Vulvar Rhabdomyosarcoma (VR) is a rare gynecological cancer primarily found in children. This case report discusses the diagnosis, treatment, and management complexities of a 19-year-old patient with a slow-growing vulvar lesion.

A 19-year-old female with obesity and non-alcoholic fatty liver disease presented with a left vulvar lesion measuring 11 x 7 x 7 cm that was noticed five months ago. Core needle biopsy of the lesion revealed findings consistent with rhabdomyosarcoma. A Positron Emission Tomography-Computed Tomography (PET-CT) scan showed a hypermetabolic 8.3 x 6.7 x 6.7 cm mass in the left vulvar area, extending to the vagina, rectal wall, and anal region along with enlarged left inguinal lymph nodes.

The patient was treated with the Intergroup Rhabdomyosarcoma Study-IV (IRS-IV) protocol for 16 weeks with vincristine, dactinomycin, and cyclophosphamide. Concurrent chemoradiotherapy was administered between weeks 9-14, followed by continuation chemotherapy until week 28. Interim PET-CT scan prior to concurrent chemoradiotherapy revealed a reduced mass size to 3.8 x 2.8 cm and resolved left inguinal lymphadenopathy.

Despite completing treatment, the patient reported persistent back pain and mobility issues three weeks later. A subsequent PET-CT scan showed hypermetabolic lesions at vertebral locations C6, T9, T12, and L1-L3, along with the left ischium and bilateral femoral shafts. Thoracic vertebrae biopsy confirmed rhabdomyoblasts. Patient underwent palliative radiotherapy and spinal stabilization then proceeded with second line therapy with 1 cycle of GemcitabineDocetaxel but showed progression of symptoms described as persistent bleeding (hematuria) and neutropenia. Further diagnostics done to the patient showed possible bone marrow involvement. Unfortunately, the patient expired owing to symptoms of cancer progression.

The management of adult-onset VR presents a significant therapeutic challenge, largely attributable to the scarcity of clinical trials and tailored treatment regimens for this specific age group. Outcomes documented in existing literature for adult VR cases present with recurrence, disease progression, and mortality. The treatment landscape in adults is complicated by comorbidities which may influence both the therapeutic choices and outcomes. Given these intricate challenges, this case echoes the need for research efforts aimed at developing management protocols specifically designed for adults with VR.

Contarini’s syndrome refers to the occurrence of bilateral pleural effusion which has different causes for each hemithorax. Based on extensive literature search, this is a rare finding and to date, only two published cases have recorded tuberculous effusion on one side. In this paper, the authors aim to present a case of Contarini’s syndrome, and to give emphasis that such condition with different etiologies exists and should be considered in managing bilateral effusion. This is a case of a 69-year-old female with a 7-week history of dyspnea, 2-pillow orthopnea, fever, and right-sided chest discomfort. Patient sought consultation and was prescribed with Diclofenac and Cefalexin with no relief. Patient was then admitted and intubated due to worsening dyspnea. Patient was managed as COVID-19 confirmed critical with viral myocarditis, CAP-HR, and diabetic ketoacidosis. Initial chest x-ray showed right-sided pleural effusion. Thoracentesis was done and revealed exudative pleural fluid (PF) with WBC of 20,000 with neutrophilic predominance and negative RT-PCR MTB. Cytology revealed acute inflammatory pattern. Klebsiella pneumoniae ESBL was isolated. Antibiotics were shifted to levofloxacin and meropenem. Repeat chest x-ray showed left-sided pleural effusion. Thoracentesis was done and revealed exudative PF with WBC of 1,680 with neutrophilic predominance. No organism was isolated. RT-PCR for MTB was detected. Thus, anti-TB therapy was initiated. However, ETA TB culture showed resistance to isoniazid, rifampicin, and pyrazinamide. Patient was referred to PMDT for MDR-TB treatment. Bilateral effusion has resolved with no recurrence, and with uneventful removal of bilateral chest tubes. Patient was eventually extubated and transferred to the ward. Patient however developed HAP, was re-intubated and eventually expired due to the septic shock from VAP. This case report highlights the importance of weighing risk versus benefit in deciding to perform bilateral thoracentesis when there is a clinical suspicion of an alternate or concurrent diagnosis.

Necrotizing fasciitis of the breast is a rare but potentially fatal soft tissue infection. It may occur primarily in patients without any direct cause, and less commonly after undergoing elective surgical procedures such as cosmetic mammoplasties and oncologic resections.

