Multiple Lytic Bone Lesions Research Articles

Lymphangiomatosis of Bone and Visceral Parenchyma: Report of Two Cases

Lymphangiomatosis is a rare disorder that occurs mainly in children or during the first two decades of life. It is characterized by a diffuse proliferation of lymphatic channels involving the bones, visceral parenchyma, and soft tissue. Most cases of lymphangiomatosis have bone and visceral involvement and usually present with chylothorax, chylous ascites, chylous pericardial effusion, or acute symptoms that are related to the affected organs. The authors experienced two cases that presented with chylothorax and multiple lytic bone lesions. Chest drainage and chemical pleurodesis were performed for treatment of the chylothorax. In one case, lytic bone lesions were found only in the right scapula and bone lengthening with an Ilizarov frame was performed for growth arrest in the right humerus. In the other case, lytic bone lesions were found in both femurs and both humeri, the right tibia, and the right scapula; and were particularly severe in the right tibia and femur. The lytic bone lesion, osteosclerosis, pathologic fracture, and pseudoarthrosis were so severe that weight-bearing was impossible. Internal fixation was performed with an intramedullary nail in the left femur.

Macrofocal Multiple Myeloma in Young Patients: A Distinct Entity with Favorable Prognosis.

Introduction: Most patients with multiple myeloma (MM) present with multiple lytic bone lesions, extensive marrow plasmacytosis and anemia. Furthermore in several of them hypercalcemia and renal impairment are evident at diagnosis. Over the years, we have seen occasional young patients with MM who presented with multiple lytic bone lesions but without intervening infiltration of bone marrow i.e. a pattern consisting of macrofocal MM.Patients and methods: In order to assess the clinical and laboratory features and the outcome of patients with macrofocal MM we performed a retrospective analysis of symptomatic patients with MM ≤40 years of age at diagnosis who received primary treatment over a 20 year period (from January 1, 1985 to December 31, 2004). The diagnosis of macrofocal MM required the presence of lytic bone lesions and the absence of obvious bone marrow plasmacytosis (BMPC<10%) on bone marrow aspirate and/or biopsy.Results: Among 51 patients ≤40 years at the time of initial treatment, 10 patients fulfilled the criteria of macrofocal MM. Patients' median age, gender, myeloma heavy and light chain were similar among patients of the 2 groups. When compared with patients with typical MM, patients with macrofocal pattern were less anemic (p=0.018), none had hypercalcemia (p=0.1), renal impairment (p=0.17), elevated serum LDH (p=0.14), or stage 3 according to the International Staging System (ISS) (p=0.13). Four of the 10 patients with macrofocal MM versus 0 of 41 patients with typical MM (p=<0.01) had a prior diagnosis of solitary bone plasmacytoma. Approximately 80% of patients in both groups received primary treatment based on high-dose dexamethasone regimens (VAD or VAD-like regimens). An objective response was noted in 55% of patients with macrofocal MM and in 50% of patients without this pattern. The median survival of patients with typical MM was 57 months and has not been reached in patients with macrofocal MM (p=0.087). In the latter group of patients, median survival is projected to exceed 8 years. The 10 year survival rate is 20% and 5% in patients with or without macrofocal myeloma respecrively.Conclusion: Our analysis indicates that macrofocal MM is a distinct entity in young patients with MM. Despite multiple lytic bone lesions, such patients have features of low tumor burden and an improved survival when compared with young patients with typical MM. Future studies including gene profiling may reveal potential biological differences among the two groups of patients.

Hypercalcemia in B-Cell Chronic Lymphocytic Leukemia: Report of a Case and Review of the Literature.

