|A 44-year-old woman was admitted to the emergency room
presenting a voluminous neck and axillary tumor associated with an
upper left limb swelling and progressively severe dyspnea. At triage,
the patient stated that the swelling had presented itself about three
years earlier and that during this time she had never sought medical
care, had self-prescribed steroid medication and had managed to
camouflage the bulky mass to her relatives. Clinical examination
showed a massive swelling with evidence of mediastinal syndrome
(venous distension of the neck, upper limb oedema, compensatory
collateral circles and shortness of breath) (Figure 1).
|Laboratory evaluation showed an increased neutrophil count
(18.5×103/μl) and lymphopenia (0.45×103/μl), probably related to
steroid abuse. Inflammatory markers were elevated, while LDH was
only slightly increased (274 UI/l, UNL 225 UI/l). A total body,
contrast-enhanced CT scan revealed bulky lymphadenopathy
involving the left retro-mandibular, laterocervical and mediastinal
districts (75×65 mm), as well as left axilla (80×80 and 90×120 mm)
dislocating the local vascular structures. The mass also involved the
left breast and resulted in severe compression of the left lung (Figure
2). Due to hemodynamic instability, only a core needle biopsy could be
performed. Histopathological examination documented the presence of Reed-Sternberg cells (CD30+, CD15-/+, CD20+/-, PAX-5+, CD45-,
CD3-, CD68-, ALK-), diagnostic of a classical sclero-nodular Hodgkin
lymphoma (HL). After the first course of ABVD (doxorubicin,
bleomicin, vinblastine, dacarbazine), the clinical conditions improved
with reduced dyspnea and visible shrinking of the mass.
Unfortunately, 20 days later, the patient developed left-arm cellulitis
and pneumonia that rapidly precipitated into irreversible septic shock
by multidrug-resistant Acinetobacter baumannii.
|HL accounts for approximately 0.6% of all new cancer cases in the
USA. It is unique in that the neoplastic Hodgkin and Reed–Sternberg
(HRS) cells of classical HL account for only 1% of the tumor tissue in
most cases, with various inflammatory cells comprising the tumor
microenvironment. Recent research has identified that HRS cells
originate from germinal center B-cells .
|In present days, characterized by early diagnosis and advanced
medical techniques, the presentation of a ‘visible’ lymphoma with a 3-
year delay appears rather remarkable, particularly in a country like
Italy where the National Health System makes medical care available
at no cost to all patients. The patient’s habitus is reminiscent of the
early days cases reported by Thomas Hodgkin in 1832, at a time when
no effective treatment was available. At diagnosis, HL patients are staged using the Ann Arbor System  and divided into 2 groups:
those with limited-stage disease, typically including those with stage I
or II disease (Involvement of 2 or more lymph node regions on the
same side of the diaphragm); and those with advanced-stage (stage III
or IV) disease.
|Another interesting aspect of this case is that, despite the massive
tumour mass and the years that elapsed from presentation to
diagnosis, the lymphoma still had a limited stage II. To date, studies
have established chemotherapy and involved-field radiotherapy
(IFRT) as standard therapy in limited stages disease: 2 cycles of ABVD
followed by 20 Gy for early favorable HL (according to the German
Hodgkin Study Group criteria: No large mediastinal adenopathy, No
Extra Nodal disease, 1-2 involved nodal regions, ESR ≤ of 50 mm/hr)
; 4 cycles of ABVD plus 30 Gy for those with risk factors , like
our patient. The outcome achieved by primary treatment with ABVD
in major clinical trials and large single-institution series has improved
over the years from the late 1980s through the early 2000s with a 5-
year OS rate increased from just over 70% to approximately 90% .
|In conclusion, it is likely that this woman lived with the stigma of
having cancer, hiding her tumor and endangering her life, for a
condition otherwise highly curable.
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