Giant Ameloblastoma and the Risk of Recurrence: Experiences from a Tertiary Hospital.

BACKGROUND: Ameloblastoma is an aggressive tumor of odontogenic epithelium that grows slowly with propensity for bone expansion. Sometimes it may grow to very large sizes also known as giant ameloblastoma (GA) which may affect function and even pose a threat to life. OBJECTIVE: To present the pattern of presentation of GAs seen in a tertiary centre in Northern Nigeria. PATIENTS AND METHODS: A retrospective study of patients seen with GA at the Oral and Maxillofacial Clinic of a tertiary health facility of Northern Nigeria between January 2006 to December 2019. All patients with complete documentation in the folder, theatre register and histopathologic records were recruited for the study. Data were analyzed using SPSS version 23. RESULTS: GAs accounted for 30.2% (48) of all the ameloblastomas operated during the period with a male dominance of 62.5% (30), giving a ratio of 1.7:1(M:F). The age range was between 12 and 65 years with a mean age of 35.04years (±14.5) and the mandible was the most affected jaw compared to the maxilla (12.5%). Lesions with lowest and highest weight were found on the mandible (77g and 1640g respectively). The treatment most given was mandibulectomy (unspecified) with 20.0% followed by Rt and Lt mandibulectomies with 14.0% each. CONCLUSION: GA was found to account for 30.2% of all lesions seen within the period under review, while the mandible was the most affected jaw bone.

CONTEXTE: L'améloblastome est une tumeur agressive d'épithélium odontogène qui se développe lentement avec une propension à expansion osseuse. Parfois, il peut atteindre de très grandes tailles aussiconnu sous le nom d'améloblastome géant (GA) qui peut affecter la function et constituent même une menace pour la vie. OBJECTIF: Présenter le modèle de présentation des AGvu dans un centre tertiaire dans le nord du Nigeria. PATIENTS ET METHODES: Une étude rétrospective des patients vu avec GA à la clinique orale et maxillo-faciale d'un tertiaire établissement de santé du nord du Nigéria entre janvier 2006 et Décembre 2019. Tous les patients avec une documentation complète dans le dossier, le registre des théâtres et les dossiers histopathologiques étaient recruté pour l'étude. Les données ont été analysées à l'aide de la version SPSS23. RÉSULTATS: Les AG représentaient 30.2 % (48) de tous les améloblastomes opérés pendant la période avec un home dominance de 62.5% (30), ce qui donne un rapport de 1.7:1 (M:F).la fourchette se situait entre 12 et 65 ans avec un âge moyen de 35.04 ans(±14.5) et la mandibule était la mâchoire la plus touchée comparéeau maxillaire (12.5 %). Lésions de poids le plus faible et le plus élevé ont été trouvés sur la mandibule (77g et 1640g respectivement). Le traitement le plus administré était la mandibulectomie (non spécifiée) avec 20.0 %, suivis des mandibulectomies Rt et Lt avec 14.0 % chacun. CONCLUSION: L'AG représentait 30.2 % de tous les lésions observées au cours de la période considérée, tandis que la mandibule était l'os de la mâchoire le plus touché. Mots-clés: Améloblastome géant, Risque de récidive, Expérience.

Giant Aggressive Odontogenic Fibroma in a 15-month-old Female: Case Report and Review of Literature.

INTRODUCTION: Odontogenic fibroma (OF) is a rare benign tumor of mesenchymal odontogenic tissue with variable global incidence. Two variants comprising extraosseous /peripheral (POF) and intraosseous /central (COF) have been identified based on the anatomical origin of the tumor. OF isslow growing, and often asymptomatic with characteristic radiographic appearance of a unilocular or multilocular cyst frequently associated with unerupted or displaced teeth while histologically, varying amount of inactive odontogenic epithelium embedded in a mature relatively dense collagenous stroma is diagnostic. The absence of odontogenic epithelium does not exclude the diagnosis of odontogenic fibroma. CASE REPORT: A 15 months old female presented with 3 months history of left lower jaw swelling which showed aggressive growth following incisional biopsy. Parents said lesion was noticed for the first time as pinpoint on the submandibular area and was too small to warrant any worries. Initial radiograph revealed absence of bony involvement but the second showed evidence of bony involvement. An impression of soft tissue sarcoma was made and incisional biopsy was sent for pathological diagnosis which revealed peripheral odontogenic fibroma. The rapidity of growth following incisional biopsy bellied a benign lesion. CONCLUSION: An aggressive odontogenic fibroma with multifocal points in a 15months old girl.