This is a case of a 46-year-old female with stage IIIA invasive ductal carcinoma of the left breast treated with modified radical mastectomy presenting with a necrotizing infection involving the bilateral breast regions and left lateral abdomen six days after operation. She was managed with broad-spectrum antibiotics and radical debridement with right mastectomy, followed by wound coverage with split-thickness skin grafting. This is the eight case of breast necrotizing fasciitis occurring after mastectomy for breast cancer reported in the literature.

BACKGROUND AND OBJECTIVE

Tuberculosis (TB) remains to be prevalent in the Philippines and globally. Female genital tuberculosis has devastating and permanent consequences, hence, timely and adequate treatment is needed. Since more data regarding optimal duration of treatment of genital tuberculosis are needed, this study compares the treatment response at six months and after at least nine months of treatment, with the intention of determining the most practical management for genital tuberculosis.

A retrospective chart review was conducted for newly diagnosed cases of genital tuberculosis who met the inclusion criteria. Treatment response was categorized into clinical, microbiologic, histologic, radiologic, and sonographic responses. Responses to treatment were evaluated as either partial or complete at the 6th month and after at least 9 months of treatment, and the proportions were compared.

Out of 140 charts retrieved, only 43 were included. Statistically significant difference was found only in clinical response, primarily due to patients who did not achieve resumption of menstruation within the f irst six months of treatment. The rest of the treatment responses and adverse drug events are equally the same for both time periods.

Results of this study show that the proportion of patients with microbiologic, histologic, radiologic, and sonographic response to treatment at the 6th month did not significantly differ to the proportion of patients who responded at the 9th or 12th month of treatment. This leads to a conclusion that the 6-month treatment regimen will be more practical in treating genital tuberculosis, except in amenorrheic premenopausal women who may warrant extension of treatment. Further studies on post-treatment rates of relapse and sonographic resolution are needed.

Neuropsychiatric SLE (NPSLE) comprises the neurologic and psychiatric syndromes observed in patients with SLE after exclusion of other causes. The diagnosis of NPSLE is challenging due to diverse clinical manifestations and absence of laboratory or radiologic biomarkers.

We present the case of a patient with SLE with a chronic isolated seizure and was successfully managed with antiepileptic medication and high-dose corticosteroids.

Seizures may occur as an isolated manifestation of an SLE flare. Ischemic and inflammatory causes of seizure may coexist in active lupus and both should be considered in managing patients. A prompt and holistic workup to rule out metabolic, infectious, and structural neural causes and lupus disease activity of seizures is prudent for patients with SLE.

Weber-Christian Disease (WCD), or relapsing febrile nodular panniculitis is a rare form of subcutaneous fat inflammation commonly affecting women aged 40-75 years old. It is rarer in children with only 1 previously reported case in the Philippines. It presents as erythematous nodules, frequently affecting the lower extremities. There is no standard treatment, but reportedly improves with steroid therapy, cyclosporine, and immunomodulators.

This is a case of a 12-year-old-female, who presented with a 4-month history of palpable facial nodules, thigh pains, and recurrent fever. Work-up for hypersensitivity reaction, autoimmune condition, hematologic problem, or infectious etiology yielded unremarkable findings, and corresponding management had no significant response. Patient developed firm lesions on the abdomen which was sent for biopsy and showed results consistent with WCD. Steroid, hydroxychloroquine, and topical indomethacin were then started and noted gradual improvement of the lesions. Patient was then discharged improved with lesions noted to progressively decrease in size and with no appearance of newer lesions upon follow-up.

WCD is a rare form of nodular panniculitis, more so in the younger age group. It is characterized by presence of cutaneous lesions associated with systemic symptoms. Skin biopsy is necessary to confirm its diagnosis. Visceral organ involvement and failure to respond to treatment may result to poor prognosis, and occasionally leads to death.

A 61-year-old woman presented with a 2-month history of non-painful left eye proptosis. Imaging studies showed a superotemporal mass in the left orbit with intracranial extension. Surgical excision of the orbitocranial mass was performed and histopathologic examination revealed metastatic papillary thyroid carcinoma. She subsequently underwent total thyroidectomy. Orbital metastasis from thyroid carcinoma is rare and can be the initial manifestation of occult disease in 63% of cases.