A 53 year old man with Rai stage IV CLL was being treated with R-HyperCVAD when he presented between cycles with fatigue, lethargy and pancytopenia. Clinical examination revealed diffuse adenopathy and splenomegaly. Serum calcium was 13.6 mg/dl, phosphorus level was 2.7 mg/dl, and alkaline phosphatase was 54 U/L. PTH was 4 Units/L (10–65), PTHrP < 0.2 pmole/L (0–1.9), 1–25 (OH) Vitamin D < 10pgm/ml (22–67). Quantitative immunglobulins: IgA 10 mg/dl, IgG 252 mg/dl, IgM 50 mg/dl. Immunofixation revealed a faint IgG lambda paraprotein. There were no lytic lesions on skeletal survey. Bone marrow biopsy revealed focal large cell transformation (Richter's syndrome). Cytogenetics revealed 3 metaphases with complex cytogenetic abnormalities, indicating clonal evolution. The hypercalcemia resolved with appropriate therapy, but despite subsequent treatment with CAMPATH, he died 2 weeks after diagnosis.A review of reported patients with CLL and hypercalcemia in the literature was performed from 1980 onwards using MEDLINE and PubMed; only those cases in which clinical aspects, biochemistry, PTH levels, imaging studies and concurrent pathology (if obtained) were documented, are summarized in this analysis (n=13).Rai stage: I n=1, II n=4, III n=3, IV n=5. Immunoreactive PTH levels were low or normal in 100% of patients. In 5 cases in which it was measured, 1–25 (OH) Vitamin D levels were not elevated. PTHrP was normal in 2 cases and elevated in 1. In nine patients, multiple lytic bone lesions were present on skeletal radiology. Two patients had osteopenia without lytic lesions. Two had no lytic lesions.Six of ten patients had evidence of transfomation on lymph node and/or bone marrow biopsy performed at the time of evaluation for hypercalcemia.Prognosis was poor (range 0.5–12 months) with only one patient surviving post allo-transplant.In conclusion, hypercalcemia in CLL is rare. Osteolytic lesions are present in the majority of cases. PTH levels are low, and thus this hormone is not mediating the hypercalcemia. The evidence is also against a role of elevated Vitamin D. Histological transformation is seen in half of the cases. Survival is poor after diagnosis of hypercalcemia. The mechanisms(s) of the osteolysis and hypercalcemia remain to be defined.

Multiple Lytic Bone Lesions in an 85-Year-Old Man

An 85-year-old, Russian-speaking man of Eastern European origin with a past medical history of Parkinson disease, hypothyroidism, and chronic anemia was evaluated for gradual increase in shortness of breath that worsened 2 days before admission. The patient did not complain of any chest pain, fever, cough, nausea, or vomiting. Physical examination showed an elderly man with shortness of breath during talking and at rest. His temperature was 36.18C; pulse, 100 beats per minute; respiratory rate, 30 to 40 breaths per minute; and blood pressure, 140/70 mm Hg. Examination of the cardiovascular system was unremarkable. Auscultation of the lungs revealed decreased breath sounds at the right base and bronchial breath sounds on the right side. No crackles, rhonchi, or wheezing were found. Physical examination of the patient’s abdomen showed splenomegaly, but no significant hepatomegaly. No lymphadenopathy was found. Examination of the nervous system showed no evidence of focal neurologic signs or evidence of any neuropathy. Examination of the neck showed deformities of the cervical spine, reportedly caused by fractures. The patient also complained of chronic bone pain for many years. Abnormal laboratory tests included serum sodium, 124 mEq/L; hemoglobin, 10.7 g/dL; hematocrit, 31.4%; and thyroid stimulating hormone, 3.2 mIU/mL. Both the white blood cell count and the platelet count were within normal limits. Total gamma globulins were slightly elevated (4.3

Multiple lytic bone lesions in an 85-year-old man: type 1 Gaucher disease, adult onset (nonneuronopathic form).

An 85-year-old, Russian-speaking man of Eastern European origin with a past medical history of Parkinson disease, hypothyroidism, and chronic anemia was evaluated for gradual increase in shortness of breath that worsened 2 days before admission. The patient did not complain of any chest pain, fever, cough, nausea, or vomiting. Physical examination showed an elderly man with shortness of breath during talking and at rest. His temperature was 36.18C; pulse, 100 beats per minute; respiratory rate, 30 to 40 breaths per minute; and blood pressure, 140/70 mm Hg. Examination of the cardiovascular system was unremarkable. Auscultation of the lungs revealed decreased breath sounds at the right base and bronchial breath sounds on the right side. No crackles, rhonchi, or wheezing were found. Physical examination of the patient’s abdomen showed splenomegaly, but no significant hepatomegaly. No lymphadenopathy was found. Examination of the nervous system showed no evidence of focal neurologic signs or evidence of any neuropathy. Examination of the neck showed deformities of the cervical spine, reportedly caused by fractures. The patient also complained of chronic bone pain for many years. Abnormal laboratory tests included serum sodium, 124 mEq/L; hemoglobin, 10.7 g/dL; hematocrit, 31.4%; and thyroid stimulating hormone, 3.2 mIU/mL. Both the white blood cell count and the platelet count were within normal limits. Total gamma globulins were slightly elevated (4.3

Case 38-2004

A 40-year-old man noted a lump on the top of his head. Imaging studies showed a dural-based tumor invading bone and soft tissue. The lesion was excised and a diagnosis of plasmacytoma was made. Additional staging studies disclosed multiple lytic bone lesions, with no paraprotein and a negative bone marrow biopsy and aspirate. The discussants review recent advances in the management of multiple myeloma.