INTRODUCTION: Le fibrome odontogène (FO) est une tumeur bénigne rare du tissu odontogène mésenchymateu dont l'incidence globale est variable. Deux variantes comprenant des fibromes extra-osseux /périphérique (POF) et intra-osseuse / centrale (COF) ont été identifiés en fonction de l'origine anatomique de la tumeur. La POF est à croissance lente, et souvent asymptomatique avec l'apparence radiographique caractéristique d'un kyste uniloculaire ou multiloculaire fréquemment associé à des dents non érigées ou déplacées alors que histologiquement, une quantité variable d'épithélium inactif noyé dans un stroma collagène mature relativement dense est le diagnostic. L'absence d'épithélium odontogène n'exclut pas le diagnostic de fibrome odontogène. RAPPORT DE CAS: Une femme de 15 mois a présenté un gonflement de la mâchoire inférieure gauche depuis 3 mois de gonflement de la mâchoire inférieure gauche qui a montré une croissance agressive après une biopsie incisionnelle. Les parents ont déclaré que la lésion avait été remarquée pour la première fois sur forme de point d'épingle dans la zone submandibulaire et était trop petite pour justifier une quelconque inquiétude. La radiographie initiale a révélé l'absence d'atteinte osseuse mais la seconde a montré des signes d'atteinte osseuse. Une impression de sarcome des tissus mous et une biopsie incisionnelle a été envoyée pour un diagnostic pathologique qui a révélé un fibrome odontogène périphérique. La rapidité de la croissance après la biopsie incisionnelle a fait croire à une lésion bénigne. CONCLUSION: Un fibrome odontogène agressif avec des points multifocaux chez une jeune fille de 15 mois. Mots clés: Fibrome odontogène, Périphérique, Central, Agressif.

Sarcomatoid Renal Cell Carcinoma in an Adolescent with Sickle Cell Anaemia.

Malignancies have been reported to occur in people with sickle cell disease. Renal medullary carcinoma (RMC), also tagged seventh sickle cell nephropathy, is an aggressive cancer seen almost exclusively in people with sickle cell disease with more than 160 cases reported worldwide, but only few cases were reported in patients with sickle cell anaemia (HBSS) and from Nigeria. Sarcomatoid renal cell carcinoma is a renal tumour of any histologic variant containing foci of high-grade malignant spindle cells. We report an adolescent girl with sickle cell anaemia (HBSS) who presented with left renal tumour, histology of which confirmed a diagnosis of sarcomatoid renal cell carcinoma (sRCC). Surgical debulking and palliative care with chemotherapy were given, and she demised 10 months after. The rarity of the case and challenges of managing a cancer in the background of a chronic haematologic disorder are highlighted.

Clinico-pathologic characterisation of metastatic prostate cancer in the Radiotherapy and Oncology Department, Ahmadu Bello University Teaching Hospital, Zaria-Nigeria: 2006-2009.

