Lupine Publishers | Central Nervous System Manifestation of Multiple Myeloma: A Case Report

Lupine Publishers | Open Access Journal of Oncology and Medicine

Abstract

Multiple myeloma accounts for <1% of all malignancies and Central nervous system (CNS) manifestation of multiple myeloma (MM) is rare. Multiple myelomais characterized by malignant transformation of plasma cells that produces immunoglobulin chains. The accumulation of plasma cells in bone marrow results in clinical features like anaemia, osteolytic lesions, hypocalcaemia, renal failure and immunodeficiency [1,2]. The involvement of central nervous system is very uncommon in multiple myeloma and it may present early or late during the course of disease. The neurological symptoms can be diffuse headache, persistent vomiting, vertigo, weakness in limbs, urinary incontinence etc. Symptoms can be attributed to spinal cord or nerve root compression, peripheral neuropathies, and uremia [3,4]. Likely risk factors of CNS manifestation are unfavourable cytogenetics, high tumour load, low marrow involvement. In this report we describe a patient with CNS involvement after receiving systemic chemotherapy and symptomatic treatment.
Keywords: Central nervous system, Multiple myeloma

Case Report

A 52 years pleasant lady presented with complaint of pain in left leg with lower backache and tingling sensations for 10 days. Subsequently she developed pain in right leg followed by urine incontinence. MRI Lumbosacral Spine was done which reported ill defined lesions at L3-L4 vertebra (with associated periosseous fluid collections) and multiple focal lesions in the L4 vertebra, bilateral sacral ala and both iliac bones along with indentations upon the thecal sac by spondyliticridges, secondary spinal canal stenosis and neural foraminal narrowing at multiple levels and left exiting nerve root compression at L3-L4, L5-S1 levels (Table 1). She underwent L1-L5 stabilization, L3 decompression with laminectomy, biopsy, posterolateral fusion with local autograft. Histopathology reported plasma cell dyscrasia at L3-L4 level. Immunohistochemistry reported tumor cells are focally positive for CD-138 and kappa light chain and negative for lambda light chain. On further evaluation bone marrow biopsy reported plasma cells approximately 50% of total nucleated cells suggestive of plasma cell myeloma. Bone marrow aspiration shows plasmacytosis approximately 24% of total nucleated cells which confirmed the diagnosis. She received 15 cycles of Bortezomib and Thalidomide.

Table 1:

Table 2:

In view of persistent pain in legs MRI dorsal spine was done which reported well defined altered intensity expansile lesion in L3 vertebra extending into epidural space. She received palliative external beam radiotherapy to L3 vertebra 30Gy/10 fractions. She remained asymptomatic for a month. Then she presented with complaint of lower limbs weakness and nausea, loss of appetite, constipation, weight loss (Table 2). USG whole abdomen was done which reported multiple hypoechoic masses in epigastric region. Whole body PET CT reported metabolically active soft mass lesion in gastro hepatic region measuring 9.7x5.5 cm SUV max 20.3, recto uterine pouch measuring 9.2 x 7.8cm SUV 20.9, left iliac fossa measuring 7.2x4.0cm- ?mitotic, metabolically active mesenteric nodes measuring upto 2.2cm-? mitotic and metabolically active lytic lesion involving L3 vertebra with paravertebral soft tissue component infiltrating left psoas muscles.

Table 3:

Table 4:

To confirm the diagnosis CT guided core needle biopsy from supraumbilical mass was done which was compatible with multiple myeloma. She then received 1st cycle of Bortezomib, cyclophosphamide and dexamethasone. After few days, she developed headache, giddiness and syncopal attacks. On evaluation CEMRI brain reported diffuse leptomeningeal enhancement involving both cerebral as well as cerebellar hemispheres. Mild enhancement of basal cistern with associated marginal dilation of ventricular system (Figure 1). In view of above mentioned complaints, cerebrospinal fluid study was done (Table 3) (Figure 2). CEMRI spine was done which reported no abnormal enhancement in vertebra, cord or thecal sac (Figures 3 & 4). She received 7 fractions (14 Gy) out of 12 planned fractions (24Gy) of whole brain radiotherapy and two intra thecal injection of methotrexate. MRI upper abdomen with MRCP reported large mass replacing entire head and body region of pancreas. Mass encases and compresses CBD with mild bilobar intra hepatic biliary radical dilation. There is also encasement of second part of duodenum with possible infiltrartion of adjacent hepatic parenchyma as well. Features are suggestive of aggressive pancreatic malignanacy, possibility of pancreatic lymphoma. Thickening and edema of gall bladder wall was seen which was suggestive of concomitant cholecystitis. She was advised for ERCP with SEMS placement which was refused by attendants (Figure 5). In view of her deranged liver function test, both radiotherapy and methotrexate were discontinued and she was maintained on supportive care (Table 4).

