the pressure on the lungs and airway (e.g., leaning forward or sitting upright).

Oxygen therapy: Administer supplemental oxygen, titrating to achieve desired oxygen saturation (typically ≥ 90%).

Medications: Bronchodilators or corticosteroids may be indicated to help reduce airway inflammation if prescribed.

Emotional Support: Provide a calm environment, use active listening, and provide reassurance.

Anxiolytics: Consider medications such as lorazepam or midazolam to manage anxiety and agitation, especially if these symptoms are affecting the patient’s ability to breathe.

Nutritional Support: Assess appetite and ability to swallow: Tumor compression can affect the esophagus and cause dysphagia (difficulty swallowing). Work with the dietitian and speech therapist if needed.

Small, frequent meals: Offer soft foods or high-calorie, high-protein supplements if the patient has difficulty eating.
Hydration: Ensure the patient is well-hydrated, particularly if there is an issue with swallowing. Consider enteral feeding (via a nasogastric tube or gastrostomy tube) if oral intake becomes insufficient.

Medication management Administer chemotherapy as ordered, monitoring for side effects such as nausea, vomiting, fatigue, and neutropenia (low white blood cell count). Educate the patient and family about the expected side effects and when to report them.

Corticosteroids: Often used in cases of mediastinal masses to reduce inflammation and swelling around the tumor. Monitor for side effects like fluid retention, hyperglycemia, or mood changes.
Antibiotics: If the patient is at risk for infection (due to chemotherapy or immunosuppression), administer antibiotics as needed.

Psychosocial and Emotional Support: Provide Supportive Care: Addressed both physical and emotional needs by offering empathy and comfort. Cancer patients, especially those with advanced disease and metastasis, may experience depression, anxiety, and existential distress.

Family Support: Ensured that the family is kept informed about the patient’s condition, treatment options, and expected outcomes. Encourage family members to be involved in care.

Conclusion

This case highlights the clinical challenges associated with T-cell lymphoblastic lymphoma (T-LBL), particularly in the context of a rapidly worsening anterior mediastinal mass with metastasis. The young adult patient initially presented with significant respiratory distress, a common complication due to the mass effect of the mediastinal tumor on surrounding structures. The timely diagnosis and prompt initiation of chemotherapy were crucial in managing the condition and stabilizing the patient. This patient was referred to Adayar Cancer Institute for further cycles chemotherapy. Now his condition is stable. Our team follows up his progress and support him through his recovery.

Despite the aggressive nature of T-LBL, this case underscores the importance of early intervention and the effectiveness of chemotherapy in controlling the disease. Following the transfer to a specialized facility for continued treatment, the patient has shown significant clinical improvement, now stable under ongoing chemotherapy. This case emphasizes the need for a high degree of clinical suspicion and rapid response in managing T-LBL, particularly when presenting with severe symptoms like respiratory distress due to mediastinal involvement.

In conclusion, T-cell lymphoblastic lymphoma, though rare, should be considered in the differential diagnosis of young patients with rapidly enlarging mediastinal masses, particularly when associated with symptoms like respiratory compromise. Early diagnosis, appropriate staging, and prompt treatment are critical to achieving a favorable outcome.

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A case report on rapidly worsening anterior mediastinal mass with metastasis