AIMS AND OBJECTIVES: To evaluate the Clinico-pathologic Characteristics of Metastatic prostate cancer patients seen in the Radiotherapy and Oncology Department, Ahmadu Bello University Teaching Hospital, Nigeria. MATERIALS AND METHODS: Between January 2006 and December 2009, a period of 4 years, 72 new patients with prostate cancers were seen of which 43 patients had distant metastases. Only patients with histologic confirmation of prostate cancer and having metastases were included in the study irrespective of age, co-morbidity and performance status. Patients' folders were reviewed retrospectively with a structured pro forma. Information retrieved from patient's folder included age, histology, Gleason score, co-morbidities, interval between diagnosis and referral to oncology unit, interval between referral and presentation at oncology unit, PSA at diagnosis and presentation at oncology unit, sites of metastases, bones sites involved in bone metastases, types of treatment received and follow up status. Results were analysed using Epi Info soft ware Version 3.4.1; 2007 Edition. RESULTS: 43 patients had distant metastases from prostate cancer during initial evaluation at presentation in the radiotherapy and oncology centre. The mean age was 66.2 years (range, 47-82 years, median age 66 years and modal age group was 65-69 years). Co-morbidity was seen in 18 patients, with hypertension being the commonest (HT=14, DM=2 and HIV 2 patients). No morbidity seen in 25 patients. The range of duration from diagnosis to referral was 1-84 months. Only 20 patients presented at radiotherapy and oncology centre within 6 months of diagnosis and 18 patients presented after 12 months of diagnosis. 33 patients presented within 1 month of being referred for further management. 6 patients reported within 2 months and 2 patients within 3 months and another 2 patients within 4 months. Only 27 patients had PSA done at diagnosis. No PSA was done in 16 patients. The PSA range at diagnosis was 10.0-232 ng/ml, mean PSA was 67.46 ng/ml while only 40 patients did PSA on presentation for further management with a range of 1-245 ng/ml and a mean of 57.95 ng/ml. The histology report revealed adenocarcinoma and transitional carcinoma in 42 and 1 patients respectively. The Gleason score range was 6-10, with a mean score of 7.8. The Gleason score was not reported in 3 patients. Multiple organs involvement by metastases was seen in 16 patients. Bone metastases was the commonest (35), followed by lungs (8), liver (7), Virchow's lymph nodes (6), brain (5), and soft tissue (5). The lumbar vertebrae was the commonest site of bone metastases (32) followed by the sacrum (17), pelvis (11), and long bones (7). 3 patients had metastases to the ribs and 2 patients each to the skull, sternum and cervical spines. All the patients received hormonal therapy, 30 patients received palliative radiotherapy, 29 patients had surgical castration, 25 patients had radical radiotherapy to pelvis, 18 patients had systemic chemotherapy and definitive surgery was done in 16 patients. Only 6 patients received Ibandronate due to cost. 13 patients are alive and attending follow up, 11 confirmed dead and 19 lost to follow up. CONCLUSION: The patients were referred for management late after surgery. Bones, predominately the lumbosacral spines was the commonest organ involved in metastases. Skeletal survey with bone scan and plain x-rays especially lumbosacral spines should be part of the staging investigation for all patients with advanced stage. There is need for more awareness on other treatment modalities emphasizing the multidiscipline and multimodality management of prostate cancer.

Lymphadenopathic Kaposi's sarcoma in an immunocompetent adult.

Kaposi's sarcomas (KS) are vascular lesions which usually originate from multiple sites in the mid-dermis extending to the dermis. The aetiology is unknown, but infection from human herpes virus type 8 has been suggested. Several reports of KS had come from Africa initially and from worldwide later due to the close association with HIV/AIDS. Prior to this however, KS was very frequent in Eastern Europe, Italy and the United States where it existed in an indolent form in the elderly men of Jewish ancestry. KS may also be due to iatrogenic immune suppression from chronic use of steroids, elevated degree of expression of numerous cytokines and angiogenic growth factors including TNF alpha, IL-6, bFGF, HIVtat protein and oncostatin M. Lymphadenopathic KS involves the lymph-nodes, viscera and the gastrointestinal tract and may run a disseminated and aggressive course. We are reporting one such case in an immunocompetent male.

Pattern of oncologic emergencies seen in adult cancer patients attending the Radiotherapy and Oncology Centre, Ahmadu Bello University Teaching Hospital, Zaria - Nigeria.

AIMS AND OBJECTIVES: To evaluate the Pattern of Oncologic Emergencies seen in Adult cancer patients and the treatment modalities used. MATERIALS AND METHODS: Between January 2004 and December 2008, a total of 1824 (M:F = 1:1.8) new patients were seen. 196 (M:F = 1:1.4) consecutive patients with histologically confirmed malignancies presenting with or having oncologic emergencies were treated and have been reviewed. Patients' folders were reviewed retrospectively with a structured pro forma. Results were analysed using Epi Info soft ware Version 3.4.1; 2007 Edition. RESULTS: The median age was 49 years and mean age of 42 years (range, 15 - 82 years). M: F = 1:1.4. 162 patients had oncologic emergencies at presentation while 21 during treatments and 13 during follow up. At the time of diagnosis of oncologic emergency, 126 were not on any treatment, 42 patients on hormonal therapy and 28 patients were on diverse chemotherapy. All the patients presented late with 108 patients presenting with metastatic disease and 88 patients with locally advanced disease. Only 35 patients were treated within 1 week of onset of emergency. 59 patients had cervical cancer, 31 patients with breast cancer and 28 patients with prostate cancer. Tumour haemorrhage wass the commonest oncologic emergency seen in 107 patients followed by bone pain with imminent cord compression from bone metastases in 59 patients. Of 107 patients with tumour haemorrhage, 54 patients had cardiovascular collapse with 7 having acute renal failure. Similarly, of the 107 with tumour haemorrhage, 56 patients bled from cervical cancer, 12 patients from breast cancer and 8 patients from urinary bladder. 129 patients were treated with teletherapy, 31 patients had chemotherapy, 27 patients had emergency surgery and 5 patients had chemoradiation. Oncologic emergencies were corrected in 126 patients. CONCLUSION: Tumour haemorrhage is the commonest oncologic emergency in this environment and teletherapy is the commonest therapy used. More radiotherapy centres are needed for prompt treatment and their usefulness in managing emergencies should be made known. Oncologic emergencies are commonly seen in metastatic and locally advanced disease.