Figure 1:

Figure 2:

Figure 3:

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Figure 5:

Discussion

Central nervous system is a rare location of involvement in MM, however it should be considered if a patient with MM presents with neurologic symptoms. Patients with MM often have neurological complications, either due to metabolic disorders such as hypocalcaemia, uraemia and hyper viscosity or due to peripheral neuropathy, spinal cord compression and cranial nerve infiltration [1,2]. The most common cause is spinal cord compression and cranial nerve infiltration [3,4]. The patient may present as a known case of MM with CNS features or as a new case with CNS symptoms. The clinical presentation depends on degree and site of infiltration. The symptoms and signs are headache, memory loss, behavioural changes, convulsions, nausea, vomiting, vertigo, urinary incontinence, backache, and limb weakness [3,4]. These symptoms can be attributed to spinal cord or nerve root compression, raised intracranial tension, meningeal inflammation [5]. In our case she presented with the complaint of headache, giddiness and syncopal attacks. This can be explained by CEMRI brain imaging which reported diffuse leptomeningeal enhancement involving both cerebral as well as cerebellar hemispheres. CSF study was done to reported to be negative. She received 7 fractions out of 12 fractions planned for whole brain radiotherapy and two injections of intrathecal Methotrexate injections [6]. In view of deranged LFT, radiotherapy and IT chemotherapy was stopped and was managed conservatively. She passed away after 2 weeks due to sepsis with LRTI with MM as underlying cause.

Conclusion

In conclusion this patient had an aggressive disease in view of abdominal deposits and unresponsiveness to multiple lines of systemic disease. CNS involvement in cases of MM portends extremely poor prognosis and median overall survival of <6 months. While in this case there was CSF infiltration which would portend even poorer prognosis but she succumbed to sepsis due to Lower respiratory tract infection. CNS disease in MM can be effectively palliated especially because of relative radio sensitivity of MM due to which early responses are seen at lesser doses and an early diagnosis can improve quality of life effectively.

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Lupine publishers | Head Cancer and Metastasic Neck. What Have We Advanced in the Last Years?

Lupine Publishers | Open Access Journal of Oncology and Medicine

Abstract

In 2012, 5.210 new cases of head and neck tumors were estimated in the USA, with an increasing incidence due to tobacco and alcohol habits in the population. A large percentage of the cases debut as a locally advanced disease, so control of the disease is key and we look for the best therapeutic strategy to achieve good survival rates while maintaining quality of life. We present the case of a 60-year-old patient in which our objectives to be presented are the assessment of comorbidity, toxicity and survival.

Clinical Case

A 60-year-old man without medical illnesses to be highlighted. He came to the emergency room in July 2016 due to injury at the cervical level of 1 month of evolution, with progressive growth and breathness, with also difficulty for eating. Also asthenia, anorexia and loss of weight not quantified in the last month.
Physical Examination: Weight 42 kg Head and Neck: mass of hard consistency of approximately 10 cm in diameter in the cervical left region that seems to deflect trachea. Pulmonary auscultation: generalized hypoventilation with some expiratory wheeze.

Additional Tests

a. Fibroscopy (August 2016) ulcerated lesion from right vallecula to mouth of Killian. Paresis of both vocal cords.
b. PET-CT (August 2016): Extensive tumor in pharynxlarynx- esophagus (> 6 cm) Lymph nodes in left IB-II spaces (> 6 cm). If confirmed, carcinoma would correspond to T3 N3 (stage IVB) (Figure 1).
c. Laryngeal Biopsy: Moderately differentiated and keratinizing squamous cell carcinoma.

