“Overall survival rate was 74% at follow-up (median 12 months), with 18% having parasellar recurrences and 38% progressing to systemic MM after presentation of a solitary plasmacytoma (median 3 months)…”
Solitary plasmacytoma (MM) led to research into Extramedullary Multiple Myeloma (MM) which led to writing this post about suprasellar plasmacytomas. In short, whether monoclonal proteins are IN your bones or collecting OUTside your bones, or maybe collecting IN your skull, it is all about monoclonal proteins.
The point of this post is that there is a tiny group of newly or recently diagnosed patients who’s lesion doesn’t fit into the usual or customary MM diagnostic criteria.
Rarity of diagnosis is relative-
- A single plasmycytoma is rare (3%-5% of annual 32K MM diagnoses)-
- A single plasmycytoma that is outside of the bone is extra medullary and even more rare-
- A single plasmacytoma that is in the base of the skull is the rarest of all-
The important thing to remember is the therapy plan, according to the studies linked below. If I understand the preferred treatment, surgical excision of the lytic lesion and then local radiation to site results in the best outcome.
While no research will support this thinking, I still believe that each and every newly diagnosed multiple myeloma patient- regardless of where and how many lytic lesions you have- all will live better, longer lives by adding evidence-based, non-conventional therapies to your therapy plan.
- Anti-angiogenic nutrition-
- Anti-angiogenic, anti-inflammatory supplementation-
- Lifestyle therapies such as sauna, exercise, sleep, etc.-
If you’ve received a rare diagnosis about your monoclonal proteins and you have questions, scroll down the page, post a question or comment and I will reply to you ASAP.
Hang in there,
- MM Survivor
- MM Cancer Coach
- Director PeopleBeatingCancer
Five primary case patients include four men and one woman, ages 60–77, followed up to three years. A systematic review identified 65 additional patients, of whom 65% presented with cranial nerve palsies and 15% with hypopituitarism.
Sixteen percent had history of known multiple myeloma (MM) while 37% were diagnosed concurrently with MM on presentation of parasellar plasmacytoma.
Imaging showed median tumor size of 38 mm (range, 4–70 mm), with MRI intensity similar to that of other sellar masses.
Surgical biopsy with immunohistochemical studies confirmed plasmacytoma diagnosis. Eighty-one percent underwent parasellar radiotherapy, and chemotherapy initiated in 59% of the 69 patients with MM.
Overall survival rate was 74% at follow-up (median 12 months), with 18% having parasellar recurrences and 38% progressing to systemic MM after presentation of a solitary plasmacytoma (median 3 months)…
Parasellar plasmacytomas are rare tumors that should be considered in the differential diagnosis for lesions involving the sella and arising from the clivus, especially when cranial nerve paresis is apparent, even in the absence of known MM.
Although recurrence rates for parasellar plasmacytoma is low, patients should be monitored for progression to MM. Treatment depends on the presence of systemic disease at diagnosis…”
- Extramedullary plasmacytoma, a subtype of plasmacytoma, accounts for <1% of all head and neck tumours.
- Sellar region plasmacytoma can mimic other sellar region tumours clinically and radiologically.
- Tissue histology is recommended to achieve an accurate tissue diagnosis.
- The final diagnosis of plasmacytoma is confirmed by ruling out the presence of multiple myeloma through serum and urine investigations.
- Local irradiation therapy is the treatment of choice.
“Plasmacytoma of the skull-base is a rare entity. Differential diagnosis includes chordoma, osteosarcoma, carcinoma nasopharynx, meningioma, metastatic carcinoma, lymphoma, and multiple myeloma. Accurate and precise diagnosis is extremely important for plasmacytoma of the skull-base as its treatment and prognosis is different from other skull-base lesions.
A 41-year-old man presented with concerns of headache, diplopia, and left eye strabismus. A magnetic resonance image (MRI) of his brain showed a large expansile mass measuring 51 mm involving the clivus and central skull-base. Trans-sphenoidal tumor decompression was done. A biopsy confirmed the plasmacytoma.
A positron emission tomography-computed tomography (PET-CT) scan showed a single 2-(18F) fluoro-D-glucose (FDG) avid lesion at the skull-base. The results of all other relevant investigations such as hemoglobin, renal function test, serum calcium, serum protein immunoelectrophoresis, serum quantitative immunoglobulin, bone marrow biopsy, serum lactate dehydrogenase, and beta-2 microglobulin levels were within normal limits.
He was treated with radical radiotherapy. He developed complete clinical response after radiotherapy…”
Plasmacytomas are a collection of plasma cells that occur as a solitary lesion or in conjunction with multiple myeloma. Intracranial location is uncommon but should be considered as management differs. Plasmacytomas in the suprasellar region are rare but should be considered in the differential diagnosis of suprasellar masses.
Clinical presentation and imaging findings have similarities and overlap between pituitary adenomas and plasmacytomas, so the diagnosis depends on biopsy and pathological evaluation. Immunohistological staining is often necessary due to structural similarities to adenomas.
Isolated cases may be treated with radiation alone and surgery is reserved for symptoms due to mass effect. Systemic therapy is given if there is evidence of multiple myeloma.
In this case report, we present a 52-year-old male who presented with worsening blurry vision associated with headaches and epistaxis of four months duration. CT of the head showed a large mass involving the sella and skull base.
Labs showed normal calcium, creatinine, and intact pituitary function. Biopsy of the mass was initially diagnosed as a pituitary adenoma but repeat pathology revealed plasmacytoma. Body imaging revealed diffuse lytic lesions. Bone marrow biopsy and serum electrophoresis were consistent with a diagnosis of multiple myeloma. The patient underwent radiation therapy to the suprasellar mass followed by systemic therapy for multiple myeloma with bortezomib, lenalidomide, and dexamethasone. The patient achieved a very good partial response…”