Extrapulmonary tuberculosis: fine needle aspiration cytology diagnosis.

BACKGROUND: The increasing prevalence of extrapulmonary manifestation of tuberculosis with the HIV scourge is a cause for concern. OBJECTIVE: To determine the role of fine needle aspiration cytology (FNAC) in the diagnosis of extrapulmonary tuberculosis. PATIENTS AND METHODS: This is a consecutive 9-year analysis of patients with peripheral lymphadenopathy. All the patients had fine needle aspiration. Smears were made, fixed in 95% alcohol and stained with hematoxylin and eosin and Zeihl Neelsen stains. RESULTS: 48 patients, 31 males and 17 females, were analyzed. The mean age was 27.9 years. Aspirates were mainly from cervical lymph nodes. Four patients were HIV 1 seropositive. Macroscopically, 23 (48%) of the aspirates were purulent and 7 (14.6%) had caseous material. Microscopy showed granular eosinophilic material (caseation), multinucleated giant cells, epithelioid cells, neutrophils and lymphocytes. Staining for acid-fast bacilli was positive in 23 (48%) cases. CONCLUSION: Early diagnosis of extrapulmonary tuberculosis in a resource-limited setting can be achieved with fine needle aspiration cytology technique (FNAC). This will ensure prompt treatment and thus reduce attendant morbidity and mortality.

Cisplatin chemotherapy for haemostasis in bleeding cervical cancer: experience from a resource-poor setting.

BACKGROUND: Cervical cancer is the commonest cancer in northern Nigeria. There are only two radiotherapy centers in the north and four centres in the south, each with only one megavoltage machine for a population of over 140 million. The number of patients requiring radiotherapy for various malignancies is beyond the available facilities and expertise leading to long waiting time and disease progression with its attendant sequelae. This is the basis of using other orthodox treatment modalities as first line. PATIENTS AND METHODS: Between January 2006 and December 2007, 116 patients with histologically confirmed cervical cancer with vaginal bleeding as the predominant symptom were treated. Patients presenting with torrential haemorrhage were excluded from this study as they constitute oncologic emergencies. Patients were interviewed with a structured pro forma on a 3-weekly basis during chemotherapy schedules to assess and evaluate per vaginal bleeding and discharge. Dose of chemotherapy was 70 mg/m² every 3 weeks. Results were analysed using Epi Info soft ware Version 3.4.1; 2007 Edition. RESULTS: The median age was 49 years (27-80 yrs). 62 patients were having per vagina bleeding for more than 6 months before commencement of chemotherapy (range 1-60 months). 49 patients had blood transfusion before chemotherapy, average of 2.7 pints of blood transfused per patient. 84 had at least FIGO stage IIIA disease. Squamous cell carcinoma is the commonest histology type followed by adenocarcinoma with 95 and 16 patients respectively. 81 patients had complete cessation of per vagina bleeding with 69 having complete cessation on or before 4th course of chemotherapy (9th week) and complete cessation of per vagina discharges was seen in 52 patients. 115 patients had a performance status KPS of below 80 prior to chemotherapy, and after completing 6 cycles, 100 patients had KPS of 80 and above. CONCLUSION: In resource-poor setting, Cisplatin based chemotherapy can be used by medical, gynaecological oncologists and general practitioners to control vaginal bleeding and improve the quality of life of patients pending radiotherapy. For optimal treatment with chemoradiotherapy, government and non-governmental agencies must do all it takes to remedy the problems of shortage of resources.