Cholestatic jaundice and bone lesions in an elderly woman

A 74-year-old Caucasian female presented with jaundice, clay-colored stools, diarrhea, and fatigue of 3 months' duration, accompanied by a weight loss of 6.8 kg. Physical examination demonstrated mild hepatomegaly. Initial blood work revealed abnormal liver biochemistries with a cholestatic pattern. An abdominal CT scan showed intrahepatic bile-duct dilatation without masses but with multiple lytic and blastic bone lesions. A sacral bone biopsy established the diagnosis. Endoscopic ultrasound, endoscopic retrograde cholangiopancreatography, CT scan, bone biopsy and liver biopsy. Systemic mastocytosis affecting the biliary system resulting in a primary-sclerosing-cholangitis-like picture combined with diffuse blastic and lytic bone lesions. Biliary stenting, histamine 1- and 2-receptor blockers, and chemotherapy (cladribine).

Ptosis und Ophthalmoplegie als Leitsymptome eines multiplen Myeloms

A 59-year-old woman was diagnosed as having solitary bone plasmacytoma of the sternum which was resected. Five month later she presented with a pathological fracture of the clavicle. On examination she had a ptosis and an ophthalmoplegia. A mass in the clivus extending into the left sphenoid sinus as well as multiple osteolytic lesions in the skull were shown by cranial MRI. Skeletal survey showed multiple osteolytic lesions. Laboratory test did not show any specific abnormalities. The biopsy taken from the mass at the sphenoid sinus demonstrated plasmacytoma. The diagnosis of multiple myeloma was based on the histological evidence of plasmacytoma and the occurrence of multiple lytic bone lesions although no infiltration of bone marrow and none of the specific laboratory findings were present. The patient underwent local radiotherapy with 30 Gy followed by systemic chemotherapy. The symptoms regressed completely under this therapy. Various cranial nerve syndromes such as the superior orbital fissure syndrome are most often caused by tumors at the skull base. Knowledge of the histological entity is essential for the correct diagnosis and the appropriate therapy because rare tumors like multiple myeloma may also cause such syndromes.

Tumeur maxillaire révélatrice d'un lymphome agressif de type B non Burkitt chez un mauritanien porteur du virus HTLVI

We report a case of agressive B cell lymphoma with jaw tumour, multiple lytic bone lesions and hypercalcemia in a HTLVI sero positive man from Mauritania. Some epidemiological data suggest a possible increased risk of B lymphoid neoplasias among HTLVI carriers.

Seventeen-year survival in multiple myeloma.

We report a case of multiple myeloma surviving 17 years since diagnosis and 13 years in continuous complete remission since a relapse 4 years after initial treatment. Despite the prolonged remission, multiple lytic bone lesions have not healed.

Flail Chest and Multiple Myeloma

To the Editor.— Multiple myeloma is a common disease that universally involves the skeletal system. Although rib lesions are common, flail chest resulting from multiple rib fractures or disarticulation is extremely rare. The occurrence of such a complication in a patient with multiple myeloma prompted this report and a review of the literature of the past 20 years. Report of a Case.— A 54-year-old man was admitted to the hospital because of low back pain of four months' duration. Diagnosis of multiple myeloma was established on the basis of multiple lytic bone lesions and bone marrow plasmacytosis (80% of the nucleated cells) associated with hypercalcemia. He was transferred to Little Rock Veterans Administration Medical Center for treatment. On arrival he had dyspnea secondary to flail chest resulting from spontaneous disarticulation of several ribs from the sternum. The rest of the findings from the physical examination were within the normal range.

Extramedullary Myeloma With Metastatic Features

MULTIPLE myeloma is a disease mostly confined to medullary sites, but may have varied extraosseous manifestations.^1,2 Most commonly, these extraosseous lesions occur in the nasopharynax.³The occurrence of extramedullary plasmacytoma in the retroperitoneum is rare, although isolated cases have been reported.¹Recently, we have had the opportunity to study a patient who initially had a case of combined myelomatosis with extramedullary subcutaneous lesions and who, at relapse, was found to have a huge retroperitoneal mass. This patient responded to pyrazofurin after treatment with nine other chemotherapeutic agents failed. These findings have recently been corroborated.⁴ <h3>Report of a Case</h3> A 66-year-old woman had multiple lytic bone lesions, a large (6×10-cm), subcutaneous, biopsy-proved plasmacytoma of the right posterior area of her rib cage, bone marrow infiltration with 40% immature plasma cells, free k-chains in her urine, and a serum protein electrophoresis pattern with an IgG k-paraprotein spike of

Secondary Open-angle Glaucoma in Histiocytosis X

A 9 month-old boy developed secondary open-angle glaucoma associated with fever and multiple lytic bone lesions 2 1/2 months after cataract aspiration. An open bone biopsy provided a diagnosis of histiocytosis X. Hisiocytes similar to those in the bone were identified in a specimen of aqueous humor from the affected eye. No other cells were found. The abnormal histiocytes may have caused the glaucoma by infiltrating the trabecular angle structures and mechanically obstructing aqueous outflow from the eye.