Figure 1: PET-CT August 2016: Pathological adenopathies> 6 cm in left IB-II spaces

So, confirmed Squamous cell carcinoma stage cT4N3 Mx.The case is presented in the Tumor Committee, deciding treatment with Chemoradiotherapy and 1 cycle of Docetaxel+ Cisplatin (60% dose reduction because of frailty/ malnutrition) previous to induction due to the large tumor volume and waiting for start radiotherapy. Tracheotomy is performed prior to starting because of the risk of airway obstruction and also gastrostomy is placed for nutritional support.
On 31th August 2016 the patient starts on RT concomitantly to Cisplain ( receiving 2 cycles on 12.09.2016 and 10.10.2016 and 70 Gy) During the treatment, he achieves a good general condition until January 2017, when he goes to Otolaryngology Clinics referring dysphagia again although he maintains weight in 51 kg. PET-CT is performed: disease progression with soft tissue increase in the pharyngoesophageal junction despite the good response of cervical adenopathies and the partial response of the primary tumor. New bilateral subpleural pulmonary nodules suggestive of metastasis. Figure 2 Due to progression, he restarts treatment with chemotherapy (palliative intention) with ERBITAX scheme (Paclitaxel 80 mg / m2 weekly (3 / 4s) + Cetuximab 400 mg / m2 followed by 250 mg / m2) with good tolerance,only highlighting secondary rash to cetuximab (predictive factor). After 3 cycles he presents significant partial response (Figure 3) and continues until 6 cycles. After 10 cycles it is considered whether to stop paclitaxel and follow on with cetuximab, but given the good tolerance they remain both of them. However, in January 2018, he presented a new pulmonary progression, so we decided to start a new strategy with inmunotherapy (Nivolumab), receiving 2 cycles to date.

Figure 2: PET-TC January 2017: locoregional tumor progression.

Figure 3: TC March 2017: Partial response of left adenopathy

Discussion

a. The concept of fragility is evaluated incorrectly through the Performance Status (PS). The ACE scale 27 assesses comorbidities and compares survival to having an advanced stage, so that is a fact to take into account more than the ECOG at the time of choosing the treatment.
b. When a patient progress to chemoradiotherapy, we have two good “palliative chemo” options: phase III EXTREME study(5-FU + Cisplatin + Cetuximab) or phase II of Hitt (Paclitaxel-Cetuximab) with fewer side effects, which is an alternative to cisplatin, achieving good response rates (20.43%) [1].
c. After 6 cycles of Paclitaxel + Cetuximab can be considered to keep Cetuximab as monotherapy, continue both or suspension until progression.
d. If pulmonary metastatic disease is controlled for> 1 year, we can consider surgical intervention.
e. Support treatment improves tolerance to chemotherapy. The indication of prophylactic enteral nutrition is a controversial issue, so we have to individualize.
f. We need predictive factors for each tumor type that we can know about before hand the prognosis and guide the treatment according to it. We must raise multidisciplinary strategies with the aim of achieving the best treatment sequence to improve survival in a population with few therapeutic options [2]

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Targeting the Immune Checkpoint in Cancer: Is This a Viable Treatment Option for AML? by Steven J Coles in Open Access Journal of Oncology and Medicine (OAJOM) - Lupine Publishers

The immune suppressive mechanisms displayed by malignant cells are considered a central process in the pathogenesis of cancer. Research in this area has gained significant momentu mover the past 20 years, with several immune checkpoints identified, including; CTLA-4, CD200/CD200R, Tim-3/Galectin-9 and PD-L1/PD-1 (Figure 1). Whilst characterising the molecular basis of leukaemia for risk stratification remains at the forefront of AML research; this must now extend to understating how the seimmune checkpoint path ways fit into the equation. A good example of why this is important is to consider CD200expression level in AML, which is a negative prognostic indicator [1]. CD200 is an immunosuppressive lig and, that when engaged with its receptor CD200R, has the capacity to attenuate T-cell and NK-cell anti-tumour activity in AML. Interestingly, most cases of CBF AML express high levels of CD200, yet CBF AML performs relatively well clinically. This paradox suggests there is a complex interplay between AML molecular heterogeneity and immune surveillance. Given the recent development and FDA approval of several immune checkpoint therapies, a full understanding of these processes and integration with standard molecular risk stratification is warranted.

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