Leiomyosarcoma uteri in a white woman.

Sarcoma of the corpus uteri should be considered as a differential diagnosis in postmenopausal women presenting with severe abdominal pain, thought to be due to degenerative changes of leiomyoma uteri. We present a rare case of leiomyosarcoma in a postmenopausal White woman. Diagnosis was missed preoperatively for degenerating uterine fibroid.

Adnexal skin tumors in Zaria, Nigeria.

BACKGROUND: Adnexal skin tumors share many features in common and differentiate along one line. Their detailed morphological classification is difficult because of the variety of tissue elements and patterns seen. They may be clinically confused with other cutaneous tumors. The aim of this report is to review and classify all adnexal tumors seen in a pathology department over a 16-year period. METHOD: A 16-year retrospective analysis of all adnexal skin tumors seen in a large University Teaching Hospital in Nigeria from January 1991- December 2006. All tissue specimens were fixed in 10% formalin, processed in paraffin wax and stained with Haematoxylin and Eosin. Histology slides were retrieved, studied and lesions characterized. RESULTS: Fifty-two adnexal tumors were seen, accounting for 0.9% of all cutaneous tumors seen within the same period. The median age was 33 years (range: 4 days-70 years). Clinical presentations varied from discreet swellings and nodules to ulcerated masses. Five patients presented with recurrent lesions. Only two cases had a clinical diagnosis of adnexal tumor. Twenty-four (46%) of the lesions were distributed in the head and neck region. Duration of symptoms was 2 months to 15 years (median: 12 months). Tumours of the sweat gland were the commonest--41 (78.8%); they comprised predominantly eccrine acrospiroma (17), characterized histologically by solid nests of round to polygonal cells with clear to eosinophilic cytoplasm, forming tubules in areas. Tumours of sebaceous gland were 7 (13.5%); they comprised mainly Nevus sebaceous of Jadassohn (6), composed of immature sebaceous glands and pilar structures microscopically and a solitary sebaceous adenoma. Tumours of hair follicle were 4 (7.7%) and included trichoepithelioma, characterized microscopically by multiple horn cysts and epithelial tracts connecting abortive pilar structures and a trichofolliculoma. Forty-six lesions (88.5%) were benign and six (11.5%) malignant. CONCLUSION: Adnexal skin tumors have distinct histological patterns which differentiates them from other cutaneous tumors. They are commonly distributed in the head, neck and trunk. The commonest variants are those of eccrine sweat gland origin. Malignant adnexal tumors are uncommon in our setting.

Adnexal Skin Tumors in Zaria; Nigeria

Background: Adnexal skin tumors share many features in common and differentiate along one line. Their detailed morphological classification is difficult because of the variety of tissue elements and patterns seen. They may be clinically confused with other cutaneous tumors. The aim of this report is to review and classify all adnexal tumors seen in a pathology department over a 16 year period. Method: A 16-year retrospective analysis of all adnexal skin tumors seen in a large University Teaching Hospital in Nigeria from January 1991- December 2006. All tissue specimens were fixed in 10formalin; processed in paraffin wax and stained with Haematoxylin and Eosin. Histology slides were retrieved; studied and lesions characterized. Results: Fifty-two adnexal tumors were seen; accounting for 0.9of all cutaneous tumors seen within the same period. The median age was 33 years (range: 4 days -70 years). Clinical presentations varied from discreet swellings and nodules to ulcerated masses. Five patients presented with recurrent lesions. Only two cases had a clinical diagnosis of adnexal tumor. Twenty-four (46) of the lesions were distributed in the head and neck region. Duration of symptoms was 2 months to 15 years (median: 12 months). Tumours of the sweat gland were the commonest- 41(78.8); they comprised predominantly eccrine acrospiroma(17); characterized histologically by solid nests of round to polygonal cells with clear to eosinophilic cytoplasm; forming tubules in areas. Tumours of sebaceous gland were 7 (13.5); they comprised mainly Nevus sebaceous of Jadassohn (6); composed of immature sebaceous glands and pilar structures microscopically and a solitary sebaceous adenoma. Tumours of hair follicle were 4 (7.7) and included trichoepithelioma; characterized microscopically by multiple horn cysts and epithelial tracts connecting abortive pilar structures and a trichofolliculoma. Forty-six lesions (88.5) were benign and six (11.5) malignant. Conclusion: Adnexal skin tumors have distinct histological patterns which differentiates them from other cutaneous tumors. They are commonly distributed in the head; neck and trunk. The commonest variants are those of eccrine sweat gland origin. Malignant adnexal tumors are uncommon in our setting

Ovarian pregnancy presenting as ovarian tumour: report of 2 cases.