Osteosclerotic myeloma and peripheral neuropathy. A case report.

A patient presented with cervical and axillary adenopathy, bilateral symmetrical sensory-motor peripheral neuropathy, and multiple lytic bone lesions with osteosclerotic margins. Biopsy of the lymph nodes and bones revealed this to be a plasma cell tumor. A review of the literature has revealed 36 cases of osteosclerotic myeloma, 37 cases of peripheral neuropathy with myeloma, and the association of the two in 13 cases. The further association with plasmacytomas of the lymph nodes is extremely rare and has been reported in only 5 cases.

Occult Follicular Thyroid Carcinoma: An Unusual Presentation of Multiple Lytic Bony Metastasis in the Skull of a 66-Year Malay Man

Follicular carcinoma of the thyroid usually presents as nodules with rare cases showing metastasis at diagnosis. But occult follicular carcinoma without any thyroid enlargement and presenting as multiple lytic bone lesions is unusual and relatively rare. Here we report a 66-year old Malay man presenting with occipito-parietal swelling for last two years. He had no thyroid enlargement; no thyroid related symptoms and his biochemical thyroid profile was normal. Computerised tomography of skull reported multiple lytic lesions in the occipito-parietal region. FNAC and histopathological examination confirmed the diagnosis of metastatic follicular carcinoma. We present this case because of the rarity of the disease. Ibrahim Med. Coll. J. 2007; 1(2): 25-27 Key Words: Occult follicular carcinoma thyroid, skull metastasis, normal thyroid findings. doi: 10.3329/imcj.v1i2.2906

Congenital generalized fibromatosis. Case report, with roentgen manifestations.

Since the original description of congenital generalized fibromatosis by Stout (5) in 1954, only 2 cases of this rare condition could be found in the literature. The disease consists of a heterogeneous group of hyperplastic and dysplastic lesions of connective tissue, occupying a poorly defined position between proliferation and repair vs. unequivocal neoplasia (4). In none of the 3 previous case reports was there any significant discussion of the bony manifestations. It is the purpose of this paper to record another example and describe the roentgen manifestations in relation to the skeletal system. Case Report A white male infant was admitted to the Denver Children's Hospital at the age of three months, chiefly because of difficulty of breathing, loose, bloody bowel movements, and failure to gain weight. The illness had its onset at the age of one month with diarrhea and the appearance of subcutaneous nodules. The child was hospitalized at that time and roentgen examination showed a pulmonary infiltration, inflammatory in appearance, and multiple lytic bone lesions. At this first admission a biopsy of a subcutaneous lesion was interpreted as showing neurofibromatosis but on subsequent biopsy this diagnosis was changed to congenital generalized fibromatosis. After discharge, the child failed to thrive and he was readmitted to The Children's Hospital. Additional study revealed an uneventful prenatal history and labor. The mother and father were well. Siblings included a brother of seven, a sister aged two, both healthy, and an infant who had died thirty-six hours after birth, reportedly because of a “collapsed lung.” Physical examination showed a malnourished, poorly developed infant in moderate respiratory distress. The temperature was 99.4° F., pulse 152, and respirations 28. The height was 54.9 cm.; the weight, 8 lb.; the head circumference, 36.7 cm. Auscultation of the chest demonstrated decreased breath sounds and a tachycardia with P2 greater than A2. There were no murmurs. The liver was palpable 1.5 cm. below the right costal margin. Multiple soft subcutaneous boggy tumors were obin all the extremities, the thorax, and buttocks. Laboratory examination revealed a hemoglobin of 9.4 gin. per 100 c.c. and a leukocyte count of 10,000. Urinalysis was negative. Throat cultures showed growth of Staphylococcus aureus. Stool cultures showed Endamoeba coli were 1+ for occult blood. Additional laboratory findings were as follows: serum calcium, 9.8 mg. per 100 c.c, ionized protein, 5.2 mg. per 100 c.c, inorganic phosphorus 5.2 mg. per 100 c.c, alkaline phosphatase, 8.6 mg. per 100 c.c, total serum protein, 5.1 gm, per 100 c.c, with an albumin-globulin ratio of 0.7. Electrophoresis revealed a slight accentuation of the alpha-2 fraction and a depression of the alpha-1 globulins. The patient did poorly in the hospital and died after ten days. Bronchopneumonia was the immediate cause of death.