Posterior fossa malformations are rare cyst-like pathologies of the central nervous system. Outcomes of patients with these conditions are largely documented in fetal or early childhood studies as most cases are non-compatible with life. Also, different schemes to categorize these occurrences have been proposed. One classification puts forth that the entities are a continuum of pathologies, called the Dandy Walker Complex, wherein Mega Cisterna Magna is the most benign to Dandy Walker Malformation as the most severe form. We report the first case of a patient with a mixed presentation of Mega Cisterna Magna having extracranial manifestations of Dandy Walker Malformation reaching her adult years. The patient is a 26-year-old female who was apparently well until she presented with recurrent headache and seizure episodes of 2-year duration. She had an unremarkable birth and childhood history, apart from learning difficulties in school. In her adult years, she gave birth to an infant with multiple physical anomalies. She has a maternal uncle with abnormal facie and intellectual disability. Physical examination of the patient exhibited a bulging occiput, hypertelorism, down-slanting palpebral fissures, large globular nose, large ear lobules, high arched palate, and clinodactyly. Neurologic examination was unremarkable. Magnetic resonance imaging confirmed Mega Cisterna Magna and was deemed non-surgical. She remained seizure-free during admission as well as on followup, maintained on Phenytoin. The existence of a Dandy-Walker continuum is still debated, as the link among the disease entities are yet to be established using developmental or genetic studies. This case, however, supports the Dandy-Walker Complex classification by demonstrating a rare combination of Mega Cisterna Magna with features of Dandy-Walker Malformation in an adult. This can contribute to disease definition and eventually to the discovery of the pathobiological mechanisms of posterior fossa cysts, and to appropriate diagnosis and management.

BACKGROUND

Granulomatous mastitis (GM) of the breast has long posed a clinical dilemma in terms of diagnosis and management. GM can range from acute to chronic inflammations, which will have treatment implications. Inflammation of the breast may clinically mimic breast carcinomas and pyogenic abscesses. Thus, in the absence of known etiology, such as trauma or breastfeeding, GM may be difficult to diagnose. Currently, the incidence of GM is 2.4 per 100,000 women and 0.37% of the total population in the US. The rarity of GM contributes to it being a poorly understood disease. It has no definite clinical features and is often confused with a neoplasm or infection clinically and radiologically.

The study aimed to describe the clinical characteristics of GM seen in the Breast Care Clinic in the Philippine General Hospital (PGH) from January 2015 to June 2019. This study would initially provide institutional data on GM that is relevant in the Philippines.

This is a retrospective observational study of patients with GM seen in the Philippine General Hospital, a national tertiary referral hospital, from January 2015 to June 2019.

A total of 43 patients with pathological findings of GM from January 1, 2015 to June 15, 2019 were recorded. Among these 43 patients, 98% were female. The median age was 38.9 ± 11.3 years old. In 60.5% (26 out of 43) of patients, the initial impression was breast malignancy. The most common clinical presentation in 69.8% (30 out of 43) of the subjects was a breast mass. In more than 50% of the patients, breast ultrasonography was the initial imaging performed. The histopathologic profile of the patients showed inflammation, of which, the greatest were that of chronic granulomatous inflammation (46.5%, n-20). Treatment options performed were tended more medical (53.5%, n-23) than surgical (16.3%, n-7).Among those who received medical treatment, the therapeutics given were antitubercular medications (34.9%, n-15) and antibiotics (16.3%, n-7), while the others had a combination of antitubercular and antibiotic regimen medications (2.3%, n-1); unknown treatment (25.6%, n-11) and none (11.6%, n-5). For patient outcomes, no mortalities were recorded during the study period. However, most patients had inconsistent followups. Approximately 7%-23% of the patients who had followed up within the six months showed improvement or resolution of symptoms.

This study assessed the clinical profiles of patients with GM in a national tertiary referral hospital. Internationally, there is still no consensus on the algorithm and management of GM patients. However, the authors recommend a close follow-up every two weeks to re-evaluate patient response to the medical regimen being administered. The authors recommend a prospective study with a longer follow-up period to gain a deeper understanding of GM in Filipinos.

OBJECTIVE

Preterm birth is a major cause of complications leading to death of children under 5 years old. Infections are important to be identified because antimicrobial treatment may prevent or delay progression to preterm delivery. This study determined if routine screening tests of infections are useful in the clinical management of preterm labor.

A cross-sectional (descriptive) study was done involving 417 pregnant patients who had preterm labor and was subsequently admitted from 2015 to 2019 at a tertiary hospital in the Philippines, using review of past medical records, inpatient charts, and admission charts.

Majority of the patients delivered at less than 34 weeks, most of the culture results turned out negative, and urine tests were more commonly employed as screening tests for preterm labor. The endocervical and rectovaginal swab studies had no significant growths. Asymptomatic bacteriuria was diagnosed in 1 out of 10 patients and they were subsequently started on antibiotic treatment. Majority of the patients who were given antibiotics delivered within 48 hours from admission. 

The routine use of cultures in the assessment of preterm labor is costly and is unlikely to be helpful in the clinical management of patients in preterm labor.