We present 2 cases of ovarian masses diagnosed as tumours but turned out to be pregnancy. Ovarian pregnancy, a form of ectopic gestation has a distinct pathology though it can be a source of diagnostic difficulty clinically and intraoperatively. A total of 71 ectopic pregnancies were seen in the department from January 2001 to December 2005, of these only two were ovarian ectopics. Both patients were nulliparous and presented with lower abdominal pains, abdominal masses and menstrual irregularity. They both had laparotomy and total right salpingo-oophorectomy. Grossly, the ovaries were enlarged, grey tan and globular. Focal ruptures in the wall of the ovaries showed protruding fetal parts. Microscopy showed chorionic villi within and in continuity with ovarian stroma and corpus luteum. They were both diagnosed ovarian ectopics. A good knowledge and understanding of the gross pathology, combination of imaging studies and high index of suspicion should help in making an intra-operative diagnosis.

Foetal chondrodysplasia: intrauterine diagnosis.

Chondrodysplasias are rare occurrence. We present a case of short-limbed chondrodyplasia diagnosed in a 22 week fetus of a 28 year old. She was para 4(+0) with two children alive. She had similar occurrences in her last two pregnancies. The index pregnancy and the last two eventful pregnancies were preceded with ingestion of trado-herbal drug to treat subfertility.

Histopathologic analysis of epidermal skin tumours and tumour-like lesions in Ahmadu Bello University Teaching Hospital, Zaria.

OBJECTIVE: A histopathologic analysis of epidermal skin tumours and tumour-like lesions seen between 1991 - 2000 in the Department of Pathology, Ahmadu Bello University Teaching Hospital [A.B.U.T.H], Zaria is presented. PATIENTS AND METHOD: These tumours were classified according to World Health Organisation's International Histological Classification for Skin Tumours and were tabulated. RESULTS: A total of 350 such lesions comprising 9.9% of all cutaneous neoplasms seen within the study period were collected. Overall, they have a male to female ratio of 1.7:1. Malignant tumours constituted 72.5%; benign tumours 18.3% and tumour-like lesions 9.2%. The commonest malignant lesion was squamous cell carcinoma, which constituted 68.3% of all the lesions with a male to female ratio of 1.7:1. Epidermal cyst comprised 16.3% with a male to female ratio of 1.5:1. CONCLUSION: It is concluded that epidermal tumours and tumour-like lesions are not uncommon in Zaria and they show a male preponderance with squamous cell carcinoma being the commonest epidermal tumour and it also predominantly affects males.

Pattern of Cutaneous Malignant Melanoma in Zaria; Nigeria

Background: Twenty percent of the world's melanoma is seen in Black Africans and Asians. Melanoma in Nigerians would appear to be arising from existing epidermal melanocytes and not from preexisting naevus cells.Methods: All diagnosed cases of cutaneous malignant melanoma in the Department of Pathology Ahmadu Bello University Teaching Hospital; Zaria; Nigeria over a ten-year study period (1991-2000) were reviewed. The age; sex and anatomical site of the lesion were obtained from the request forms. The histopathological patterns of distribution; presence of dark brown melanin pigments; nucleolar appearance and Clark's histological grading were studied. The data was analysed and tabulated into frequency tables.Results: Fifty four cases of cutaneous malignant melanoma were reviewed. The over all male: female sex ratio was 1.3:1.0 with a wide age distribution from the second to seventh decades and two peaks in fifth and sixth decades. The foot and lower limbs were the most common sites. Seventy two percent of the cases were of high histological grade (Clark's level III and IV); thirty-six cases were distributed predominantly in sheets while 50 cases had dark brown melanin pigments. All showed characteristic prominent eosinophilic nucleolus.Conclusion: Malignant melanoma is a common skin tumour in Zaria. It is commonest in the fifth and sixth decades. The feet and lower limbs are predominantly affected