Insights on Lipomatosis after Platinum-Based Chemotherapy Use in Pediatric Oncology: A Case Report.

Agents of platinum-based chemotherapy, such as cisplatin or carboplatin, are used in the treatment of a wide range of malignancies that affect children, such as brain tumors, osteosarcoma, neuroblastoma, hepatoblastoma, and germ cell tumors (GCTs). The Cyclophosphamide Equivalent Dose (CED) calculator for reproductive risk does not take platinum-based chemotherapy into account, despite the fact that it accounts for the majority of chemotherapy medications that are typically administered for pediatric GCTs. As a result, exposure to platinum-based drugs throughout infancy can have predictable long-term effects such as infertility, as well as other rare encounters such as lipoma formation and lipomatosis. Lipomas are the most prevalent benign soft tissue tumor subtype. They may be either solitary entities or engaged in multiple lipomatosis, which may have a familial origin or be an acquired disorder. Chemotherapy is a possible cause of lipomatosis. Chemotherapy based on cisplatin has been linked to a variety of long-term consequences, including kidney damage, neurotoxicity, and pulmonary toxicity, and may even create secondary cancers. However, lipoma development is known to occur in fewer than 1 in 100 individuals, and only a few examples of multiple cutaneous lipomatosis triggered by this therapy have been documented. Here we present a very rare case of lipomatosis in a pediatric patient with GCT under cisplatin therapy, which might be the third report of this kind affecting children.

Macropolycyte in Pediatrics.

Macropolycytes are tetraploid neutrophils produced during accelerated myelopoiesis. They have been reported in adults with pernicious anemia, sepsis, and after cytotoxic chemotherapy. Two pediatric cases are reported, one after granulocyte colony-stimulating factor treatment and the other following Kawasaki disease, respectively.

Immune status of patients with inherited bone marrow failure syndromes.

Immune function abnormalities have been reported in patients with Fanconi anemia (FA), dyskeratosis congenita (DC) and, rarely, in Shwachman-Diamond syndrome (SDS), and Diamond-Blackfan anemia (DBA), but large systematic studies are lacking. We assessed immunological parameters in 118 patients with these syndromes and 202 unaffected relatives. We compared the results in patients with reference values, and with values in relatives after adjusting for age, sex, corticosteroid treatment, and severe bone marrow failure (BMF). Adult patients (≥18 years) with FA had significantly lower immunoglobulins (IgG, IgA and IgM), total lymphocytes, and CD4 T cells than reference values or adult relatives (P < 0.001); children with FA had normal values. Both children and adults with FA had lower B- and NK cells (P < 0.01) than relatives or reference values. Patients with DC had essentially normal immunoglobulins but lower total lymphocytes than reference values or relatives, and lower T-, B-, and NK-cells; these changes were more marked in children than adults (P < 0.01). Most patients with DBA and SDS had normal immunoglobulins and lymphocytes. Lymphoproliferative responses, serum cytokine levels, including tumor necrosis factor-α and interferon-γ, and cytokine levels in supernatants from phytohemagglutinin-stimulated cultures were similar across patient groups and relatives. Only patients with severe BMF, particularly those with FA and DC, had higher serum G-CSF and Flt3-ligand and lower RANTES levels compared with all other groups or relatives (P < 0.05). Overall, immune function abnormalities were seen mainly in adult patients with FA, which likely reflects their disease-related progression, and in children with DC, which may be a feature of early-onset severe disease phenotype.

The ribosome-related protein, SBDS, is critical for normal erythropoiesis.

Although anemia is common in Shwachman- Diamond syndrome (SDS), the underlying mechanism remains unclear. We asked whether SBDS, which is mutated in most SDS patients, is critical for erythroid development. We found that SBDS expression is high early during erythroid differentiation. Inhibition of SBDS in CD34(+) hematopoietic stem cells and early progenitors (HSC/Ps) and K562 cells led to slow cell expansion during erythroid differentiation. Induction of erythroid differentiation resulted in markedly accelerated apoptosis in the knockdown cells; however, proliferation was only mildly reduced. The percentage of cells entering differentiation was not reduced. Differentiation also increased the oxidative stress in SBDS-knockdown K562 cells, and antioxidants enhanced the expansion capability of differentiating SBDS-knockdown K562 cells and colony production of SDS patient HSC/Ps. Erythroid differentiation also resulted in reduction of all ribosomal subunits and global translation. Furthermore, stimulation of global translation with leucine improved the erythroid cell expansion of SBDS-knockdown cells and colony production of SDS patient HSC/Ps. Leucine did not reduce the oxidative stress in SBDS-deficient K562 cells. These results demonstrate that SBDS is critical for normal erythropoiesis. Erythropoietic failure caused by SBDS deficiency is at least in part related to elevated ROS levels and translation insufficiency because antioxidants and leucine improved cell expansion.

Lipomatosis of the sciatic nerve secondary to compression by a desmoid tumor.

Lipomatosis of nerve is a rare benign tumor-like process characterized by infiltration of the epineurium by adipose and fibrous tissue leading to nerve enlargement. We describe a case of lipomatosis of the sciatic nerve compressed by an adjacent desmoid tumor. This case supports the hypothesis that lipomatosis of nerve may form as a result of irritation or compression by adjacent structures.

Recurrent growth of lipomas after previous treatment with phosphatidylcholine and deoxycholate.

Injections with phosphatidylcholine- and deoxycholate-containing substances are used to reduce localized fat accumulations. Recent studies have suggested that this procedure may be a non-surgical alternative for reducing the size of lipomas. However, only short-term data about efficacy are available, and it is unclear whether a permanent reduction in lipoma size is possible. In the present case we report a patient who presented with recurrent growth lipomas that were previously successfully treated using phosphatidylcholine and deoxycholate.

Thyrolipoma and thyrolipomatosis: 5 case reports and historical review of the literature.

Because thyrolipoma (adenolipoma of thyroid) and thyrolipomatosis (diffuse lipomatosis of thyroid) are distinctively rare conditions with only few cases reported in the literature, we are reporting 5 additional cases. All the 5 patients were adult females, with ages from 38 to 79 years, who presented with thyroid masses. Four of the patients had normal thyroid function tests and one had mild hypothyroidism. All patients received partial or total thyroidectomy. The thyroid specimens showed either circumscribed yellow-tan masses (cases 1, 2, and 3) or diffuse yellow-brown discoloration (cases 4 and 5). Histologic examination revealed abundant mature fat infiltrating the affected thyroid tissue in 3 distinct patterns: (1) fat infiltration limited to follicular adenomas (thyrolipoma); (2) fat diffusely infiltrating throughout the thyroid gland (thyrolipomatosis); or (3) fat infiltration involving both follicular adenoma and their surrounding thyroid tissue. Because of the rarity of thyroid fat-containing lesions, confusion in differential diagnosis may occasionally occur. It is important to be aware during frozen section that these lesions may present as extrathyroidal nodules, which can be radioactive on intraoperative scan for parathyroid glands. In addition, a papillary thyroid carcinoma was also identified in one case of thyrolipomatosis. All patients recovered well after surgery and there has been no recurrence of the lesions after 1 to 24 years of clinical follow-up. In summary, we are reporting 5 rare cases of thyrolipoma and thyrolipomatosis with distinct histologic patterns. Previously reported cases of thyrolipomatosis were reviewed and analyzed with the current cases.

Encephalocraniocutaneous lipomatosis: A case report with review of literature.

Encephalocraniocutaneous lipomatosis (ECCL) or Haberland syndrome is an uncommon sporadic neurocutaneous syndrome of unknown origin. The rarity and common ignorance of the condition often makes diagnosis difficult. The hallmark of this syndrome is the triad of skin, ocular and central nervous system (CNS) involvement and includes a long list of combination of conditions. Herein we report a case of a 5-month-old male child who presented to our centre with complaint of seizure. The patient had various cutaneous and ocular stigmatas of the disease in the form of patchy alopecia of the scalp, right-sided limbal dermoid and a nodular skin tag near the lateral canthus of the right eye. MRI of the brain was conducted which revealed intracranial lipoma and arachnoid cyst. The constellation of signs and symptoms along with the skin, ocular and CNS findings led to the diagnosis of ECCL.

Topical capsaicin for the treatment of acute lipodermatosclerosis and lobular panniculitis.

Topical capsaicin is well known to reduce nociceptive pain and neurogenic inflammation by depleting substance P. Its fibrinolytic and antithrombotic effects are less well known. We report two cases of acute lipodermatosclerosis which did not respond to several conventional treatment regimens and one case of acute lobular panniculitis in a pregnant woman with venous insufficiency, who were successfully treated within a short period of 3 weeks with topical 0.075% capsaicin cream.

Severity of presentation is associated with time to recovery in spinal epidural lipomatosis.

We present a patient with prednisone-induced spinal epidural lipomatosis (SEL) and relatively acute neurologic deterioration followed by rapid recovery after surgical decompression. SEL is a rare disease characterized by hypertrophy of epidural fat, most commonly associated with exogenous steroid use. To our knowledge, an analysis of the dynamics of steroid dose related to time to onset has never been performed, or of patient presentation features with respect to patient outcome. We retrospectively reviewed the literature for English language series and case reports of SEL associated with prednisone use from 1975-2013. Data were compiled for 41 patients regarding the prescribed dose of prednisone and length of treatment, as well as the severity of symptoms on the Ranawat scale, time to onset, time to recovery, and degree of recovery of neurological symptoms. Fisher's exact test and analysis of variance were used for comparing proportions, and p values <0.05 were considered statistically significant. We found that the mean cumulative dose of prednisone trended towards an association with a lack of recovery (p=0.06) and may be related to rate of recovery. Prescribed prednisone dose varied inversely with the time before onset of neurological symptoms, but failed to reach statistical significance. Higher severity of presenting symptoms on the Ranawat scale were found to be associated with a higher likelihood of delayed recovery (p=0.035). Patients with symptoms lower on the Ranawat scale more frequently experienced complete neurologic recovery, though this did not reach significance. The acuity of neurological deterioration was not related to the time to recovery or ultimate degree of recovery. Severity of presentation on the Ranawat scale is associated with rate of recovery and may be related to degree of recovery in SEL patients. Cumulative dose of prednisone may be related to degree and rate of recovery. Prescribed dose of prednisone may be related to time to onset of neurological symptoms. Acuity of neurological deterioration is not related to rate or degree of recovery.

Hepatitis associated with Chinese herbs.

Traditional Chinese herbal medicines are widely available in Western society and are popular as a form of 'natural' alternative medicine. Their use is increasing, as they are perceived to be free of side effects, but they remain largely unregulated. We describe two patients who suffered severe hepatitis, one of whom died, after taking Chinese herbal remedies for minor complaints. We also review the English-language literature on hepatitis associated with Chinese herbs. Two products appear to be implicated frequently: Jin bu huan was taken by 11 patients, and Dictamnus dasycarpus was taken by six patients, including both fatal cases. It is difficult to provide conclusive evidence of what caused hepatitis, as these products are mixtures that may contain adulterants. These cases highlight not only the potential dangers of these products to consumers but also the need for greater control of their manufacture and use.

[Pelvic lipomatosis--what to do?]. / Lipomatosis pelvis--was tun?

A diffuse fatty infiltration of all the intrapelvic organs (bladder, rectum, sigmoid, colon) was present in a 67-year old-man. This led to the typical topographical and functional changes in the organs affected. Treatment has been very controversial. Surgical measures such as exeresis of the fat tissue with or without the intrapelvic organs affected by the abnormal fat deposition with subsequent drainage (colostomy/ileum conduit) were carried out. Conservative treatment with or without temporary cortisone administration was recommended. Pelvic lipomatosis is diagnosed radiologically. If there is no unequivocal restriction in the function of the affected organs, treatment should remain conservative.

Familial multiple lipomatosis.

Familial multiple lipomatosis is a rare hereditary syndrome with a proposed autosomal-dominant inheritance. A case of an 89-year-old man with this disease is presented, along with his pedigree. Clinical features, genetic evidence, and treatment options are reviewed.

[Two cases of spinal epidural lipomatosis].

Two cases of spinal epidural lipomatosis (SEL) were reported. Patient 1 was on oral corticosteroid and patient 2 was obese and had prostate cancer. Patient 1 was a 45-year-old man diagnosed as sarcoid myelopathy at C 5/6 vertebral body levels and had been placed on oral corticosteroid therapy for 14 months. He showed spastic paraplegia, hypesthesia below C 4 level with distal dominancy and dysesthesia below Th 6 level. MRI revealed epidural lipomatosis from Th 3 to Th 9 vertebral bodies, which presented high in T 1 weighted image (WI) and T 2 WI, and non-signal in STIR image. On axial image spinal cord was compressed by this mass. Patient 2 was a 73-year-old man with spastic paraplegia, and superficial and deep sensory disturbances below Th 6. He had been obese (BMI 26.1) upon admission. He was diagnosed as prostate cancer with bone metastasis. On MRI of the thoracic spine revealed epidural mass of high in T 1 WI and T 2 WI, and non-signal in STIR image. SEL is a rare condition known as hyperplasia of normal fat tissue in epidural space which sometimes compresses the spinal cord or spinal nerve roots resulting in neurologic deficit. SEL should be kept in mind as having possible neurologic complications in obese patients or ones on long term steroid therapy.

[Method of injection necrotherapy of benign tumors of the subcutaneous cellular tissue]. / Metod in"ektsionnoi nekroterapii dobrokachestvennykh opukholei podkozhnoi kletchatki.

With the aim of exterminating small (up to 3--4 cm. in diameter), single or multiple subcutaneous fat tumors (lipoma etc.) the author suggests to use transcutaneous injections of solutions possessing necrotizing properties (10% calcium chloride, 96% alcohol, formalin) into the tumor instead of its surgical removal. The injection should be done under local anesthesia, slowly, the amount of solution not exceeding 0.5--1 of the volume of the tumor. The necrotherapy has been used for 4 years upon 14 patients (32 tumors); besides, the author made 12 self-injections. In 3 cases a repeated injection was required. There were no complications or recurrences.

[Lipomatous hypertrophy of the interatrial septum is a rare, benign cause of atrial arrhythmia]. / Lipomatøs hypertrofi af atrieseptum er en sjælden benign årsag til atrial arytmi.

Lipomatous hypertrophy of the interatrial septum is a pathological thickening of the interatrial septum characterized by a benign infiltration of fat. It usually occurs in older, obese persons and in most cases the diagnosis is made incidentally. Atrial arrhythmias may occur and in rare cases also superior vena cava syndrome caused by obstruction of venous inflow to the right atrium. We present a case of lipomatous hypertrophy of the interatrial septum in an 81-year-old woman with atrial arrhythmia.